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Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment

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Abstract

Interstitial lung disease is a common pulmonary manifestation of connective tissue diseases and results in significant morbidity and mortality. Although all connective tissue diseases are linked by underlying autoimmunity, there is significant variability in the presentation and clinical course of interstitial lung disease associated with the different types of connective tissue diseases. In this paper, we review the prevalence, patterns, predictors, and prognosis of interstitial lung disease secondary to the most common forms of connective tissue diseases. We have also highlighted the available evidence regarding treatment options for interstitial lung disease due to different connective tissue diseases.

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Data Availability

All data generated or analyzed during this study are included in this published article.

Abbreviations

ILD:

Interstitial lung disease

CTD:

Connective tissue disease

SSc:

Systemic sclerosis

RA:

Rheumatoid arthritis

PM:

Polymyositis

DM:

Dermatomyositis

SS:

Sjögren’s syndrome

SLE:

Systemic lupus erythematosus

MCTD:

Mixed connective tissue disease

HRCT:

High-resolution computed tomography

UIP:

Usual interstitial pneumonia

NSIP:

Non-specific interstitial pneumonia

OP:

Organizing pneumonia

DAD:

Diffuse alveolar damage

CPK:

Creatinine phosphokinase

FVC:

Forced vital capacity

DLCO:

Diffusion capacity for carbon monoxide

BAL:

Bronchoalveolar lavage

IPAF:

Interstitial pneumonia with autoimmune features

PH:

Pulmonary hypertension

HSCT:

Hematopoietic stem cell transplantation

References

  1. Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, Lee JS, Leslie KO, Lynch DA, Matteson EL, Mosca M, Noth I, Richeldi L, Strek ME, Swigris JJ, Wells AU, West SG, Collard HR, Cottin V, CTD-ILD EATFoUFo (2015) An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J 46(4):976–987. https://doi.org/10.1183/13993003.00150-2015

    Article  CAS  PubMed  Google Scholar 

  2. Wells AU, Denton CP (2014) Interstitial lung disease in connective tissue disease–mechanisms and management. Nat Rev Rheumatol 10(12):728–739. https://doi.org/10.1038/nrrheum.2014.149

    Article  CAS  PubMed  Google Scholar 

  3. Levi Y, Israeli-Shani L, Kuchuk M, Epstein Shochet G, Koslow M, Shitrit D (2018) Rheumatological assessment is important for interstitial lung disease diagnosis. J Rheumatol 45(11):1509–1514. https://doi.org/10.3899/jrheum.171314

    Article  PubMed  Google Scholar 

  4. Cottin V, Brown KK (2019) Interstitial lung disease associated with systemic sclerosis (SSc-ILD). Respir Res 20(1):13. https://doi.org/10.1186/s12931-019-0980-7

    Article  PubMed  PubMed Central  Google Scholar 

  5. Akter T, Silver RM, Bogatkevich GS (2014) Recent advances in understanding the pathogenesis of scleroderma-interstitial lung disease. Curr Rheumatol Rep 16(4):411. https://doi.org/10.1007/s11926-014-0411-1

    Article  CAS  PubMed  Google Scholar 

  6. McNearney TA, Reveille JD, Fischbach M, Friedman AW, Lisse JR, Goel N, Tan FK, Zhou X, Ahn C, Feghali-Bostwick CA, Fritzler M, Arnett FC, Mayes MD (2007) Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum 57(2):318–326. https://doi.org/10.1002/art.22532

    Article  PubMed  Google Scholar 

  7. Steen VD, Conte C, Owens GR, Medsger TA Jr (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37(9):1283–1289

    Article  CAS  Google Scholar 

  8. Steen VD, Owens GR, Fino GJ, Rodnan GP, Medsger TA Jr (1985) Pulmonary involvement in systemic sclerosis (scleroderma). Arthritis Rheum 28(7):759–767

    Article  CAS  Google Scholar 

  9. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, Tscholakoff D (1990) Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 176(3):755–759. https://doi.org/10.1148/radiology.176.3.2389033

    Article  CAS  PubMed  Google Scholar 

  10. Steele R, Hudson M, Lo E, Baron M, Canadian Scleroderma Research G (2012) Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis. Arthritis Care Res (Hoboken) 64(4):519–524. https://doi.org/10.1002/acr.21583

    Article  Google Scholar 

  11. Suliman YA, Dobrota R, Huscher D, Nguyen-Kim TD, Maurer B, Jordan S, Speich R, Frauenfelder T, Distler O (2015) Brief report: pulmonary function tests: high rate of false-negative results in the early detection and screening of scleroderma-related interstitial lung disease. Arthritis Rheumatol 67(12):3256–3261. https://doi.org/10.1002/art.39405

    Article  CAS  PubMed  Google Scholar 

  12. Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, Haslam PL, Vassilakis DA, Black CM, du Bois RM (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165(12):1581–1586. https://doi.org/10.1164/rccm.2106012

    Article  PubMed  Google Scholar 

  13. Kim DS, Yoo B, Lee JS, Kim EK, Lim CM, Lee SD, Koh Y, Kim WS, Kim WD, Colby TV, Kitiaichi M (2002) The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 19(2):121–127

    PubMed  Google Scholar 

  14. Goldin JG, Lynch DA, Strollo DC, Suh RD, Schraufnagel DE, Clements PJ, Elashoff RM, Furst DE, Vasunilashorn S, McNitt-Gray MF, Brown MS, Roth MD, Tashkin DP, Scleroderma Lung Study Research G (2008) High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 134(2):358–367. https://doi.org/10.1378/chest.07-2444

    Article  PubMed  Google Scholar 

  15. Walker UA, Tyndall A, Czirjak L, Denton C, Farge-Bancel D, Kowal-Bielecka O, Muller-Ladner U, Bocelli-Tyndall C, Matucci-Cerinic M (2007) Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis 66(6):754–763. https://doi.org/10.1136/ard.2006.062901

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Fertig N, Domsic RT, Rodriguez-Reyna T, Kuwana M, Lucas M, Medsger TA Jr, Feghali-Bostwick CA (2009) Anti-U11/U12 RNP antibodies in systemic sclerosis: a new serologic marker associated with pulmonary fibrosis. Arthritis Rheum 61(7):958–965. https://doi.org/10.1002/art.24586

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Okano Y, Steen VD, Medsger TA Jr (1993) Autoantibody reactive with RNA polymerase III in systemic sclerosis. Ann Intern Med 119(10):1005–1013

    Article  CAS  Google Scholar 

  18. Sacks DG, Okano Y, Steen VD, Curtiss E, Shapiro LS, Medsger TA Jr (1996) Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3RNP antibody. J Rheumatol 23(4):639–642

    CAS  PubMed  Google Scholar 

  19. Hudson M, Pope J, Mahler M, Tatibouet S, Steele R, Baron M, Fritzler MJ, Canadian Scleroderma Research G (2012) Clinical significance of antibodies to Ro52/TRIM21 in systemic sclerosis. Arthritis Res Ther 14(2):R50. https://doi.org/10.1186/ar3763

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Hant FN, Ludwicka-Bradley A, Wang HJ, Li N, Elashoff R, Tashkin DP, Silver RM, Scleroderma Lung Study Research G (2009) Surfactant protein D and KL-6 as serum biomarkers of interstitial lung disease in patients with scleroderma. J Rheumatol 36(4):773–780. https://doi.org/10.3899/jrheum.080633

    Article  CAS  PubMed  Google Scholar 

  21. Gladman DD, Kung TN, Siannis F, Pellett F, Farewell VT, Lee P (2005) HLA markers for susceptibility and expression in scleroderma. J Rheumatol 32(8):1481–1487

    CAS  PubMed  Google Scholar 

  22. Peljto AL, Steele MP, Fingerlin TE, Hinchcliff ME, Murphy E, Podlusky S, Carns M, Schwarz M, Varga J, Schwartz DA (2012) The pulmonary fibrosis-associated MUC5B promoter polymorphism does not influence the development of interstitial pneumonia in systemic sclerosis. Chest 142(6):1584–1588. https://doi.org/10.1378/chest.12-0110

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Winstone TA, Assayag D, Wilcox PG, Dunne JV, Hague CJ, Leipsic J, Collard HR, Ryerson CJ (2014) Predictors of mortality and progression in scleroderma-associated interstitial lung disease: a systematic review. Chest 146(2):422–436. https://doi.org/10.1378/chest.13-2626

