A 47-year-old female patient with active Systemic lupus erythematosus (SLE) - skin, joint, hematological, and renal involvement was admitted for rapidly progressive renal failure at the background of very high anti-dsDNA and pANCA antibodies. Active pathogenetic treatment was started pulse therapy with methylprednisolone, intravenous immunoglobulins (IVIG) and cyclophosphamide, blood transfusions were performed, symptomatic treatment with infusions ad diuretic was initiated. Due to progression of renal failure with volume overload and increase in urea and creatinine levels renal-replacement therapy was initiated. The patient developed E. faecalis and E. durans sepsis with disseminated intravascular coagulation and ground-glass-type lung changes and the condition further deteriorated to fatal multiorgan failure. The authors discuss the role of pANCA antibodies in SLE with renal and lung involvement.
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