Abstract
BackgroundInterstitial lymphocytic lung disease (ILLD), a recently recognized complication of primary immunodeficiencies (PID), is caused by immune dysregulation, abnormal bronchus-associated lymphoid tissue (BALT) hyperplasia, with subsequent progressive loss of pulmonary function. Various modes of standard immunosuppressive therapy for ILLD have been shown as only partially effective.ObjectivesTo retrospectively evaluate the safety and efficacy of abatacept or rituximab in treatment of ILLD in children with PID.Methods29 children (median age 11 years) with various forms of PID received one of the two therapy regimens predominantly based on the lesions’ immunohistopathology: children with prevalent B-cell lung infiltration received rituximab (n = 16), and those with predominantly T-cell infiltration received abatacept (n = 17). Clinical and radiological symptoms were assessed using a severity scale developed for the study.ResultsThe targeted therapy with abatacept (A) or rituximab (R) enabled long-term control of clinical (A 3.4 ± 1.3 vs. 0.6 ± 0.1; R 2.8 ± 1 vs. 0.7 ± 0.05, p < 0.01) and radiological (A 18.4 ± 3.1 vs. 6.0 ± 2.0; R 30 ± 7.1 vs. 10 ± 1.7, p < 0.01) symptoms of ILLD in both groups and significantly improved patients’ quality of life, as measured by the total scale (TS) score of 57 ± 2.1 in treatment recipients vs. 31.2 ± 1.9 before therapy (p < 0.01).ConclusionsILLD histopathology should be considered when selecting treatment. Abatacept and rituximab are effective and safe in differential treatment of ILLD in children.
Highlights
Primary immunodeficiencies (PID) comprise a heterogeneous group of more than 400 inherited conditions with associated immune dysfunctions [1]
Rituximab and Abatacept Are Effective in Interstitial lymphocytic lung disease (ILLD)
Targeted immunosuppressive therapy is effective in achieving control of ILLD in patients with inborn errors of immunity
Summary
Primary immunodeficiencies (PID) comprise a heterogeneous group of more than 400 inherited conditions with associated immune dysfunctions [1]. Interstitial lymphocytic lung disease (ILLD) is a recently characterized nonmalignant PID complication [4]. In a cohort of adult patients who had common variable immunodeficiency (CVID), the incidence of ILLD was reported as ~ 22% [5] and as high as 38–52% in monogenic immune dysregulation syndromes such as CTLA4 [6] and LRBA [7] deficiencies. Recent studies have shown abatacept to be effective in controlling symptoms of LRBA and CTLA deficiency, including ILLD [20, 21]. Interstitial lymphocytic lung disease (ILLD), a recently recognized complication of primary immunodeficiencies (PID), is caused by immune dysregulation, abnormal bronchus-associated lymphoid tissue (BALT) hyperplasia, with subsequent progressive loss of pulmonary function. Various modes of standard immunosuppressive therapy for ILLD have been shown as only partially effective
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