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- Title
Běžná variabilní imunodeficience a granulomatózní/lymfocytární intersticiální plicní nemoc (GLILD).
- Authors
Doubková, M.; Doubek, M.; Richter, S.; Chovancová, Z.
- Abstract
Introduction: Common variable immunodeficiency (CVID) is a heterogeneous disorder representing the most common group of symptomatic congenital disorders of antibody production. It is characterized by IgG, IgA and/or IgM hypogammaglobulinemia accompanied by an impaired antibody response after antigenic stimulation. In addition to infectious complications, this disease is often complicated by clinical manifestations of immune dysregulation. These complications also include granulomatous-lymphocytic interstitial lung disease (GLILD), a multisystemic granulomatous and/or inflammatory disease significantly increasing the mortality and morbidity of these patients. Materials and methods: A total of eight patients with CVID/GLILD were followed at our pulmonary department in the years 2002-2023 (5 females, age range 20-33 years, median age at diagnosis 23 years; 3 males, age range 23-49 years; median age at diagnosis 23 years). All patients were non-smokers. In most patients, the diagnosis of CVID preceded the diagnosis of CVID/GLILD. Histological evidence of GLILD was confirmed in 6/8 patients (75 %). Pulmonary involvement was assessed based on lung function parameters and imaging methods (posterior chest radiograph and chest high-resolution computed tomography [HRCT]). Results: CVID/GLILD is presented in eight cases with different characteristics of pulmonary and extrapulmonary involvement. The extent of pulmonary parenchymal involvement on chest HRCT, assessed at the initial examination by an automated analysis method, was in the range of 6-33 % (with a mean of 18%) in our patients. All patients were treated with immunosuppressive agents, two of them with corticosteroids in monotherapy and the others with combined immunosuppressants. The median follow-up from CVID/GLILD diagnosis was 9.5 years. The median survival was not reached as only one patient died. Conclusion: Interdisciplinary cooperation between pulmonologists, hematologists, radiologists and immunologists is the basis of our care for patients with CVID/GLILD. Immunosuppressant therapy is long-term, with a risk of disease relapse, but without a major risk of infectious complications. Since corticosteroid monotherapy is insufficient in most cases, combined therapy with rituximab and another immunosuppressant (rituximab with corticosteroids, rituximab mycophenolate) appears to be more effective.
- Publication
Studia Pneumologica et Phthiseologica, 2023, Vol 83, Issue 5, p159
- ISSN
1213-810X
- Publication type
Article