    Article  PubMed  Google Scholar 

  24. Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, Corte TJ, Sander CR, Ratoff J, Devaraj A, Bozovic G, Denton CP, Black CM, du Bois RM, Wells AU (2008) Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 177(11):1248–1254. https://doi.org/10.1164/rccm.200706-877OC

    Article  PubMed  Google Scholar 

  25. Goh NS, Hoyles RK, Denton CP, Hansell DM, Renzoni EA, Maher TM, Nicholson AG, Wells AU (2017) Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol 69(8):1670–1678. https://doi.org/10.1002/art.40130

    Article  CAS  PubMed  Google Scholar 

  26. Ciancio N, Pavone M, Torrisi SE, Vancheri A, Sambataro D, Palmucci S, Vancheri C, Di Marco F, Sambataro G (2019) Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases. Multidiscip Respir Med 14:17. https://doi.org/10.1186/s40248-019-0179-2

    Article  PubMed  PubMed Central  Google Scholar 

  27. Morisset J, Vittinghoff E, Elicker BM, Hu X, Le S, Ryu JH, Jones KD, Haemel A, Golden JA, Boin F, Ley B, Wolters PJ, King TE Jr, Collard HR, Lee JS (2017) Mortality risk prediction in scleroderma-related interstitial lung disease: the SADL model. Chest 152(5):999–1007. https://doi.org/10.1016/j.chest.2017.06.009

    Article  PubMed  PubMed Central  Google Scholar 

  28. Wu W, Jordan S, Becker MO, Dobrota R, Maurer B, Fretheim H, Ye S, Siegert E, Allanore Y, Hoffmann-Vold AM, Distler O (2018) Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model. Ann Rheum Dis 77(9):1326–1332. https://doi.org/10.1136/annrheumdis-2018-213201

    Article  CAS  PubMed  Google Scholar 

  29. Ryerson CJ, O'Connor D, Dunne JV, Schooley F, Hague CJ, Murphy D, Leipsic J, Wilcox PG (2015) Predicting mortality in systemic sclerosis-associated interstitial lung disease using risk prediction models derived from idiopathic pulmonary fibrosis. Chest 148(5):1268–1275. https://doi.org/10.1378/chest.15-0003

    Article  PubMed  Google Scholar 

  30. Strange C, Bolster MB, Roth MD, Silver RM, Theodore A, Goldin J, Clements P, Chung J, Elashoff RM, Suh R, Smith EA, Furst DE, Tashkin DP, Scleroderma Lung Study Research G (2008) Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med 177(1):91–98. https://doi.org/10.1164/rccm.200705-655OC

    Article  CAS  PubMed  Google Scholar 

  31. Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel DE, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, Scleroderma Lung Study Research G (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354(25):2655–2666. https://doi.org/10.1056/NEJMoa055120

    Article  CAS  PubMed  Google Scholar 

  32. Goh NS, Veeraraghavan S, Desai SR, Cramer D, Hansell DM, Denton CP, Black CM, du Bois RM, Wells AU (2007) Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum 56(6):2005–2012. https://doi.org/10.1002/art.22696

    Article  PubMed  Google Scholar 

  33. Launay D, Remy-Jardin M, Michon-Pasturel U, Mastora I, Hachulla E, Lambert M, Delannoy V, Queyrel V, Duhamel A, Matran R, De Groote P, Hatron PY (2006) High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J Rheumatol 33(9):1789–1801

    PubMed  Google Scholar 

  34. Savarino E, Bazzica M, Zentilin P, Pohl D, Parodi A, Cittadini G, Negrini S, Indiveri F, Tutuian R, Savarino V, Ghio M (2009) Gastroesophageal reflux and pulmonary fibrosis in scleroderma: a study using pH-impedance monitoring. Am J Respir Crit Care Med 179(5):408–413. https://doi.org/10.1164/rccm.200808-1359OC

    Article  PubMed  Google Scholar 

  35. Richardson C, Agrawal R, Lee J, Almagor O, Nelson R, Varga J, Cuttica MJ, Dematte JD, Chang RW, Hinchcliff ME (2016) Esophageal dilatation and interstitial lung disease in systemic sclerosis: a cross-sectional study. Semin Arthritis Rheum 46(1):109–114. https://doi.org/10.1016/j.semarthrit.2016.02.004

    Article  PubMed  PubMed Central  Google Scholar 

  36. Morales-Cardenas A, Perez-Madrid C, Arias L, Ojeda P, Mahecha MP, Rojas-Villarraga A, Carrillo-Bayona JA, Anaya JM (2016) Pulmonary involvement in systemic sclerosis. Autoimmun Rev 15(11):1094–1108. https://doi.org/10.1016/j.autrev.2016.07.025

    Article  PubMed  Google Scholar 

  37. Bauer PR, Schiavo DN, Osborn TG, Levin DL, St Sauver J, Hanson AC, Schroeder DR, Ryu JH (2013) Influence of interstitial lung disease on outcome in systemic sclerosis: a population-based historical cohort study. Chest 144(2):571–577. https://doi.org/10.1378/chest.12-2768

    Article  PubMed  PubMed Central  Google Scholar 

  38. Nihtyanova SI, Schreiber BE, Ong VH, Rosenberg D, Moinzadeh P, Coghlan JG, Wells AU, Denton CP (2014) Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol 66(6):1625–1635. https://doi.org/10.1002/art.38390

    Article  PubMed  Google Scholar 

  39. Khanna D, Tseng CH, Farmani N, Steen V, Furst DE, Clements PJ, Roth MD, Goldin J, Elashoff R, Seibold JR, Saggar R, Tashkin DP (2011) Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group. Arthritis Rheum 63(10):3078–3085. https://doi.org/10.1002/art.30467

    Article  PubMed  PubMed Central  Google Scholar 

  40. Steen VD, Medsger TA Jr (2000) Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 43(11):2437–2444. https://doi.org/10.1002/1529-0131(200011)43:11<2437:AID-ANR10>3.0.CO;2-U

    Article  CAS  PubMed  Google Scholar 

  41. Steen VD, Medsger TA (2007) Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 66(7):940–944. https://doi.org/10.1136/ard.2006.066068

    Article  PubMed  PubMed Central  Google Scholar 

  42. Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Muller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumanovics G, Coleiro B, Feierl E, Szucs G, Von Muhlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kotter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Stork J, Morovic-Vergles J, Walker UA (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809–1815. https://doi.org/10.1136/ard.2009.114264

    Article  PubMed  Google Scholar 

  43. Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM, Goldin J, Arriola E, Strange C, Bolster MB, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel D, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M, Khanna D, Li N, Li G, Scleroderma Lung Study Research G (2007) Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med 176(10):1026–1034. https://doi.org/10.1164/rccm.200702-326OC

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC, Goldin J, Arriola E, Volkmann ER, Kafaja S, Silver R, Steen V, Strange C, Wise R, Wigley F, Mayes M, Riley DJ, Hussain S, Assassi S, Hsu VM, Patel B, Phillips K, Martinez F, Golden J, Connolly MK, Varga J, Dematte J, Hinchcliff ME, Fischer A, Swigris J, Meehan R, Theodore A, Simms R, Volkov S, Schraufnagel DE, Scholand MB, Frech T, Molitor JA, Highland K, Read CA, Fritzler MJ, Kim GHJ, Tseng CH, Elashoff RM, Sclerodema Lung Study III (2016) Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med 4(9):708–719. https://doi.org/10.1016/S2213-2600(16)30152-7

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM, Investigators ST (2019) Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 380(26):2518–2528. https://doi.org/10.1056/NEJMoa1903076

    Article  CAS  PubMed  Google Scholar 

  46. Khanna D, Albera C, Fischer A, Khalidi N, Raghu G, Chung L, Chen D, Schiopu E, Tagliaferri M, Seibold JR, Gorina E (2016) An open-label, Phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial. J Rheumatol 43(9):1672–1679. https://doi.org/10.3899/jrheum.151322

    Article  PubMed  Google Scholar 

  47. Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME, Baron M, Chung L, Fierlbeck G, Lakshminarayanan S, Allanore Y, Pope JE, Riemekasten G, Steen V, Muller-Ladner U, Lafyatis R, Stifano G, Spotswood H, Chen-Harris H, Dziadek S, Morimoto A, Sornasse T, Siegel J, Furst DE (2016) Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet 387(10038):2630–2640. https://doi.org/10.1016/S0140-6736(16)00232-4

