Author/Editor		Kralj, E; Vizjak, A; Strokol, H; Krivec, B; Žuran, I
Title		Fulminant Wegener's granulomatosis with pulmonary capillaritis and diffuse alveolar hemorrhage: case report
Type		članek
Source		In: Vizjak A, Rozman B, Ferluga D, editors. Autoimmune systemic diseases. Proceedings of the 30th memorial meeting for professor Janez Plečnik; 1999 Dec 2-3; Ljubljana. Ljubljana: Faculty of medicine, Institute of pathology,
Publication year		1999
Volume		str. 25-9
Language		eng
Abstract		Clinical manifestations of Wegener's granulomatosis (WG) are very heterogenous, and frequently nonspecific and depend on distribution and extensiveness of vasculitis in organs. WG became more definable with discovery of antineutrophil cytoplasmic antibodies (ANCA). We report a case of fulminant WG with a shor 1/month course, very nonspecific symptomatology of arthralgias and unexpected sudden worsening with severe affliction of lungs resulting in death. Histomorphological examination of autoptic tissue samples revealed diffuse, massive alveolar hemorrhage with leukocytoclastic necrotizing pulmonary capillaritis. Peculiarity of the presented case is extremely severe diffuse involvement of lungs alongside with extremely sparse vascular changes in some other organs. Histomorphological criteria for diagnosis of WG were actually fulfilled, yet the exceptionality of this case lies in the fact that the granulomatous reaction was found in only one sample of lung tissue, necrotizing mixed cellular small-vessel vasculitis was present in only scarce small arteries and arterioles in the appendix, duodenum and spleen and small veins in lungs. Necrotizing crescentic glomerulonephritis involved only 5 out of 150 renal corpuscles. The diagnosis of WG was confirmed by C-ANCA positive for proteinase 3.
Descriptors		WEGENER'S GRANULOMATOSIS ANTIBODIES, ANTINEUTROPHIL CYTOPLASMIC HEMORRHAGE RESPIRATORY TRACT DISEASES IMMUNOHISTOCHEMISTRY FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT SLOVENIA VASCULITIS