Churg-Strauss Syndrome and kidney

Abstract

CSS diagnosis is difficult since none of neither clinical nor histopathological elements are characterologic symptoms of this disease. Aims: To describe clinical diagnosis criteria for Churg-Strauss Syndrome in one female patient; to identify the affected organs and to describe the treatment and physiopathology of this disease. It is about a patient of 32 years old admitted to hospital presenting dyspnea, syncopal signs and asthma. At the moment of physical examination she presented Livedo Reticularis, peripheral edemas, recent hypertension and weakness and hypesthesia in both legs. Laboratory results showed pronounced eosinophilia, subnephrotic proteinuria, hematuria, leukocyturia, ANCAs (-) and negative parasitological feces analysis. Echocardiogram and Doppler echocardiography presented serious systolic function and mural thrombus decline in ventricles, skin leukocytoclastic vasculitis, multiple mononeuritis and pauci-immune crescentic glomerulonephritis. The presence of leukocytoclastic vasculitis, crescentic glomerulonephritis together with asthma association, eosinophilia, heart failure and multiple mononeuritis have led us to diagnose Churg-Strauss Syndrome. The patient was treated with Methylprednisolone pulse therapy (1g/day for 3 days), then 1mg/kg/day oral dose and a final dose tapering. At the beginning coagulation was prevented by heparin and then with Acenocoumarol. Enalapril was also indicated. We concluded that the patient presented a Churg-Strauss Syndrome with clear clinical elements, in spite of not having histopathological parameters such as necrosis/infiltrate, eosinophilic perivascular granuloma. Concerning renal damage, she presented a pauci-immune crescentic glomerulonephritis ANCAs (-).