    Article  CAS  PubMed  Google Scholar 

  48. Khanna D, Denton CP, Lin CJF, van Laar JM, Frech TM, Anderson ME, Baron M, Chung L, Fierlbeck G, Lakshminarayanan S, Allanore Y, Pope JE, Riemekasten G, Steen V, Muller-Ladner U, Spotswood H, Burke L, Siegel J, Jahreis A, Furst DE (2018) Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis 77(2):212–220. https://doi.org/10.1136/annrheumdis-2017-211682

    Article  CAS  PubMed  Google Scholar 

  49. van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, Schuerwegh AJ, Marijt EW, Vonk MC, Schattenberg AV, Matucci-Cerinic M, Voskuyl AE, van de Loosdrecht AA, Daikeler T, Kotter I, Schmalzing M, Martin T, Lioure B, Weiner SM, Kreuter A, Deligny C, Durand JM, Emery P, Machold KP, Sarrot-Reynauld F, Warnatz K, Adoue DF, Constans J, Tony HP, Del Papa N, Fassas A, Himsel A, Launay D, Lo Monaco A, Philippe P, Quere I, Rich E, Westhovens R, Griffiths B, Saccardi R, van den Hoogen FH, Fibbe WE, Socie G, Gratwohl A, Tyndall A, Group EESS (2014) Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA 311(24):2490–2498. https://doi.org/10.1001/jama.2014.6368

    Article  CAS  PubMed  Google Scholar 

  50. Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B, Mayes MD, Nash RA, Crofford LJ, Eggleston B, Castina S, Griffith LM, Goldstein JS, Wallace D, Craciunescu O, Khanna D, Folz RJ, Goldin J, St Clair EW, Seibold JR, Phillips K, Mineishi S, Simms RW, Ballen K, Wener MH, Georges GE, Heimfeld S, Hosing C, Forman S, Kafaja S, Silver RM, Griffing L, Storek J, LeClercq S, Brasington R, Csuka ME, Bredeson C, Keever-Taylor C, Domsic RT, Kahaleh MB, Medsger T, Furst DE, Investigators SS (2018) Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med 378(1):35–47

    Article  Google Scholar 

  51. Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Kazantzi A, Sirinian C, Karampetsou M, Yiannopoulos G, Andonopoulos AP (2010) Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford) 49(2):271–280. https://doi.org/10.1093/rheumatology/kep093

    Article  CAS  Google Scholar 

  52. Jordan S, Distler JH, Maurer B, Huscher D, van Laar JM, Allanore Y, Distler O, group ERs (2015) Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis 74(6):1188–1194. https://doi.org/10.1136/annrheumdis-2013-204522

    Article  CAS  PubMed  Google Scholar 

  53. Steen VD, Medsger TA Jr (1998) Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum 41(9):1613–1619. https://doi.org/10.1002/1529-0131(199809)41:9<1613:AID-ART11>3.0.CO;2-O

    Article  CAS  PubMed  Google Scholar 

  54. Ando K, Motojima S, Doi T, Nagaoka T, Kaneko N, Aoshima M, Takahashi K (2013) Effect of glucocorticoid monotherapy on pulmonary function and survival in Japanese patients with scleroderma-related interstitial lung disease. Respir Investig 51(2):69–75. https://doi.org/10.1016/j.resinv.2012.12.002

    Article  PubMed  Google Scholar 

  55. Nadashkevich O, Davis P, Fritzler M, Kovalenko W (2006) A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol 25(2):205–212. https://doi.org/10.1007/s10067-005-1157-y

    Article  CAS  PubMed  Google Scholar 

  56. Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, Roberts C, Desai S, Herrick AL, McHugh NJ, Foley NM, Pearson SB, Emery P, Veale DJ, Denton CP, Wells AU, Black CM, du Bois RM (2006) A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 54(12):3962–3970. https://doi.org/10.1002/art.22204

    Article  CAS  PubMed  Google Scholar 

  57. Miele CH, Schwab K, Saggar R, Duffy E, Elashoff D, Tseng CH, Weigt S, Charan D, Abtin F, Johannes J, Derhovanessian A, Conklin J, Ghassemi K, Khanna D, Siddiqui O, Ardehali A, Hunter C, Kwon M, Biniwale R, Lo M, Volkmann E, Torres Barba D, Belperio JA, Sayah D, Mahrer T, Furst DE, Kafaja S, Clements P, Shino M, Gregson A, Kubak B, Lynch JP, 3rd, Ross D, Saggar R (2016) Lung transplant outcomes in systemic sclerosis with significant esophageal dysfunction. A comprehensive single-center experience. Ann Am Thorac Soc 13(6):793–802.

  58. Pradere P, Tudorache I, Magnusson J, Savale L, Brugiere O, Douvry B, Reynaud-Gaubert M, Claustre J, Borgne AL, Holm AM, Schultz HH, Knoop C, Godinas L, Fisher AJ, Hirschi S, Gottlieb J, Le Pavec J, Working Group on Heart/Lung Transplantation in Systemic S (2018) Lung transplantation for scleroderma lung disease: An international, multicenter, observational cohort study. J Heart Lung Transpl 37(7):903–911. https://doi.org/10.1016/j.healun.2018.03.003

    Article  Google Scholar 

  59. Esposito AJ, Chu SG, Madan R, Doyle TJ, Dellaripa PF (2019) Thoracic manifestations of rheumatoid arthritis. Clin Chest Med 40(3):545–560. https://doi.org/10.1016/j.ccm.2019.05.003

    Article  PubMed  PubMed Central  Google Scholar 

  60. Tanoue LT (1998) Pulmonary manifestations of rheumatoid arthritis. Clin Chest Med 19(4):667–685

    Article  CAS  Google Scholar 

  61. Lee HK, Kim DS, Yoo B, Seo JB, Rho JY, Colby TV, Kitaichi M (2005) Histopathologic pattern and clinical features of rheumatoid arthritis-associated interstitial lung disease. Chest 127(6):2019–2027. https://doi.org/10.1378/chest.127.6.2019

    Article  PubMed  Google Scholar 

  62. Raimundo K, Solomon JJ, Olson AL, Kong AM, Cole AL, Fischer A, Swigris JJ (2019) Rheumatoid arthritis-interstitial lung disease in the United States: prevalence, incidence, and healthcare costs and mortality. J Rheumatol 46(4):360–369. https://doi.org/10.3899/jrheum.171315

    Article  PubMed  Google Scholar 

  63. Gabbay E, Tarala R, Will R, Carroll G, Adler B, Cameron D, Lake FR (1997) Interstitial lung disease in recent onset rheumatoid arthritis. Am J Respir Crit Care Med 156(2 Pt 1):528–535. https://doi.org/10.1164/ajrccm.156.2.9609016

    Article  CAS  PubMed  Google Scholar 

  64. Habib HM, Eisa AA, Arafat WR, Marie MA (2011) Pulmonary involvement in early rheumatoid arthritis patients. Clin Rheumatol 30(2):217–221. https://doi.org/10.1007/s10067-010-1492-5

    Article  PubMed  Google Scholar 

  65. Suzuki A, Ohosone Y, Obana M, Mita S, Matsuoka Y, Irimajiri S, Fukuda J (1994) Cause of death in 81 autopsied patients with rheumatoid arthritis. J Rheumatol 21(1):33–36

    CAS  PubMed  Google Scholar 

  66. Tanaka N, Kim JS, Newell JD, Brown KK, Cool CD, Meehan R, Emoto T, Matsumoto T, Lynch DA (2004) Rheumatoid arthritis-related lung diseases: CT findings. Radiology 232(1):81–91. https://doi.org/10.1148/radiol.2321030174

    Article  PubMed  Google Scholar 

  67. Kim EJ, Collard HR, King TE Jr (2009) Rheumatoid arthritis-associated interstitial lung disease: the relevance of histopathologic and radiographic pattern. Chest 136(5):1397–1405. https://doi.org/10.1378/chest.09-0444

