An unusual presentation of eosinophilic granulomatosis with polyangiitis with a good response to rituximab

Abstract

IntroductionEosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis that belongs to the anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. It usually presents with late onset asthma in association with ear, nose and throat, cardiac, lung and gastrointestinal involvement. Aim of the workTo present a case of EGPA man with an unusual presentation. Case reportA 24 years old patient with EGPA presented with deep vein thrombosis and nerve palsy without a history of asthma or other usual manifestations of the disease. The left leg showed a skin rash and associated deep vein thrombosis as well as a small ulcer over the pulp of the right thumb. The total leucocyte count was 14.5 × 109/L (41% eosinophils), erythrocyte sedimentation rate was 30 mm/1st hour and C-reactive protein 12.2 mg/L. Hepatitis B and C serology, antinuclear antibody (ANA) and antiphospholipid antibodies were all negative. ANCA was positive: myeloperoxidase (MPO) 36.9 U/ml and proteinase-3 (PR3) 15.9 U/ml and urinalysis was normal. The dermal and subcutaneous blood vessels displayed fibrinoid deposits, necrosis, and neutrophilic infiltrates in keeping with vasculitis. The patient showed a prompt and good response to corticosteroid and rituximab therapy with resolution of his symptoms. ConclusionThere is a necessity to keep an open eye for the diagnosis of EGPA in patients with eosinophilia even in the absence of asthma or other usual manifestations, as prompt therapy may be organ and life-saving. B cell therapy should be considered in patients resistant to conventional therapy and those in whom preservation of fertility is desired.