Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL)

摘要

Abstract BackgroundCommon variable immunodeficiency (CVID) and other primary antibody deficiencies (PAD) are a heterogeneous group of ?300 congenital disorders affecting the immune system. Until recently, efforts to measure health-related quality of life (QoL) in PAD patients have utilised generic QoL tools and disease-specific tools for other conditions. Still, the full impact of the disease is probably not understood. We evaluated the performance of the CVID_QoL, a novel disease-specific QoL instrument for adults with CVID, on Norwegian PAD patients and compared the results to those of the generic WHOQOL-BREF.MethodsRespondents were recruited through the Norwegian Centre for Rare Disorders’ patient database. Included patients fulfilled the following criteria (all three): 1.) Age ≥18 years, 2.) a PAD diagnosis, 3.) currently on immunoglobulin therapy.?The CVID_QoL is a 32-item questionnaire. Global CVID_QoL scores were compared between Norwegian PAD patients and Italian CVID patients.ResultsIn total, 83 PAD patients filled out the CVID_QoL, 63% had CVID, 76% were females. 32 patients filled out the WHOQOL-BREF.?Feasibility was high (1% missing). Internal consistency for the emotional- (Cronbach’s α-value?= 0.91) and relational functioning (α?= 0.77) subscales was high, but questionable for the gastrointestinal and skin symptoms subscale (α?= 0.66). Convergent validity varied from weak to strong (range 0.3–0.8).?Floor and ceiling effects were present.ConclusionsAlthough many disease-specific characteristics are probably shared with CVID and other PAD, the CVID_QoL captures some, but not all, dimensions of PAD patients’ QoL. More evaluations of the CVID_QoL’s performance in different contexts are needed.