    Article  PubMed  PubMed Central  Google Scholar 

  68. Kelly CA, Saravanan V, Nisar M, Arthanari S, Woodhead FA, Price-Forbes AN, Dawson J, Sathi N, Ahmad Y, Koduri G, Young A, British Rheumatoid Interstitial Lung N (2014) Rheumatoid arthritis-related interstitial lung disease: associations, prognostic factors and physiological and radiological characteristics–a large multicentre UK study. Rheumatology (Oxford) 53(9):1676–1682. https://doi.org/10.1093/rheumatology/keu165

    Article  CAS  Google Scholar 

  69. Mori S, Koga Y, Sugimoto M (2012) Different risk factors between interstitial lung disease and airway disease in rheumatoid arthritis. Respir Med 106(11):1591–1599. https://doi.org/10.1016/j.rmed.2012.07.006

    Article  PubMed  Google Scholar 

  70. Shaw M, Collins BF, Ho LA, Raghu G (2015) Rheumatoid arthritis-associated lung disease. Eur Respir Rev 24(135):1–16. https://doi.org/10.1183/09059180.00008014

    Article  PubMed  Google Scholar 

  71. Zhu J, Zhou Y, Chen X, Li J (2014) A metaanalysis of the increased risk of rheumatoid arthritis-related pulmonary disease as a result of serum anticitrullinated protein antibody positivity. J Rheumatol 41(7):1282–1289. https://doi.org/10.3899/jrheum.131341

    Article  PubMed  Google Scholar 

  72. Harlow L, Rosas IO, Gochuico BR, Mikuls TR, Dellaripa PF, Oddis CV, Ascherman DP (2013) Identification of citrullinated hsp90 isoforms as novel autoantigens in rheumatoid arthritis-associated interstitial lung disease. Arthritis Rheum 65(4):869–879. https://doi.org/10.1002/art.37881

    Article  CAS  PubMed  Google Scholar 

  73. Chen J, Doyle TJ, Liu Y, Aggarwal R, Wang X, Shi Y, Ge SX, Huang H, Lin Q, Liu W, Cai Y, Koontz D, Fuhrman CR, Golzarri MF, Liu Y, Hatabu H, Nishino M, Araki T, Dellaripa PF, Oddis CV, Rosas IO, Ascherman DP (2015) Biomarkers of rheumatoid arthritis-associated interstitial lung disease. Arthritis Rheumatol 67(1):28–38. https://doi.org/10.1002/art.38904

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  74. Maniwa K, Ogushi F, Tani K, Ohmoto Y, Muraguchi M, Sone S (2000) Increased incidence of autoantibodies to interleukin-1a in rheumatoid arthritis with interstitial lung disease. Respirology 5(4):315–320

    CAS  PubMed  Google Scholar 

  75. Nakajima H, Harigai M, Hara M, Hakoda M, Tokuda H, Sakai F, Kamatani N, Kashiwazaki S (2000) KL-6 as a novel serum marker for interstitial pneumonia associated with collagen diseases. J Rheumatol 27(5):1164–1170

    CAS  PubMed  Google Scholar 

  76. Garcia JG, Parhami N, Killam D, Garcia PL, Keogh BA (1986) Bronchoalveolar lavage fluid evaluation in rheumatoid arthritis. Am Rev Respir Dis 133(3):450–454. https://doi.org/10.1164/arrd.1986.133.3.450

    Article  CAS  PubMed  Google Scholar 

  77. Juge PA, Lee JS, Ebstein E, Furukawa H, Dobrinskikh E, Gazal S, Kannengiesser C, Ottaviani S, Oka S, Tohma S, Tsuchiya N, Rojas-Serrano J, Gonzalez-Perez MI, Mejia M, Buendia-Roldan I, Falfan-Valencia R, Ambrocio-Ortiz E, Manali E, Papiris SA, Karageorgas T, Boumpas D, Antoniou K, van Moorsel CHM, van der Vis J, de Man YA, Grutters JC, Wang Y, Borie R, Wemeau-Stervinou L, Wallaert B, Flipo RM, Nunes H, Valeyre D, Saidenberg-Kermanac'h N, Boissier MC, Marchand-Adam S, Frazier A, Richette P, Allanore Y, Sibilia J, Dromer C, Richez C, Schaeverbeke T, Liote H, Thabut G, Nathan N, Amselem S, Soubrier M, Cottin V, Clement A, Deane K, Walts AD, Fingerlin T, Fischer A, Ryu JH, Matteson EL, Niewold TB, Assayag D, Gross A, Wolters P, Schwarz MI, Holers M, Solomon JJ, Doyle T, Rosas IO, Blauwendraat C, Nalls MA, Debray MP, Boileau C, Crestani B, Schwartz DA, Dieude P (2018) MUC5B promoter variant and rheumatoid arthritis with interstitial lung disease. N Engl J Med 379(23):2209–2219. https://doi.org/10.1056/NEJMoa1801562

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  78. Kim EJ, Elicker BM, Maldonado F, Webb WR, Ryu JH, Van Uden JH, Lee JS, King TE Jr, Collard HR (2010) Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 35(6):1322–1328. https://doi.org/10.1183/09031936.00092309

    Article  CAS  PubMed  Google Scholar 

  79. Zamora-Legoff JA, Krause ML, Crowson CS, Ryu JH, Matteson EL (2017) Progressive decline of lung function in rheumatoid arthritis-associated interstitial lung disease. Arthritis Rheumatol 69(3):542–549. https://doi.org/10.1002/art.39971

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  80. Song JW, Lee HK, Lee CK, Chae EJ, Jang SJ, Colby TV, Kim DS (2013) Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 30(2):103–112

    CAS  PubMed  Google Scholar 

  81. Dawson JK, Fewins HE, Desmond J, Lynch MP, Graham DR (2001) Fibrosing alveolitis in patients with rheumatoid arthritis as assessed by high resolution computed tomography, chest radiography, and pulmonary function tests. Thorax 56(8):622–627

    Article  CAS  Google Scholar 

  82. Solomon JJ, Chung JH, Cosgrove GP, Demoruelle MK, Fernandez-Perez ER, Fischer A, Frankel SK, Hobbs SB, Huie TJ, Ketzer J, Mannina A, Olson AL, Russell G, Tsuchiya Y, Yunt ZX, Zelarney PT, Brown KK, Swigris JJ (2016) Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 47(2):588–596. https://doi.org/10.1183/13993003.00357-2015

    Article  PubMed  Google Scholar 

  83. Hozumi H, Nakamura Y, Johkoh T, Sumikawa H, Colby TV, Kono M, Hashimoto D, Enomoto N, Fujisawa T, Inui N, Suda T, Chida K (2013) Acute exacerbation in rheumatoid arthritis-associated interstitial lung disease: a retrospective case control study. BMJ Open 3(9):e003132. https://doi.org/10.1136/bmjopen-2013-003132

    Article  PubMed  PubMed Central  Google Scholar 

  84. Conway R, Low C, Coughlan RJ, O'Donnell MJ, Carey JJ (2014) Methotrexate and lung disease in rheumatoid arthritis: a meta-analysis of randomized controlled trials. Arthritis Rheumatol 66(4):803–812. https://doi.org/10.1002/art.38322

    Article  CAS  PubMed  Google Scholar 

  85. Hyldgaard C, Hilberg O, Pedersen AB, Ulrichsen SP, Lokke A, Bendstrup E, Ellingsen T (2017) A population-based cohort study of rheumatoid arthritis-associated interstitial lung disease: comorbidity and mortality. Ann Rheum Dis 76(10):1700–1706. https://doi.org/10.1136/annrheumdis-2017-211138

    Article  PubMed  Google Scholar 

  86. Tsuchiya Y, Takayanagi N, Sugiura H, Miyahara Y, Tokunaga D, Kawabata Y, Sugita Y (2011) Lung diseases directly associated with rheumatoid arthritis and their relationship to outcome. Eur Respir J 37(6):1411–1417. https://doi.org/10.1183/09031936.00019210

    Article  CAS  PubMed  Google Scholar 

  87. Fischer A, Brown KK, Du Bois RM, Frankel SK, Cosgrove GP, Fernandez-Perez ER, Huie TJ, Krishnamoorthy M, Meehan RT, Olson AL, Solomon JJ, Swigris JJ (2013) Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol 40(5):640–646. https://doi.org/10.3899/jrheum.121043

