Incidence and outcome of pauci‐immune rapidly progressive glomerulonephritis in Wessex, UK: a 10‐year retrospective study

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Abstract
Background. The Wessex Renal Unit serves a large stable population (2.5 million). Pauci‐immune rapidly progressive glomerulonephritis (RPGN) is a frequent cause of acute renal failure requiring admission to our unit (∼8%). At a population level, little is known of the epidemiology and outcome of RPGN. Methods. Between 1 April 1986 and 31 March 1996, 141 cases of biopsy proven pauci‐immune RPGN were seen in the Wessex region. The records of 128 patients were reviewed. Median (range) follow‐up was 1.8 (0.9–9.64) years from diagnosis. Results. The incidence of 4 per million was stable throughout the period. No clustering was seen. The diagnosis was made (median, 25th, 75th centile) 78.5 (45, 166) days after symptom onset. Co‐morbidity (mostly hypertension) was seen in 47% of patients. Other organs affected were lungs 63%, nose/sinuses 50%, joints 42%, muscle 33%, skin 22% and nervous system 14%. Anti‐neutrophil cytoplasmic antibody (ANCA) was positive in 73%; cytoplasmic ANCA 34%, peri‐nuclear ANCA 26% and undifferentiated 14%. Twenty‐seven per cent tested ANCA negative. The differences between the groups were small; time to diagnosis was shorter in the ANCA negative (−ve) group (P=0.02) and there were more airway symptoms in the ANCA positive (+ve) group (PP=0.0004) and age (P=0.004) were poor prognostic markers. Ten per cent were transplanted, graft survival was 90% at 1 year, no recurrence was seen. Conclusions. This study, looking at a large cohort, has established the incidence and outcome of ANCA +ve and ANCA −ve RPGN in a defined stable population. It stresses the similarities between ANCA +ve and ANCA −ve cases and supports the notion that pauci‐immune RPGN is part of a continuum of vasculitic illness. In this series transplantation is a safe option.