Increased production of IL-5 and dominant Th2-type response in airways of Churg–Strauss syndrome patients

• Published in: Rheumatology (Oxford, England) Vol. 51; no. 10; pp. 1887 - 1893
• Main Authors: Jakiela, Bogdan, Szczeklik, Wojciech, Plutecka, Hanna, Sokolowska, Barbara, Mastalerz, Lucyna, Sanak, Marek, Bazan-Socha, Stanislawa, Szczeklik, Andrzej, Musial, Jacek
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.10.2012
• Subjects: Adult; Biological and medical sciences; Bronchoalveolar Lavage Fluid - chemistry; Bronchoalveolar Lavage Fluid - immunology; Chemokine CCL17 - analysis; Chemokine CCL22 - analysis; Chemokine CCL26; Chemokines, CC - analysis; Churg-Strauss Syndrome - immunology; Churg-Strauss Syndrome - metabolism; Diseases of the osteoarticular system; Eosinophils - immunology; Female; Gene Expression - immunology; Humans; Interleukin-5 - analysis; Male; Medical sciences; Middle Aged; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Human; Granulomatous vasculitis; Rheumatology; Churg and Strauss syndrome; Th2 lymphocytes; Cardiovascular disease; chemokines; interleukin-5; Vascular disease; Chemokine; Respiratory tract; Churg-Strauss syndrome; Interleukin 5; Immunological investigation; Bronchoalveolar lavage; T-Lymphocyte; Production; bronchoalveolar lavage fluid; Th2 lymphocyte
• ISSN: 1462-0324; 1462-0332; 1462-0332
• DOI: 10.1093/rheumatology/kes171

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis associated with eosinophilia and asthma. We assessed the local immune response in airways of CSS patients with different activity of the disease. Concentration of IL-5, CCL17, CCL22 and CCL26 (ELISA) together with cell expression of T-helper-related genes (real-time PCR array) were measured in bronchoalveolar lavage fluid (BALF) sampled from 11 patients with active CSS, 11 patients with CSS in remission and 9 control subjects with bronchial asthma. In active CSS, both BALF and blood eosinophil counts were increased (P<0.01). BALF cells in active disease were characterized by an increased expression of Th2 and regulatory-type transcripts: STAT6, STAT3, GATA3, IL4, IL5 and IL10 as compared with asthmatics, and STAT5A, CCR4, FOXP3, IL4, IL5 and IL10 when compared with inactive CSS. There was significant increase in BALF concentration of IL-5 and CCL26 in exacerbation of CSS. CCR4-active chemokines were detected more frequently in active disease. We found a strong positive correlation between clinical parameters of disease activity (BVAS, eosinophilia) and expression of IL4, IL5, IL10 and STAT5A. These results indicate that as compared with asthma, active-CSS patients have much stronger local Th2 response in the airways. Airway cells may contribute to lung eosinophilia in CSS by producing IL-5 and eosinophil active chemokines.