The Principles of Diagnosis of Churg-Strauss Syndrome, in the Authors’ Experience

Background: The diagnosis of Churg-Strauss syndrome (CSS) poses considerable diagnostic difficulties as the histological confirmation of eosinophilic necrotising vasculitis is obtained in a small number of patients with advanced disease. It therefore seems that CSS may be suspected in patients with asthma and peripheral blood eosinophilia, and the diagnosis is confirmed by the occurrence of defined clinical manifestations of histologically confirmed or unconfirmed vasculitis. The aim of the paper was to outline the principles of diagnosis of CSS in the authors’ material. Material and methods: We analysed 38 patients. The assessments performed in each patient included complete and differential blood counts, blood biochemistry, urinalysis, chest and paranasal sinus radiographic imaging, and echocardiography. Twenty-two out of 23 patients presenting with cardiac manifestations underwent cardiac magnetic resonance imaging (MRI). Two patients underwent mediastinoscopy and four underwent laparotomy. Results: Only in 13 out of 38 patients was the diagnosis of CSS was confirmed histologically, and in the remaining patients the diagnosis was based on clinical manifestations. In 23 patients, the diagnosis was based on cardiac manifestations associated with the involvement of the myocardium by inflammation, which was documented by MRI. In 9 cases, the diagnostic evaluation was prompted by cutaneous changes. Six patients presented with gastrointestinal complaints, 15 patients suffered from peroneal nerve palsy, and one from polyneuropathy and central nervous system symptoms. Conclusions: The diagnosis of CCS in our material was mainly based on clinical manifestations. The collection of material for histopathology was difficult and required an invasive approach in most cases. In 13 out of 38 patients eosinophilic vasculitis was confirmed histologically. The development of marked peripheral blood eosinophilia in an asthmatic patient with a history of allergy, sinusitis, and pulmonary infiltrates in the presence of specific organ manifestations was most consistent with CSS. Indirect proof came in the form of the meeting of the classification criteria developed by the American College of Rheumatology, which differentiated CSS from other vasculitides.