Can HLA-DRB4 Help to Identify Asthmatic Patients at Risk of Churg-Strauss Syndrome?

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/ µ l: (P < 0.05), need of beclomethasone >1000-2000 µ g/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.

The common allergens in the Churg-Strauss syndrome.

BACKGROUND: The asthmatic-prodromal phase of Churg-Strauss syndrome (CSS) is usually considered allergic, but data about the involved allergens are scarce. The aim of our work was to examine the prevalence of allergy in a group of CSS patients and in two control groups of persistent asthmatic subjects selected for eosinophilia >10% [first control group patients (CGP1)] and eosinophils <6% [second control group patients (CGP2)]. METHODS: The respiratory symptoms, and the results of prick test and/or RAST for the common allergens, performed before the vasculitic phase in 51 CSS, were retrospectively evaluated and compared with those of 46 CGP1 and 50 CGP2. RESULTS: 31.4% of CSS vs 67.4% of CGP1 (P = 0.0004) and vs 58.0% CGP2 (P = 0.007) were allergic. The number of subjects with seasonal allergies was lower in CSS vs CGP1 (P = 0.0069) and vs CGP2 (P = 0.0002). The number of perennial allergies was significantly higher in CSS than in both control groups (CSS vs CGP1, P = 0.0108; CSS vs CGP2, P = 0.0079). The subjects allergic to Dermatophagoides were prevalent in CSS vs CGP1 (P = 0.0045) but not vs CGP2. CONCLUSIONS: The evidence of allergy, considered as the demonstration of specific IgE consistent with the clinical history, is present in less than one-third of CSS and the higher prevalence of seasonal allergies in the controls disagrees with persistent asthma. Allergy may be only one of several mechanisms triggering exacerbation of asthma or supporting chronic airway inflammation as in asthma in general. Alternatively, unidentified allergens may play a role.

[Churg-Strauss syndrome: is it a rare disease?]. / Sindrome di Churg-Strauss: è una malattia rara?

We describe four patients with Churg-Strauss syndrome admitted to the hospital in 1988. Some clinical and histological features of particular relief are present in these cases. All patients had been treated with corticosteroids and they are now in remission. Our work suggests that Churg-Strauss syndrome is not a rare disease and that an early diagnosis and appropriate therapy may influence favourably at least long term survival.