[The Churg-Strauss syndrome with rapidly progressive glomerulonephritis positive for antineutrophil cytoplasmic antibodies].
Renal failure developed in a patient treated for worsening spastic dyspnoea, high erythrocyte sedimentation rate and enlarged peribronchial lymph nodes by the antituberculotic regimen. Renal biopsy disclosed rapidly progressive glomerulonephritis with 95% crescents, granulomatous periglomerulonephritis vasculitis and eosinophilic interstitial infiltrates. On the basis of the positivity of antineutrophil cytoplasmic antibodies (ANCA), eosinophilia and profound ventilatory impairment the diagnosis of Churg-Strauss syndrome was established. The patient was treated by plasma exchanges and combined immunosuppression with the profound effect on erythrocyte sedimentation rate, eosinophilia a negativization of ANCA and preservation of at least minimal renal function. Further therapy was complicated by steroid diabetes, repeated leucopenia and exacerbation of spastic bronchitis and eventually by the massive gastrointestinal haemorrhage from asymptomatic gastric ulcer. There were no signs of inflammation in renal autopsy specimens with prevailing glomerulosclerosis a periglomerular fibrosis. Renal impairment is rare in Churg-Strauss syndrome and it is only exceptionally the cause of renal failure.
Tesar
V
V
Interní klinika 1. LF UK, Praha.
Rychlík
I
I
Bartůnková
J
J
Stejskalová
A
A
Honsová
E
E
Lachmanová
J
J
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies / Q000032:analysis*; D015267:Churg-Strauss Syndrome / Q000150:complications* / Q000276:immunology; D005921:Glomerulonephritis / Q000150:complications*; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis*; D008297:Male; D008875:Middle Aged
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D007074:Immunoglobulin G
Churg-Straussův syndrom s rychle progredující glomerulonefrítídou a pozitívnímí protilátkami proti cytoplazmĕ neutrofilních leukocytů (P-ANCA).