Details

Autor(en) / Beteiligte

• McAdoo, Stephen P
• Tanna, Anisha
• Randone, Olga
• Tam, Frederick W K
• Tarzi, Ruth M
• Levy, Jeremy B
• Griffith, Megan
• Lightstone, Liz
• Cook, H Terence
• Cairns, Tom
• Pusey, Charles D

Titel

Necrotizing and crescentic glomerulonephritis presenting with preserved renal function in patients with underlying multisystem autoimmune disease: a retrospective case series

Ist Teil von

• Rheumatology (Oxford, England), 2015-06, Vol.54 (6), p.1025-1032

Ort / Verlag

England: Oxford University Press

Erscheinungsjahr

2015

Link zum Volltext

Quelle

Oxford Journals 2020 Medicine

Beschreibungen/Notizen

• Necrotizing and crescentic GN usually presents with rapidly declining renal function, often in association with multisystem autoimmune disease, with a poor outcome if left untreated. We aimed to describe the features of patients who have presented with these histopathological findings but minimal disturbance of renal function. We conducted a retrospective review (1995-2011) of all adult patients with native renal biopsy-proven necrotizing or crescentic GN and normal serum creatinine (<120 μmol/l) at our centre. Thirty-eight patients were identified. The median creatinine at presentation was 84 μmol/l and the median proportion of glomeruli affected by necrosis or crescents was 32%. Clinicopathological diagnoses were ANCA-associated GN (74%), LN (18%), anti-GBM disease (5%) and HScP (3%). Only 18% of cases had pre-existing diagnoses of underlying multisystem autoimmune disease, although the majority (89%) had extra-renal manifestations accompanying the renal diagnosis. All patients received immunosuppression and most had good long-term renal outcomes (median duration of follow-up 50 months), although two progressed to end-stage renal disease within 3 years. We estimate that renal biopsy had an important influence on treatment decisions in 82% of cases. Necrotizing and crescentic GN may present in patients with no or only minor disturbance of renal function. This often occurs in patients with underlying systemic autoimmune disease; early referral for biopsy may affect management and improve long-term outcomes in these cases.