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  88. Md Yusof MY, Kabia A, Darby M, Lettieri G, Beirne P, Vital EM, Dass S, Emery P (2017) Effect of rituximab on the progression of rheumatoid arthritis-related interstitial lung disease: 10 years' experience at a single centre. Rheumatology (Oxford) 56(8):1348–1357. https://doi.org/10.1093/rheumatology/kex072

    Article  CAS  Google Scholar 

  89. Fui A, Bergantini L, Selvi E, Mazzei MA, Bennett D, Pieroni MG, Rottoli P, Bargagli E (2019) Rituximab therapy in interstitial lung disease associated with rheumatoid arthritis. Intern Med J. https://doi.org/10.1111/imj.14306

    Article  Google Scholar 

  90. Fernandez-Diaz C, Loricera J, Castaneda S, Lopez-Mejias R, Ojeda-Garcia C, Olive A, Rodriguez-Muguruza S, Carreira PE, Perez-Sandoval T, Retuerto M, Cervantes-Perez EC, Flores-Robles BJ, Hernandez-Cruz B, Urruticoechea A, Maiz-Alonso O, Arboleya L, Bonilla G, Hernandez-Rodriguez I, Palma D, Delgado C, Exposito-Molinero R, Ruibal-Escribano A, Alvarez-Rodriguez B, Blanco-Madrigal J, Bernal JA, Vela-Casasempere P, Rodriguez-Gomez M, Fito C, Ortiz-Sanjuan F, Narvaez J, Moreno M, Lopez-Corbeto M, Mena-Vazquez N, Aguilera-Cros C, Romero-Yuste S, Ordonez S, Villa-Blanco I, Gonzelez-Vela MC, Mora-Cuesta V, Palmou-Fontana N, Hernandez JL, Gonzalez-Gay MA, Blanco R (2018) Abatacept in patients with rheumatoid arthritis and interstitial lung disease: a national multicenter study of 63 patients. Semin Arthritis Rheum 48(1):22–27. https://doi.org/10.1016/j.semarthrit.2017.12.012

    Article  CAS  PubMed  Google Scholar 

  91. Manfredi A, Cassone G, Furini F, Gremese E, Venerito V, Atzeni F, Arrigoni E, Della Casa G, Cerri S, Govoni M, Petricca L, Iannone F, Salvarani C, Sebastiani M (2019) Tocilizumab therapy in rheumatoid arthritis with interstitial lung disease: a multicenter retrospective study. Intern Med J. https://doi.org/10.1111/imj.14670

    Article  Google Scholar 

  92. Kiely P, Busby AD, Nikiphorou E, Sullivan K, Walsh DA, Creamer P, Dixey J, Young A (2019) Is incident rheumatoid arthritis interstitial lung disease associated with methotrexate treatment? Results from a multivariate analysis in the ERAS and ERAN inception cohorts. BMJ Open 9(5):e028466. https://doi.org/10.1136/bmjopen-2018-028466

    Article  PubMed  PubMed Central  Google Scholar 

  93. Mochizuki T, Ikari K, Yano K, Sato M, Okazaki K (2019) Long-term deterioration of interstitial lung disease in patients with rheumatoid arthritis treated with abatacept. Mod Rheumatol 29(3):413–417. https://doi.org/10.1080/14397595.2018.1481566

    Article  CAS  PubMed  Google Scholar 

  94. Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L, Kolb M, Tetzlaff K, Stowasser S, Coeck C, Clerisme-Beaty E, Rosenstock B, Quaresma M, Haeufel T, Goeldner RG, Schlenker-Herceg R, Brown KK, Investigators IT (2019) Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 381(18):1718–1727. https://doi.org/10.1056/NEJMoa1908681

    Article  CAS  PubMed  Google Scholar 

  95. Courtwright AM, El-Chemaly S, Dellaripa PF, Goldberg HJ (2017) Survival and outcomes after lung transplantation for non-scleroderma connective tissue-related interstitial lung disease. J Heart Lung Transplant 36(7):763–769. https://doi.org/10.1016/j.healun.2016.12.013

    Article  PubMed  Google Scholar 

  96. Yazdani A, Singer LG, Strand V, Gelber AC, Williams L, Mittoo S (2014) Survival and quality of life in rheumatoid arthritis-associated interstitial lung disease after lung transplantation. J Heart Lung Transplant 33(5):514–520. https://doi.org/10.1016/j.healun.2014.01.858

    Article  PubMed  Google Scholar 

  97. Marie I, Hatron PY, Dominique S, Cherin P, Mouthon L, Menard JF (2011) Short-term and long-term outcomes of interstitial lung disease in polymyositis and dermatomyositis: a series of 107 patients. Arthritis Rheum 63(11):3439–3447. https://doi.org/10.1002/art.30513

    Article  CAS  PubMed  Google Scholar 

  98. Morisset J, Johnson C, Rich E, Collard HR, Lee JS (2016) Management of myositis-related interstitial lung disease. Chest 150(5):1118–1128. https://doi.org/10.1016/j.chest.2016.04.007

    Article  PubMed  Google Scholar 

  99. Ikeda S, Arita M, Misaki K, Mishima S, Takaiwa T, Nishiyama A, Ito A, Furuta K, Yokoyama T, Tokioka F, Noyama M, Yoshioka H, Ishida T (2015) Incidence and impact of interstitial lung disease and malignancy in patients with polymyositis, dermatomyositis, and clinically amyopathic dermatomyositis: a retrospective cohort study. Springerplus 4:240. https://doi.org/10.1186/s40064-015-1013-8

    Article  PubMed  PubMed Central  Google Scholar 

  100. Richards TJ, Eggebeen A, Gibson K, Yousem S, Fuhrman C, Gochuico BR, Fertig N, Oddis CV, Kaminski N, Rosas IO, Ascherman DP (2009) Characterization and peripheral blood biomarker assessment of anti-Jo-1 antibody-positive interstitial lung disease. Arthritis Rheum 60(7):2183–2192. https://doi.org/10.1002/art.24631

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  101. Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, Ryu JH (2001) Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 164(7):1182–1185. https://doi.org/10.1164/ajrccm.164.7.2103110

    Article  CAS  PubMed  Google Scholar 

  102. Solomon J, Swigris JJ, Brown KK (2011) Myositis-related interstitial lung disease and antisynthetase syndrome. J Bras Pneumol 37(1):100–109. https://doi.org/10.1590/s1806-37132011000100015

    Article  PubMed  PubMed Central  Google Scholar 

  103. Zhang L, Wu G, Gao D, Liu G, Pan L, Ni L, Li Z, Wang Q (2016) Factors associated with interstitial lung disease in patients with polymyositis and dermatomyositis: a systematic review and meta-analysis. PLoS ONE 11(5):e0155381. https://doi.org/10.1371/journal.pone.0155381

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  104. Marie I, Josse S, Decaux O, Dominique S, Diot E, Landron C, Roblot P, Jouneau S, Hatron PY, Tiev KP, Vittecoq O, Noel D, Mouthon L, Menard JF, Jouen F (2012) Comparison of long-term outcome between anti-Jo1- and anti-PL7/PL12 positive patients with antisynthetase syndrome. Autoimmun Rev 11(10):739–745. https://doi.org/10.1016/j.autrev.2012.01.006

    Article  CAS  PubMed  Google Scholar 

  105. Cavagna L, Trallero-Araguas E, Meloni F, Cavazzana I, Rojas-Serrano J, Feist E, Zanframundo G, Morandi V, Meyer A, Pereira da Silva JA, Matos Costa CJ, Molberg O, Andersson H, Codullo V, Mosca M, Barsotti S, Neri R, Scire C, Govoni M, Furini F, Lopez-Longo FJ, Martinez-Barrio J, Schneider U, Lorenz HM, Doria A, Ghirardello A, Ortego-Centeno N, Confalonieri M, Tomietto P, Pipitone N, Rodriguez Cambron AB, Blazquez Canamero MA, Voll RE, Wendel S, Scarpato S, Maurier F, Limonta M, Colombelli P, Giannini M, Geny B, Arrigoni E, Bravi E, Migliorini P, Mathieu A, Piga M, Drott U, Delbrueck C, Bauhammer J, Cagnotto G, Vancheri C, Sambataro G, De Langhe E, Sainaghi PP, Monti C, Gigli Berzolari F, Romano M, Bonella F, Specker C, Schwarting A, Villa Blanco I, Selmi C, Ceribelli A, Nuno L, Mera-Varela A, Perez Gomez N, Fusaro E, Parisi S, Sinigaglia L, Del Papa N, Benucci M, Cimmino MA, Riccieri V, Conti F, Sebastiani GD, Iuliano A, Emmi G, Cammelli D, Sebastiani M, Manfredi A, Bachiller-Corral J, Sifuentes Giraldo WA, Paolazzi G, Saketkoo LA, Giorgi R, Salaffi F, Cifrian J, Caporali R, Locatelli F, Marchioni E, Pesci A, Dei G, Pozzi MR, Claudia L, Distler J, Knitza J, Schett G, Iannone F, Fornaro M, Franceschini F, Quartuccio L, Gerli R, Bartoloni E, Bellando Randone S, Zampogna G, Gonzalez Perez MI, Mejia M, Vicente E, Triantafyllias K, Lopez-Mejias R, Matucci-Cerinic M, Selva-O'Callaghan A, Castaneda S, Montecucco C, Gonzalez-Gay MA (2019) Influence of antisynthetase antibodies specificities on antisynthetase syndrome clinical spectrum time course. J Clin Med. https://doi.org/10.3390/jcm8112013

