RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS WI TH SEVERE PERIPHERAL NEUROPATHY AT PRESENTATION

We describe one case of rapidly progressive glomerulonephritis whose i nitial clinical presentation was dominated by severe involvement of th e peripheral nervous system. Multiple mononeuropathy predominantly aff ecting the right median nerve and both right and left external sciatic nerves as well as both common peroneal nerves was documented on elect romyography. A moderate normochromic, normocytic anaemia (Hb 8,9 g/dl) was detected with an ESR of 132 mm and high platelet (600.000) and WB C counts. Serum urea (U) and creatinine (Cr) were elevated at 34 mmol/ L and 550 mu mol/L respectively. A 24 hour urine collection revealed a proteinuria of 3 g. The renal ultrasound (U/S) showed two normal size d kidneys and a renal biopsy was performed. Light microscopy revealed a necrotizing focal segmental glomerulonephritis with a severe interst itial nephritis (fig. 2). Fresh cellular crescents were present in 90 % of the glomeruli. Two were obsolete. No immunoglobulins were detecte d on the immunofluorescence. The Antineutrophil Cytoplasmic Antibody ( ANCA) was positive (MPO - ELISA = 130 UE) with p-ANCA pattern on the i mmunofluorescence (fig. 1). Therapy was started with methylprednisolon e (1 g/day, iv, for 3 days) and plasmapheresis (40 ml/kg of body weigh t, for 6 days), followed by oral cyclophosphamide (3 mg/Kg of BW) and prednisolone (40 mg/day). Recovery of renal function was initiated by the second session of PE. After a follow-up of 6 months the patient is on azathioprine 3 mg/day, prednisolone (60 mg/day) and immunoglobulin 400 mg/kg BW iv every 4 weeks and now has a Cr 154 mu mol/L (ClCr-38 ml/min) and no significant proteinuria. The ANCA titers progressively decreased and are now undetectable.