Acute renal failure due to rapidly progressive glomerulonephritis: An uncommon manifestation of primary Sjogren's syndrome

BACKGROUND: Renal impairment, usually due to tubulointerstitial and rarely glomerular disorders occurs in 10 to 30 % of patients with primary Sjogren' s syndrome. Extracapillary proliferation may also be observed. CASE REPORT: A 62-year-old woman with primary Sjogren's syndrome diagnosed 12 years earlier, developed microscopic polyangeitis leading to rapidly pro gressive renal failure. Antipolynuclear anticyclosine antibody (ANCA) serol ogy was positive (pANCA, anti MPO) and the renal biopsy evidenced necrotizi ng glomerulonephrities with extracapillary proliferation. Outcome was favor able despite a recurrence one year after onset. DISCUSSION: Extracapillary proliferative glomerulonephritis is characterize d by hematuria and proteinuria associated with renal failure. Renal impairm ent may worsen rapidly, sometimes leading to an emergency situation because the renal prognosis is directly related to delay to treatment This case il lustrates an uncommon complication of Sjogren's syndrome, compared with the usual tubulointerstitial disorders. The mechanism remains unknown, but out come can be favorable with rapidly initiated immunosuppressor treatment. (C ) 2000, Masson, Paris.