[A case of rapidly progressive glomerulonephritis in the course of Wegener's granulomatosis].
Wegener's granulomatosis (WG) is characterized by granulomatous vasculitis of the respiratory tract and glomerulonephritis (GN). Prognosis of this disease is poor and about 20% of untreated patients die after one year from the onset. WG was recognized in 45-year-old patient on the basis of: 1) clinical symptoms (joint pain and swollen, purpura on the skin which appeared one week after respiratory tract infection, ulceration of the tonsils and lingula), 2) results of additional testing (X-chest-ray-infiltrates of both lungs), positive results of the cANCA (titre 1:640) and rapidly progressive renal failure [the increase of serum creatinine level (Pcr) from 123.7 to 707 mumol/l (1.4 to 8.0 mg/dl) during one week]. Renal biopsy revealed extracapillary GN (cellular crescents in 7 out of 8 glomeruli and scattered foci of fibrinoid necrosis of capillary walls in all). At the beginning of the treatment Pcr raised to 884 mumol/l (10 mg/dl) and the patient required hemodialysis. He was treated with methylprednisolone (M) at flash doses of 1000 mg/24 h by three days followed by 125 mg/24 h i.v.--because of peptic ulcer, with cyclophosphamide (C-150 mg/24 h p.p.), with trimetoprim/sulphametoxazole, with pentoxifylline and omeprazol. After six weeks of the treatment in the control kidney biopsy sclerotic changes in 10 out of 13 glomeruli and diffuse interstitial fibrosis were found. However, during the same time, we observed clinical remission of the disease and the decrease of Pcr to 176.8 mumol/l (2 mg/dl). The M dosis was reduced by 5 mg every weeks and the C dosis--to 50 mg (because of the increase of aminotransferase levels) After six months of the treatment Pcr was 132.6 mumol/l (1.5 mg/dl) and CANCA titer was 1:16. In this case of RPGN, despite off the progression of the morphological changes in the kidney, we obtained the clinical remission of the disease and significant decrease of Pcr level. These results suggest that aggressive treatment of WG is justified even in patients with advanced renal failure requiring dialysis and in such patients clinical remission is possible to occur.
Klinika Nefrologii Akademii Medycznej w Poznaniu.
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Idasiak-Piechocka
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Oko
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Łochyńska
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Woźniak
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Czekalski
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D001706:Biopsy; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005921:Glomerulonephritis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007668:Kidney / Q000473:pathology; D008168:Lung / Q000000981:diagnostic imaging; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012074:Remission Induction; D006435:Renal Dialysis
D019268:Antibodies, Antineutrophil Cytoplasmic