ЭОЗИНОФИЛЬНЫЙ ГРАНУЛЕМАТОЗ С ПОЛИАНГИИТОМ

Abstract

Introduction. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as allergic granulomatosis (Churg-Stross syndrome), in the modern sense is a necrotizing multisystem vasculitis of predominantly small vessels associated with hyperproduction of antineutrophil cytoplasmic antibodies (ANCA), peripheral blood hypereosinophilia. Although the most frequently involved are the respiratory organs (rhinosinusitis, bronchial asthma, pulmonary infiltrates), EGPA affects any organs and systems (heart, kidneys, gastrointestinal tract, nervous system, skin, joints, etc.) which makes its diagnosis difficult. Aim. To present an observation of a difficult-to-diagnose clinical case of eosinophilic granulomatosis with polyangiitis. Materials and methods. The analysis of clinical, laboratory, special testing of the patient was carried out. Results. Diagnosis of EGPA in this patient was difficult. Fever up to 40°C, papulo-necrotic skin eruptions on upper and lower extremities, trunk; weight loss; multiple organ involvement of heart, lungs, kidneys, liver; positive hemoculture with isolation of Staph. haemoliticus, and from skin lesions of Staph. epidermidis – all these served as a reason to suspect staphylococcal sepsis with possible infectious endocarditis and prescribe antimicrobial therapy which was ineffective. Progression of the disease with worsening clinical manifestations, detection of blood eosinophilia (18%), positive ANCA on immunological examination, eosinophilic and lymphohistiocytic infiltration around microvessels on histological examination of the musculocutaneous flap allowed the diagnosis of EGPA to be verified. Pulse chemotherapy with cyclophosphamide and medium doses of methylprednisolone led to regression of the disease manifestations in the patient. Conclusion. The course of EGPA can include life-threatening conditions (acute myocardial infarction and pulmonary hemorrhage). Timely diagnosis and standards of active chemotherapy expand possibilities of managing this severe group of patients.