Summary
A 28-year-old male patient suffering from Wegener's granulomatosis died suddenly with signs of cardiac failure after clinical symptoms had basically subsided under chemotherapy. Autopsy revealed pulmonary granulomata, necrotizing vasculitis of the lungs and kidneys, focal and segmental necrotizing glomerulonephritis, and diffuse granulomatous and necrotizing giant cell myocarditis.
Histological confirmation of inflammation of the heart in Wegener's disease has rarely been reported. Although cardiac involvement in Wegener's granulomatosis sometimes is suspected, it is usually thought to have no major impact on the course of the disease.
By its dramatic clinical and morphologic presentation this case illustrates that the heart, in addition to the lungs and kidneys, may determine the outcome of the idiopathic granulomatous vasculitis of Wegener.
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References
Allen DC, Doherty CC, Reilly DPJO (1984) Pathology of the heart and the cardiac conduction system in Wegener's granulomatosis. Br Heart J 52:674–678
Andrassy K, Koderisch J, Rasmussen N, Ritz E (1989) Diagnostische Bedeutung antineutrophiler zytoplasmatischer Antikörper bei Wegenerscher Granulomatose und verwandten Krankheitsbildern. Dtsch med Wochenschr 114:23–26
Beigel A, Lehmann H, Müller-Hermelink KA (1980) Die Wegenersche Granulomatose. Dtsch Ärztebl 51/52:3009–3012
Budzilovich GN, Wilens SL (1960) Fulminating Wegener's granulomatose. Arch Pathol 70:653–660
Cain H (1981) Granulome und Granulomatosen. Pathologe 2:65–71
Carrington CB, Liebow AA (1966) Limited forms of angiitis and granulomatosis of Wegener's type. Am J Med 41:497–527
Dabbagh S, Chevalier R, Sturgill B (1982) Prolonged anuria and aortic insufficiency in a child with Wegener's granulomatosis. Clin Nephrol 17:155–159
Doerr W (1974) Entzündliche Veränderungen des Myokard. In: Doerr W (eds) Herz und Gefäße, Bd I, Georg Thieme, Stuttgart, pp 34–40
Fauci AS, Haynes BK, Katz P, Wolff SM (1983) Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Int Med 98:76–85
Forstot JZ, Overlie PA, Neufeld GK, Harmon CE, Forstot SL (1980) Cardiac complications of Wegener granulomatosis: a case report of complete heart block and review of the literature. Semin Arthritis Rheum 10:148–154
Godman GC, Churg J (1954) Wegener's granulomatosis. Pathology and review of the literature. Arch Pathol 58:533–553
Gröne H-J, Osborn M, Gross WL (1989) Die entzündlichen Gefäßerkrankungen der Niere. Verh Dtsch Ges Path 73:181–192
Gross WL, Lehmann H, Beigel A, Dunker A (1986) ACPA: Marker der Wegener'schen Granulomatose? Dtsch Med Wochenschr 111:1499–1500
Hornig D, Kühn H (1984) Beitrag zur „limitierten“ Wegenerschen Granulomatose beim Kind mit primärem Befall der Lunge. Pathologe 5:134–142
Klinger H (1931) Grenzformen der Periarteriitis nodosa. Frankfurter Z Pathol 42:455–480
Küstner W, Domarus HV, Wegener F (1987) Frühsymptome und Fehldiagnosen bei neun Fällen von Wegenerscher Granulomatose. Therapiewoche 28:1112–1116
Levine H, Madden TJ (1957) Wegener's granulomatose. Report of a case. Am Heart J 53:632–637
McCrea PC, Childers RW (1964) Two unusual cases of giant cell myocarditis associated with mitral stenosis and with Wegener's syndrome. Br Heart J 26:490–498
Moritz AR, Zamcheck N (1946) Sudden and unsuspected deaths of young soldiers. Arch Pathol 42:459–494
Nölle B, Specks U, Lüdermann J, Rohrbach MS, de Remee RA, Gross WL (1989) Anticytoplasmic autoantibbodies: their immunodiagnostic value in wegener granulomatosis. Ann Int Med 111:28–40
Schiavone WA, Ahmad M, Ockner SA (1985) Unusual cardiac complications of Wegener's granulomatosis. Chest 88:745–748
Shah IA, Holstege A, Riede UN (1984) Bioptic diagnosis of Wegener's granulomatosis in the absence of vasculitis and granulomas. Path Res Pract 178:407–412
Van der Woude FJ, Rasmussen FJN, Lobatto S, Wiik A, Permin H, van Es LA, van der Giessen M, van der Hem GK, The TH (1985) Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet 1:425–429
Wegener F (1939) Über eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren. Beitr Pathol Anat 102:37–68
Wegener F (1969) Die pneumogene allgemeine Granulomatosesog. Wegenersche Granulomatose. In: Kaufmann E, Staemmler M (eds) Lehrb spez path Anat, Bd I/1. Hälfte. W. de Gryter, Berlin, pp 225–299
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Weidhase, A., Gröne, H.J., Unterberg, C. et al. Severe granulomatous giant cell myocarditis in Wegener's granulomatosis. Klin Wochenschr 68, 880–885 (1990). https://doi.org/10.1007/BF01662788
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DOI: https://doi.org/10.1007/BF01662788