Features of diagnostic search in Churg–Strauss syndrome. Case report

Abstract

Churg–Strauss syndrome is a rare systemic vasculitis affecting small-caliber vessels, which has characteristic clinical manifestations in the forms of allergic rhinosinusitis, bronchial asthma (BA), eosinophilia, and pulmonary infiltrates. The reason for late diagnostics is gradual manifestation of symptoms, that typical of systemic diseases. Improving the knowledge of pediatricians, pulmonologists about the role of diagnostic search in Churg–Strauss syndrome for further routing, monitoring and correction of patient therapy. A 17-year-old boy with BA was admitted to the pulmonology department for clarification, verification of the diagnosis, and selection of therapy. Instrumental and laboratory examination was carried out: on the computer tomography (CT) scan of the thoracic organs "migrating" infiltration of both lungs, based on laboratory data may correspond to eosinophilic zones. On the CT scan of the nasal sinuses polysinusitis, polyposis; external respiration function (spirography) – no pathology; myeloperoxidase-antineutrophil cytoplasmic antibodies (ANCA) – 14 U/ml (normal 5), anti-proteinase 3 anti-neutrophil ANCA – 30 U/ml (normal 5), anti-Saccharomyces cerevisiae antibodies – 6.3–7.2 U/ml, eosinophilia (12%, abs 840 c). The result of the level of antibodies was negative later. Churg–Strauss syndrome was based on the American College of Rheumatology criteria: BA, eosinophilia 10% (abs 840), rhinosinusitis, migrating infiltrations on CT scan. He was discharged for asthma combination therapy with high age doses of inhaled corticosteroids. After treatment his condition improved, there was no need for systemic corticosteroids. The described clinical case clearly demonstrates the difficulty and duration of diagnosing Churg–Strauss syndrome due to the stage-by-stage course of the disease, the frequent (33% of cases) absence of laboratory evidence of vasculitis (ANCA-neg.) significant difficulty of verifying the diagnosis at early stages. The diagnosis provides for a comprehensive and interdisciplinary approach to diagnosis and treatment.