Author/Editor		Rott, Tomaž; Vizjak, Alenka; Koselj, Mira
Title		Organ involvement in ANCA-associated vasculitides (biopsy and autopsy study)
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. 12-8
Language		eng
Abstract		The organ involvement in patients with different ANCA target antigens, such as are proteinase 3 (PR3), myeloperoxidase (MPO), and others, is compared. Therefore, 100 patients with kidney biopsies and verified ANCA-associated vasculitis from the period 1989-1999, were selected. Moreover, the progression and the outcome of disease were followed up to their death in 39 of 207 patients with presumed ANCA-associated vasculitis. Upper respiratory tract. lung cutaneous, eye and ear lesions were more common in patients with 27%, 8% and 0% in patients with antibodies against MPO. PR3 antigen specificity is more frequently associated with widespread extension of vasculitis, epistaxis and hemoptysis, whereas MPO antigen specificity with more frequent sclerotic glomerular lesions. Clinical and/or autopsy documentation revealed that 39 patients with vasculitic syndrome died mostly from infections or/and progressive or relapsing vasculitis (82%). In some patients autopsies revealed the real more extensive vasculitis, but in some cases, they also rejected diagnosis of putative vasculitis, with vague clinical features and with low values of ANCA. In such cases, treatment with harmful drugs should be avoided or seriously substantiated with undoubted evidence of vasculitis.
Descriptors		ANTIBODIES, ANTINEUTROPHIL CYTOPLASMIC VASCULITIS FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT BIOPSY AUTOPSY SLOVENIA KIDNEY DISEASES LUNG DISEASES