Abstract
HLA-DRB4 gene is associated with Churg-Strauss syndrome (CSS), a systemic eosinophilic vasculitis with a prodromal phase characterized by severe asthma, eosinophilia, nasal polyposis, and sinusitis. Aim of this study was to evaluate if the presence of HLA-DRB4 in asthmatic patients is associated with a clinical picture resembling that of the prodromal phase of CSS. HLA-DRB1 was determined in a cohort of 159 asthmatic patients and its frequency was compared with that of 1808 blood donors. HLA-DRB4 presence/absence was correlated with clinical features, including sinusitis, nasal polyposis, eosinophils, antiasthmatic drugs, asthma severity, and pulmonary function tests. HLA-DRB4 gene was associated with severe persistent asthma before treatment (P < 0.02), near fatal or severe hypoxemic asthma (P < 0.01), sinusitis (P < 0.01), nasal polyposis (P < 0.01), number of patients with eosinophils >1000/μl: (P < 0.05), need of beclomethasone >1000–2000 μg/daily (P < 0.001), use of a third controller (P < 0.05), and oral prednisone (P < 0.02). HLA-DRB4 gene is associated in asthmatic patients with a clinical picture characterized by asthma severity, sinusitis, nasal polyposis, and eosinophilia closely resembling that of the prodromal phase of CSS and might be useful to suspect corticosteroids-masked cases of CSS.
Highlights
Chug-Strauss syndrome (CSS) is a rare form of eosinophilic necrotising antineutrophil cytoplasmic antibodies (ANCA) associated with vasculitis affecting small to medium sized vessels [1]
human leukocyte antigens (HLA)-DRB4 gene is associated with Churg-Strauss syndrome (CSS), a systemic eosinophilic vasculitis with a prodromal phase characterized by severe asthma, eosinophilia, nasal polyposis, and sinusitis
HLA-DRB4 gene is associated in asthmatic patients with a clinical picture characterized by asthma severity, sinusitis, nasal polyposis, and eosinophilia closely resembling that of the prodromal phase of CSS and might be useful to suspect corticosteroids-masked cases of CSS
Summary
Chug-Strauss syndrome (CSS) is a rare form of eosinophilic necrotising antineutrophil cytoplasmic antibodies (ANCA) associated with vasculitis affecting small to medium sized vessels [1]. In most patients, it is characterized by a prodromal phase, in which severe adult onset asthma is the main feature with rhinosinusitis and nasal polyposis, a second phase, with peripheral blood and tissue eosinophilia, and, the systemic vasculitic phase [1]. Th17 lymphocytes have been involved in the pathogenesis of both autoimmune diseases and allergy/asthma [2] and, interestingly enough, CCS shares features of allergy/asthma and autoimmunity
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