| Popis: |
Background: The common variable immunodeficiency syndrome, also known as CVID, is taught to be the second most prevalent humoral immunodeficiency and to be characterized by a reduction of at least 2 immunoglobulin classes. In contrast, in IgG subclass deficiencies more than 1 IgG subgroups are lowered. In this study we investigated whether diagnosis of CVID or IgG subclass deficiency could be deduced based on clinical manifestation and whether the extent of IgG immunoglobulin diminution could give hints for the clinics. Methods: In this retrospective study all clinical and laboratory findings of 116 patients, who have been examined during a 10-year period (1995 -2004) at our outpatient clinic for allergy and clinical immunology because of suspicion of an immunodeficiency, have been collected. Results: 23 out of 116 (14.6%) patients were diagnosed as having a probable CVID and 11 (7.0%) a possible CVID. 82 (51.9%) patients had an IgG subclass deficiency. The most prevalent clinical manifestation were recurrent or prolonged upper respiratory infections in 91 (78.5%) patients. 73 (62.9%) of them suffered from chronic rhinosinusitis. Prolonged bronchitis and pneumonias were disclosed in 49 (42.2%) and 45 (38.8%), respectively. Granulomatous disorders were found in 4 (3.5%) and neoplasia in 3 subjects (2.6%). Patients with a probable CVID had significantly more (p = 0.03) upper respiratory infections than those with a possible CVID or IgG subclass deficiency. In contrast, subjects with an IgG subclass deficiency had more frequently a respiratory allergy than those with a CVID. Conclusion: This investigation showed that with marked IgG deficiency persisting and repeating infections, as well as their complications, like bronchiectasia, were the determinating characteristics. On the other hand, clinical manifestations were not characteristic in mild or moderate IgG deficiency. A CVID could not be distinguished from an IgG subclass defect by the clinical manifestation, but by laboratory analysis only. In case of suspicion for immunoglobulin deficiency IgG subclasses should also be determined. |
