Cholesterol embolism is a multisystemic disorder with clinical manifestations that resemble vasculitis. Anti-neutrophil cytoplasmic antibodies (ANCA) are a defining feature of ANCA-associated vasculitis, and the presence of ANCA in cholesterol embolism complicates its differential diagnosis and treatment. At present, the role of ANCA in cholesterol embolism remains unclear and no effective treatment is currently available. The present study reports the case of an Asian male who presented with spontaneous cholesterol embolism with proteinase 3 (PR3)-specific ANCA, subacute interstitial nephritis and late-developing skin lesions. The 69-year-old patient was admitted to The First Affiliated Hospital of Xiamen University (Xiamen, China) complaining of chest tightness, fatigue, progressive renal failure and refractory hypertension. In addition, transient eosinophilia was detected. Following immunosuppressive therapy with steroids and cyclophosphamide for 6 months, hemodialysis treatment was initiated. Skin lesions appeared at >1 month following hemodialysis initiation; however, they were gradually improved following treatment with atorvastatin and anti-platelet aggregation therapy for 5 months. The patient was maintained on hemodialysis for ~2 years and exhibited general good health at the most recent follow-up. In addition, 11 cases of cholesterol embolism associated with ANCA reported in the literature were discussed in the present study.
Keywords: antineutrophil cytoplasmic antibodies; cholesterol embolism; cyclophosphamide; renal disease; steroids.