    Article  PubMed  PubMed Central  Google Scholar 

  106. Hall JC, Casciola-Rosen L, Samedy LA, Werner J, Owoyemi K, Danoff SK, Christopher-Stine L (2013) Anti-melanoma differentiation-associated protein 5-associated dermatomyositis: expanding the clinical spectrum. Arthritis Care Res (Hoboken) 65(8):1307–1315. https://doi.org/10.1002/acr.21992

    Article  CAS  Google Scholar 

  107. Takada T, Aoki A, Asakawa K, Sakagami T, Moriyama H, Narita I, Sato S (2015) Serum cytokine profiles of patients with interstitial lung disease associated with anti-CADM-140/MDA5 antibody positive amyopathic dermatomyositis. Respir Med 109(9):1174–1180. https://doi.org/10.1016/j.rmed.2015.07.004

    Article  PubMed  Google Scholar 

  108. Fujisawa T, Hozumi H, Kono M, Enomoto N, Nakamura Y, Inui N, Nakashima R, Imura Y, Mimori T, Suda T (2017) Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases. Respir Investig 55(2):130–137. https://doi.org/10.1016/j.resinv.2016.09.006

    Article  PubMed  Google Scholar 

  109. Sclafani A, D'Silva KM, Little BP, Miloslavsky EM, Locascio JJ, Sharma A, Montesi SB (2019) Presentations and outcomes of interstitial lung disease and the anti-Ro52 autoantibody. Respir Res 20(1):256. https://doi.org/10.1186/s12931-019-1231-7

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  110. Fujisawa T, Hozumi H, Kono M, Enomoto N, Hashimoto D, Nakamura Y, Inui N, Yokomura K, Koshimizu N, Toyoshima M, Shirai T, Yasuda K, Hayakawa H, Suda T (2014) Prognostic factors for myositis-associated interstitial lung disease. PLoS ONE 9(6):e98824. https://doi.org/10.1371/journal.pone.0098824

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  111. Johnson C, Rosen P, Lloyd T, Horton M, Christopher-Stine L, Oddis CV, Mammen AL, Danoff SK (2017) Exploration of the MUC5B promoter variant and ILD risk in patients with autoimmune myositis. Respir Med 130:52–54. https://doi.org/10.1016/j.rmed.2017.07.010

    Article  PubMed  Google Scholar 

  112. Lopez-Mejias R, Remuzgo-Martinez S, Genre F, Pulito-Cueto V, Rozas SMF, Llorca J, Fernandez DI, Cuesta VMM, Ortego-Centeno N, Gomez NP, Mera-Varela A, Martinez-Barrio J, Lopez-Longo FJ, Mijares V, Lera-Gomez L, Usetti MP, Laporta R, Perez V, Gafas AP, Gonzalez MAA, Calvo-Alen J, Romero-Bueno F, Sanchez-Pernaute O, Nuno L, Bonilla G, Balsa A, Hernandez-Gonzalez F, Grafia I, Prieto-Gonzalez S, Narvaez J, Trallero-Araguas E, Selva-O'Callaghan A, Gualillo O, Castaneda S, Cavagna L, Cifrian JM, Gonzalez-Gay MA (2020) Influence of MUC5B gene on antisynthetase syndrome. Sci Rep 10(1):1415. https://doi.org/10.1038/s41598-020-58400-0

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  113. Marie I, Hachulla E, Cherin P, Dominique S, Hatron PY, Hellot MF, Devulder B, Herson S, Levesque H, Courtois H (2002) Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 47(6):614–622. https://doi.org/10.1002/art.10794

    Article  CAS  PubMed  Google Scholar 

  114. Takada K, Kishi J, Miyasaka N (2007) Step-up versus primary intensive approach to the treatment of interstitial pneumonia associated with dermatomyositis/polymyositis: a retrospective study. Mod Rheumatol 17(2):123–130. https://doi.org/10.1007/s10165-007-0553-3

    Article  PubMed  Google Scholar 

  115. Ge Y, Peng Q, Zhang S, Zhou H, Lu X, Wang G (2015) Cyclophosphamide treatment for idiopathic inflammatory myopathies and related interstitial lung disease: a systematic review. Clin Rheumatol 34(1):99–105. https://doi.org/10.1007/s10067-014-2803-z

    Article  PubMed  Google Scholar 

  116. Huapaya JA, Silhan L, Pinal-Fernandez I, Casal-Dominguez M, Johnson C, Albayda J, Paik JJ, Sanyal A, Mammen AL, Christopher-Stine L, Danoff SK (2019) Long-term treatment with azathioprine and mycophenolate mofetil for myositis-related interstitial lung disease. Chest 156(5):896–906. https://doi.org/10.1016/j.chest.2019.05.023

    Article  PubMed  Google Scholar 

  117. Andersson H, Sem M, Lund MB, Aalokken TM, Gunther A, Walle-Hansen R, Garen T, Molberg O (2015) Long-term experience with rituximab in anti-synthetase syndrome-related interstitial lung disease. Rheumatology (Oxford) 54(8):1420–1428. https://doi.org/10.1093/rheumatology/kev004

    Article  CAS  Google Scholar 

  118. Ge Y, Zhou H, Shi J, Ye B, Peng Q, Lu X, Wang G (2015) The efficacy of tacrolimus in patients with refractory dermatomyositis/polymyositis: a systematic review. Clin Rheumatol 34(12):2097–2103. https://doi.org/10.1007/s10067-015-3065-0

    Article  PubMed  Google Scholar 

  119. Cavagna L, Caporali R, Abdi-Ali L, Dore R, Meloni F, Montecucco C (2013) Cyclosporine in anti-Jo1-positive patients with corticosteroid-refractory interstitial lung disease. J Rheumatol 40(4):484–492. https://doi.org/10.3899/jrheum.121026

    Article  CAS  PubMed  Google Scholar 

  120. Suzuki Y, Hayakawa H, Miwa S, Shirai M, Fujii M, Gemma H, Suda T, Chida K (2009) Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung 187(3):201–206. https://doi.org/10.1007/s00408-009-9146-6

    Article  CAS  PubMed  Google Scholar 

  121. Chen Z, Wang X, Ye S (2019) Tofacitinib in Amyopathic Dermatomyositis-Associated Interstitial Lung Disease. N Engl J Med 381(3):291–293. https://doi.org/10.1056/NEJMc1900045

    Article  PubMed  Google Scholar 

  122. Ameye H, Ruttens D, Benveniste O, Verleden GM, Wuyts WA (2014) Is lung transplantation a valuable therapeutic option for patients with pulmonary polymyositis? Experiences from the Leuven transplant cohort. Transplant Proc 46(9):3147–3153. https://doi.org/10.1016/j.transproceed.2014.09.163

    Article  CAS  PubMed  Google Scholar 

  123. Watanabe M, Naniwa T, Hara M, Arakawa T, Maeda T (2010) Pulmonary manifestations in Sjogren's syndrome: correlation analysis between chest computed tomographic findings and clinical subsets with poor prognosis in 80 patients. J Rheumatol 37(2):365–373. https://doi.org/10.3899/jrheum.090507

