BACKGROUND: Pauci-immune renal vasculitis with focal glomerular necrosis and crescent formation is usually associated with anti-neutrophil cytoplasmic antibodies (ANCAs). However, ANCA's are absent in up to 10% of cases, which constitutes a rarely studied variant of renal vasculitis. METHODS: This retrospective multicentre cohort study analyzed the presenting features, renal histology and outcome in 20 patients with pauci-immune crescentic necrotizing renal vasculitis in whom indirect immunofluorescence did not detect ANCA. RESULTS: Renal histology revealed a high percentage of active glomerular lesions (50%), mainly cellular crescents, 28% of them with glomerular necrosis. Chronic tissue damage with glomerulosclerosis (21%) and diffuse interstitial fibrosis (40%) was already present at diagnosis, more prominent than in historical PR3-positive patients. Infiltrates of polymorphonuclear neutrophils in glomerular capillary loops were observed in 40% of all biopsies, mainly in necrotic lesions. The subsets of interstitially infiltrating leukocytes similar to ANCA-associated disease. Microscopic polyangiitis was diagnosed in 17 patients, Wegener's granulomatosis in two and renal-limited vasculitis in one. The patients median disease extent index (DEI) of 5 (range 4-11) reflected a systemic vasculitis. ANCA-negative vasculitis was not associated with infection or malignancy. Renal outcome was correlated to DEI (P = 0.032) and serum creatinine at diagnosis (P = 0.04). The mortality rate was high (35%) and closely related to age above 65 years at diagnosis (P = 0.014). Conclusions. The histological findings and prognosis in ANCA-negative renal vasculitis are comparable with those of ANCA-positive disease. Our data underline the importance of the exact diagnosis in an active vasculitic disease process even in the absence of ANCAs.
BACKGROUND: Pauci-immune renal vasculitis with focal glomerular necrosis and crescent formation is usually associated with anti-neutrophil cytoplasmic antibodies (ANCAs). However, ANCA's are absent in up to 10% of cases, which constitutes a rarely studied variant of renal vasculitis. METHODS: This retrospective multicentre cohort study analyzed the presenting features, renal histology and outcome in 20 patients with pauci-immune crescentic necrotizing renal vasculitis in whom indirect immunofluorescence did not detect ANCA. RESULTS: Renal histology revealed a high percentage of active glomerular lesions (50%), mainly cellular crescents, 28% of them with glomerular necrosis. Chronic tissue damage with glomerulosclerosis (21%) and diffuse interstitial fibrosis (40%) was already present at diagnosis, more prominent than in historical PR3-positive patients. Infiltrates of polymorphonuclear neutrophils in glomerular capillary loops were observed in 40% of all biopsies, mainly in necrotic lesions. The subsets of interstitially infiltrating leukocytes similar to ANCA-associated disease. Microscopic polyangiitis was diagnosed in 17 patients, Wegener's granulomatosis in two and renal-limited vasculitis in one. The patients median disease extent index (DEI) of 5 (range 4-11) reflected a systemic vasculitis. ANCA-negative vasculitis was not associated with infection or malignancy. Renal outcome was correlated to DEI (P = 0.032) and serum creatinine at diagnosis (P = 0.04). The mortality rate was high (35%) and closely related to age above 65 years at diagnosis (P = 0.014). Conclusions. The histological findings and prognosis in ANCA-negative renal vasculitis are comparable with those of ANCA-positive disease. Our data underline the importance of the exact diagnosis in an active vasculitic disease process even in the absence of ANCAs.
Authors: Sanjeev Sethi; Mark Haas; Glen S Markowitz; Vivette D D'Agati; Helmut G Rennke; J Charles Jennette; Ingeborg M Bajema; Charles E Alpers; Anthony Chang; Lynn D Cornell; Fernando G Cosio; Agnes B Fogo; Richard J Glassock; Sundaram Hariharan; Neeraja Kambham; Donna J Lager; Nelson Leung; Michael Mengel; Karl A Nath; Ian S Roberts; Brad H Rovin; Surya V Seshan; Richard J H Smith; Patrick D Walker; Christopher G Winearls; Gerald B Appel; Mariam P Alexander; Daniel C Cattran; Carmen Avila Casado; H Terence Cook; An S De Vriese; Jai Radhakrishnan; Lorraine C Racusen; Pierre Ronco; Fernando C Fervenza Journal: J Am Soc Nephrol Date: 2015-11-13 Impact factor: 10.121
Authors: Bernhard Hellmich; Oliver Flossmann; Wolfgang L Gross; Paul Bacon; Jan Willem Cohen-Tervaert; Loic Guillevin; David Jayne; Alfred Mahr; Peter A Merkel; Heiner Raspe; David G I Scott; James Witter; Hasan Yazici; Raashid A Luqmani Journal: Ann Rheum Dis Date: 2006-12-14 Impact factor: 19.103
Authors: Duvuru Geetha; Caroline J Poulton; Yichun Hu; Philip Seo; Julie Anne G McGregor; Ronald J Falk; Susan L Hogan Journal: Semin Arthritis Rheum Date: 2013-12-04 Impact factor: 5.532
Authors: Andrea Peschel; Neil Basu; Alexandra Benharkou; Ricarda Brandes; Markus Brown; Andrew J Rees; Renate Kain Journal: J Am Soc Nephrol Date: 2013-11-07 Impact factor: 10.121