Běžná variabilní imunodeficience a granulomatózní/lymfocytární intersticiální plicní nemoc
| Title in English | Common variable immunodeficiency and granulomatous-lymphocytic interstitial lung diseases Introduction |
|---|---|
| Authors | |
| Year of publication | 2023 |
| Type | Article in Periodical |
| Magazine / Source | Studia Pneumologica et Phthiseologica |
| MU Faculty or unit | |
| Citation | |
| Web | https://trios.cz/odborne-casopisy/studia-p-et-p/ |
| Keywords | common variable immunodeficiency; granulomatosis; interstitial lung fibrosis; prognosis; treatment |
| Description | 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 ad-dition to infectious complications, this disease is often complicated by clinical manifestations of immune dysre-gulation. These complications also include granulomatous-lymphocytic interstitial lung disease (GLILD), a multi-systemic 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 extrapulmo-nary involvement. The extent of pulmonary parenchymal involvement on chest HRCT, assessed at the initial exa-mination 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 immunolo-gists 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 insuf-ficient in most cases, combined therapy with rituximab and another immunosuppressant (rituximab with corti-costeroids, rituximab mycophenolate) appears to be more effective. |