    Article  PubMed  Google Scholar 

  124. Nannini C, Jebakumar AJ, Crowson CS, Ryu JH, Matteson EL (2013) Primary Sjogren's syndrome 1976–2005 and associated interstitial lung disease: a population-based study of incidence and mortality. BMJ Open 3(11):e003569. https://doi.org/10.1136/bmjopen-2013-003569

    Article  PubMed  PubMed Central  Google Scholar 

  125. Sambataro G, Ferro F, Orlandi M, Sambataro D, Torrisi SE, Quartuccio L, Vancheri C, Baldini C, Matucci Cerinic M (2020) Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjgren's syndrome: a systematic review from the Italian Society of Rheumatology. Autoimmun Rev 19(2):102447. https://doi.org/10.1016/j.autrev.2019.102447

    Article  PubMed  Google Scholar 

  126. Kurumagawa T, Kobayashi H, Motoyoshi K (2005) Potential involvement of subclinical Sjogren's syndrome in various lung diseases. Respirology 10(1):86–91. https://doi.org/10.1111/j.1440-1843.2005.00627.x

    Article  PubMed  Google Scholar 

  127. Constantopoulos SH, Papadimitriou CS, Moutsopoulos HM (1985) Respiratory manifestations in primary Sjogren's syndrome. A clinical, functional, and histologic study. Chest 88(2):226–229

    Article  CAS  Google Scholar 

  128. Papathanasiou MP, Constantopoulos SH, Tsampoulas C, Drosos AA, Moutsopoulos HM (1986) Reappraisal of respiratory abnormalities in primary and secondary Sjogren's syndrome. A controlled study. Chest 90(3):370–374

    Article  CAS  Google Scholar 

  129. Vitali C, Tavoni A, Viegi G, Begliomini E, Agnesi A, Bombardieri S (1985) Lung involvement in Sjogren's syndrome: a comparison between patients with primary and with secondary syndrome. Ann Rheum Dis 44(7):455–461

    Article  CAS  Google Scholar 

  130. Palm O, Garen T, Berge Enger T, Jensen JL, Lund MB, Aalokken TM, Gran JT (2013) Clinical pulmonary involvement in primary Sjogren's syndrome: prevalence, quality of life and mortality–a retrospective study based on registry data. Rheumatology (Oxford) 52(1):173–179. https://doi.org/10.1093/rheumatology/kes311

    Article  Google Scholar 

  131. Ramos-Casals M, Brito-Zeron P, Seror R, Bootsma H, Bowman SJ, Dorner T, Gottenberg JE, Mariette X, Theander E, Bombardieri S, De Vita S, Mandl T, Ng WF, Kruize A, Tzioufas A, Vitali C, Force ESST (2015) Characterization of systemic disease in primary Sjogren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology (Oxford) 54(12):2230–2238. https://doi.org/10.1093/rheumatology/kev200

    Article  Google Scholar 

  132. Buvry C, Cassagnes L, Tekath M, Artigues M, Pereira B, Rieu V, Le Guenno G, Tournadre A, Ruivard M, Grobost V (2020) Anti-Ro52 antibodies are a risk factor for interstitial lung disease in primary Sjogren syndrome. Respir Med 163:105895. https://doi.org/10.1016/j.rmed.2020.105895

    Article  PubMed  Google Scholar 

  133. Davidson BK, Kelly CA, Griffiths ID (2000) Ten year follow up of pulmonary function in patients with primary Sjogren's syndrome. Ann Rheum Dis 59(9):709–712

    Article  CAS  Google Scholar 

  134. Hatron PY, Wallaert B, Gosset D, Tonnel AB, Gosselin B, Voisin C, Devulder B (1987) Subclinical lung inflammation in primary Sjogren's syndrome: relationship between bronchoalveolar lavage cellular analysis findings and characteristics of the disease. Arthritis Rheum 30(11):1226–1231

    Article  CAS  Google Scholar 

  135. Dalavanga YA, Constantopoulos SH, Galanopoulou V, Zerva L, Moutsopoulos HM (1991) Alveolitis correlates with clinical pulmonary involvement in primary Sjogren's syndrome. Chest 99(6):1394–1397

    Article  CAS  Google Scholar 

  136. Dalavanga YA, Voulgari PV, Georgiadis AN, Leontaridi C, Katsenos S, Vassiliou M, Drosos AA, Constantopoulos SH (2006) Lymphocytic alveolitis: a surprising index of poor prognosis in patients with primary Sjogren's syndrome. Rheumatol Int 26(9):799–804. https://doi.org/10.1007/s00296-005-0092-1

    Article  CAS  PubMed  Google Scholar 

  137. Salaffi F, Manganelli P, Carotti M, Baldelli S, Blasetti P, Subiaco S, Binci MC, Bichi Secchi E, Amici F, Cervini C (1998) A longitudinal study of pulmonary involvement in primary Sjogren's syndrome: relationship between alveolitis and subsequent lung changes on high-resolution computed tomography. Br J Rheumatol 37(3):263–269

    Article  CAS  Google Scholar 

  138. Enomoto Y, Takemura T, Hagiwara E, Iwasawa T, Fukuda Y, Yanagawa N, Sakai F, Baba T, Nagaoka S, Ogura T (2013) Prognostic factors in interstitial lung disease associated with primary Sjogren's syndrome: a retrospective analysis of 33 pathologically-proven cases. PLoS ONE 8(9):e73774. https://doi.org/10.1371/journal.pone.0073774

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  139. Gottenberg JE, Cinquetti G, Larroche C, Combe B, Hachulla E, Meyer O, Pertuiset E, Kaplanski G, Chiche L, Berthelot JM, Gombert B, Goupille P, Marcelli C, Feuillet S, Leone J, Sibilia J, Zarnitsky C, Carli P, Rist S, Gaudin P, Salliot C, Piperno M, Deplas A, Breban M, Lequerre T, Richette P, Ghiringhelli C, Hamidou M, Ravaud P, Mariette X, Club Rhumatismes et I, The French Society of R (2013) Efficacy of rituximab in systemic manifestations of primary Sjogren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry. Ann Rheum Dis 72(6):1026–1031. https://doi.org/10.1136/annrheumdis-2012-202293

    Article  CAS  PubMed  Google Scholar 

  140. Parambil JG, Myers JL, Lindell RM, Matteson EL, Ryu JH (2006) Interstitial lung disease in primary Sjogren syndrome. Chest 130(5):1489–1495. https://doi.org/10.1378/chest.130.5.1489

    Article  PubMed  Google Scholar 

  141. Deheinzelin D, Capelozzi VL, Kairalla RA, Barbas Filho JV, Saldiva PH, de Carvalho CR (1996) Interstitial lung disease in primary Sjogren's syndrome. Clinical-pathological evaluation and response to treatment. Am J Respir Crit Care Med 154(3 Pt 1):794–799. https://doi.org/10.1164/ajrccm.154.3.8810621

    Article  CAS  PubMed  Google Scholar 

  142. Bertoli AM, Vila LM, Apte M, Fessler BJ, Bastian HM, Reveille JD, Alarcon GS, Group LS (2007) Systemic lupus erythematosus in a multiethnic US Cohort LUMINA XLVIII: factors predictive of pulmonary damage. Lupus 16(6):410–417. https://doi.org/10.1177/0961203307079042

    Article  Google Scholar 

  143. Nakano M, Hasegawa H, Takada T, Ito S, Muramatsu Y, Satoh M, Suzuki E, Gejyo F (2002) Pulmonary diffusion capacity in patients with systemic lupus erythematosus. Respirology 7(1):45–49

    Article  Google Scholar 

  144. Mittoo S, Fell CD (2014) Pulmonary manifestations of systemic lupus erythematosus. Semin Respir Crit Care Med 35(2):249–254. https://doi.org/10.1055/s-0034-1371537

    Article  PubMed  Google Scholar 

  145. Jacobsen S, Petersen J, Ullman S, Junker P, Voss A, Rasmussen JM, Tarp U, Poulsen LH, van Overeem HG, Skaarup B, Hansen TM, Podenphant J, Halberg P (1998) A multicentre study of 513 Danish patients with systemic lupus erythematosus. I. Disease manifestations and analyses of clinical subsets. Clin Rheumatol 17(6):468–477

