ABSTRACT
PURPOSE: To quantify the health related quality of life in primary immunodeficiency patients. MATERIALS AND METHODS: We used generic health status and general psychological health questionnaires to determine the range of issues that needed to be considered in examining the burden of common variable immunodeficiency (CVID). RESULTS: The health status of patients with CVID was lower than that observed in normal subjects. Overall, Role-Physical and General Health scales correlated with a poorer clinical status. Surprisingly, the duration of disease did not influence health status. Being female, older, General Health Questionnaire-positive and alexithymic proved to be major risk factors associated with a poor health status. Patients with chronic lung disease and chronic diarrhea had the lowest values on the Medical Outcome Study, Short Form SF-36 (SF-36) scales. Disease severity perception was associated with the General Health Questionnaire and alexithymia status. Limitations in daily activities as a result of lower physical health were the major problems facing common variable immunodeficiency patients. CONCLUSION: Our data underlined the importance of conducting a periodical health related quality of life assessment on patients with primary antibody deficiencies and, moreover, stressed the necessity of providing psychological support to at risk patients.
Subject(s)
Female , Humans , Male , Chronic Disease , Common Variable Immunodeficiency/physiopathology , Health Status , Quality of Life , Surveys and QuestionnairesABSTRACT
INTRODUCTION: Common variable immunodeficiency is characterized by defective antibody production and recurrent pulmonary infections. Intravenous immunoglobulin is the treatment of choice, but the effects of Intravenous immunoglobulin on pulmonary defense mechanisms are poorly understood. OBJECTIVE: The aim of this study was to verify the impact of intravenous immunoglobulin on the physical properties of the sputum and on inflammatory alterations in the airways of patients with Common variable immunodeficiency associated with bronchiectasis. METHOD: The present study analyzed sputum physical properties, exhaled NO, inflammatory cells in the sputum, and IG titers in 7 patients with Common variable immunodeficiency and bronchiectasis with secretion, immediately before and 15 days after Intravenous immunoglobulin. A group of 6 patients with Common variable immunodeficiency and bronchiectasis but no sputum was also studied for comparison of the basal IgG level and blood count. The 13 patients were young (age=36±17 years) and comprised predominantly of females (n=11). RESULTS: Patients with secretion presented significantly decreased IgG and IgM levels. Intravenous immunoglobulin was associated with a significant decrease in exhaled NO (54.7 vs. 40.1 ppb, p<0.05), sputum inflammatory cell counts (28.7 vs. 14.6 cells/mm³, p<0.05), and a significant increase in respiratory mucus transportability by cough (42.5 vs. 65.0 mm, p < 0.05). CONCLUSION: We concluded that immunoglobulin administration in Common variable immunodeficiency patients results in significant improvement in indexes of inflammation of the airways with improvement in the transportability of the respiratory mucus by cough.
Subject(s)
Adult , Female , Humans , Male , Bronchiectasis , Common Variable Immunodeficiency , Immunoglobulins, Intravenous/therapeutic use , Mucociliary Clearance/physiology , Respiratory Tract Infections , Sputum , Bronchiectasis/drug therapy , Bronchiectasis/immunology , Bronchiectasis/physiopathology , Cell Count , Common Variable Immunodeficiency/drug therapy , Common Variable Immunodeficiency/immunology , Common Variable Immunodeficiency/physiopathology , Cough/immunology , Cough/physiopathology , Immunoglobulin G/analysis , Immunoglobulin M/analysis , Mucus/physiology , Nitric Oxide/analysis , Respiratory Tract Infections/drug therapy , Respiratory Tract Infections/immunology , Respiratory Tract Infections/physiopathology , Statistics, Nonparametric , Sputum/cytology , Sputum/drug effects , Sputum/immunology , Time FactorsABSTRACT
We report a 26 years old female with a common variable immunodeficiency diagnoses at the age of 21 with recurrent pulmonary and sinusal bacterial infections that had a clinical and laboratory remission. At the moment of diagnosis she had agammaglobulinemia in the protein electrophoresis, very low level of IgG and IgM and absence of IgA. Absolute counts of CD4(+) T lymphocytes were low and CD8(+) were normal. B lymphocyte count was normal. Five years later a repeated study revealed normal levels of all these parameters, excepting IgA that continued to be undetectable. We propose that the remission could be due to a decrease in suppressor activity of T lymphocytes. There is no documented evidence of infectious factors or the use of immunological therapy that could have influenced the course of the disease