    Article  CAS  Google Scholar 

  146. Lee SS, Singh S, Link K, Petri M (2008) High-sensitivity C-reactive protein as an associate of clinical subsets and organ damage in systemic lupus erythematosus. Semin Arthritis Rheum 38(1):41–54. https://doi.org/10.1016/j.semarthrit.2007.09.005

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  147. Matthay RA, Schwarz MI, Petty TL, Stanford RE, Gupta RC, Sahn SA, Steigerwald JC (1975) Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis. Medicine (Baltimore) 54(5):397–409. https://doi.org/10.1097/00005792-197509000-00003

    Article  CAS  Google Scholar 

  148. Stoll T, Seifert B, Isenberg DA (1996) SLICC/ACR Damage Index is valid, and renal and pulmonary organ scores are predictors of severe outcome in patients with systemic lupus erythematosus. Br J Rheumatol 35(3):248–254

    Article  CAS  Google Scholar 

  149. Weinrib L, Sharma OP, Quismorio FP Jr (1990) A long-term study of interstitial lung disease in systemic lupus erythematosus. Semin Arthritis Rheum 20(1):48–56

    Article  CAS  Google Scholar 

  150. Greidinger EL, Hoffman RW (2005) Autoantibodies in the pathogenesis of mixed connective tissue disease. Rheum Dis Clin North Am 31(3):437–450. https://doi.org/10.1016/j.rdc.2005.04.004

    Article  PubMed  Google Scholar 

  151. Gunnarsson R, Aalokken TM, Molberg O, Lund MB, Mynarek GK, Lexberg AS, Time K, Dhainaut AS, Bertelsen LT, Palm O, Irgens K, Becker-Merok A, Nordeide JL, Johnsen V, Pedersen S, Proven A, Garabet LS, Gran JT (2012) Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. Ann Rheum Dis 71(12):1966–1972. https://doi.org/10.1136/annrheumdis-2011-201253

    Article  PubMed  Google Scholar 

  152. Bodolay E, Szekanecz Z, Devenyi K, Galuska L, Csipo I, Vegh J, Garai I, Szegedi G (2005) Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD). Rheumatology (Oxford) 44(5):656–661. https://doi.org/10.1093/rheumatology/keh575

    Article  CAS  Google Scholar 

  153. Vegh J, Szilasi M, Soos G, Devenyi K, Dezso B, Soltesz P, Zeher M, Szegedi G, Bodolay E (2005) Interstitial lung disease in mixed connective tissue disease. Orv Hetil 146(48):2435–2443

    PubMed  Google Scholar 

  154. Kawano-Dourado L, Baldi BG, Kay FU, Dias OM, Gripp TE, Gomes PS, Fuller R, Caleiro MT, Kairalla RA, Carvalho CR (2015) Pulmonary involvement in long-term mixed connective tissue disease: functional trends and image findings after 10 years. Clin Exp Rheumatol 33(2):234–240

    PubMed  Google Scholar 

  155. Narula N, Narula T, Mira-Avendano I, Wang B, Abril A (2018) Interstitial lung disease in patients with mixed connective tissue disease: pilot study on predictors of lung involvement. Clin Exp Rheumatol 36(4):648–651

    PubMed  Google Scholar 

  156. Gunnarsson R, El-Hage F, Aalokken TM, Reiseter S, Lund MB, Garen T, Msg N, Molberg O (2016) Associations between anti-Ro52 antibodies and lung fibrosis in mixed connective tissue disease. Rheumatology (Oxford) 55(1):103–108. https://doi.org/10.1093/rheumatology/kev300

    Article  Google Scholar 

  157. Fagundes MN, Caleiro MT, Navarro-Rodriguez T, Baldi BG, Kavakama J, Salge JM, Kairalla R, Carvalho CR (2009) Esophageal involvement and interstitial lung disease in mixed connective tissue disease. Respir Med 103(6):854–860. https://doi.org/10.1016/j.rmed.2008.12.018

    Article  CAS  PubMed  Google Scholar 

  158. Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, Husain AN, Montner S, Chung JH, Cottin V, Fischer A, Noth I, Vij R, Strek ME (2016) Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J 47(6):1767–1775. https://doi.org/10.1183/13993003.01565-2015

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  159. Chartrand S, Swigris JJ, Stanchev L, Lee JS, Brown KK, Fischer A (2016) Clinical features and natural history of interstitial pneumonia with autoimmune features: A single center experience. Respir Med 119:150–154. https://doi.org/10.1016/j.rmed.2016.09.002

    Article  PubMed  Google Scholar 

  160. Ahmad K, Barba T, Gamondes D, Ginoux M, Khouatra C, Spagnolo P, Strek M, Thivolet-Bejui F, Traclet J, Cottin V (2017) Interstitial pneumonia with autoimmune features: Clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med 123:56–62. https://doi.org/10.1016/j.rmed.2016.10.017

    Article  PubMed  Google Scholar 

  161. Ito Y, Arita M, Kumagai S, Takei R, Noyama M, Tokioka F, Nishimura K, Koyama T, Notohara K, Ishida T (2017) Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. BMC Pulm Med 17(1):111. https://doi.org/10.1186/s12890-017-0453-z

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  162. Sambataro G, Sambataro D, Torrisi SE, Vancheri A, Colaci M, Pavone M, Pignataro F, Del Papa N, Palmucci S, Vancheri C (2019) Clinical, serological and radiological features of a prospective cohort of Interstitial Pneumonia with Autoimmune Features (IPAF) patients. Respir Med 150:154–160. https://doi.org/10.1016/j.rmed.2019.03.011

    Article  PubMed  Google Scholar 

  163. Sebastiani M, Cassone G, De Pasquale L, Cerri S, Della Casa G, Vacchi C, Luppi F, Salvarani C, Manfredi A (2020) Interstitial pneumonia with autoimmune features: a single center prospective follow-up study. Autoimmun Rev 19(2):102451. https://doi.org/10.1016/j.autrev.2019.102451

    Article  PubMed  Google Scholar 

  164. Sambataro G, Vancheri A, Torrisi SE, Colaci M, Pavone M, Libra A, Martorana E, Rosso R, Pignataro F, Del Papa N, Malatino L, Palmucci S, Sambataro D, Vancheri C (2020) The morphological domain does not affect the rate of progression to defined autoimmune diseases in patients with interstitial pneumonia with autoimmune features. Chest 157(1):238–242. https://doi.org/10.1016/j.chest.2019.08.2175

    Article  PubMed  Google Scholar 

  165. Fathi M, Vikgren J, Boijsen M, Tylen U, Jorfeldt L, Tornling G, Lundberg IE (2008) Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology. Arthritis Rheum 59(5):677–685. https://doi.org/10.1002/art.23571

    Article  PubMed  Google Scholar 

  166. Moore OA, Goh N, Corte T, Rouse H, Hennessy O, Thakkar V, Byron J, Sahhar J, Roddy J, Gabbay E, Youssef P, Nash P, Zochling J, Proudman SM, Stevens W, Nikpour M (2013) Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford) 52(1):155–160. https://doi.org/10.1093/rheumatology/kes289

    Article  Google Scholar 

  167. Christopher P, Denton CJFL, Goldin J, Kim G, Kuwana M, Allanore Y, Batalov A, Butrimiene I, Carreira P, Matucci-Cerinic M, Distler O, Kaliterna DM, Mihai C-M, Mogensen M, Olesińska M, Pope JE, Riemekasten G, Rodriguez-Reyne TS, Santos MJ, Van Laar J, Spotswood H, Siegel J, Jahreis A, Furst DE, Khanna D (2019) Lung function preservation in a phase 3 trial of tocilizumab (TCZ) in systemic sclerosis (SSc). Eur Respir J. https://doi.org/10.1183/13993003

    Article  Google Scholar 

  168. Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Samara K, Gilberg F, Cottin V (2020) Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med 8(2):147–157. https://doi.org/10.1016/S2213-2600(19)30341-8

    Article  CAS  PubMed  Google Scholar 

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  1. Loma Linda University Health, 11234 Anderson Street, Room 6424, Loma Linda, CA, 92354, USA

    Niranjan Jeganathan

  2. Department of Internal Medicine, Loma Linda University Health, Loma Linda, CA, 92354, USA

    Matheni Sathananthan

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Jeganathan, N., Sathananthan, M. Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment. Lung 198, 735–759 (2020). https://doi.org/10.1007/s00408-020-00383-w

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