Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Deficiency: A Retrospective Cohort Study

Fatema Mollah; Angeline Massett; Kale Bongers; Kristine Konopka; Bonnie Wang; David O'Dwyer; Anna Kovalszki

doi:10.1016/j.jaci.2023.11.347

Back

Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Deficiency: A Retrospective Cohort Study

Abstract

Open access

Peer reviewed

Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Deficiency: A Retrospective Cohort Study

Fatema Mollah, Angeline Massett, Kale Bongers, Kristine Konopka, Bonnie Wang, David O'Dwyer and Anna Kovalszki

Journal of allergy and clinical immunology, Vol.153(2 Supplement), p.AB106

02/2024

DOI: 10.1016/j.jaci.2023.11.347

Files and links (1)

url

https://doi.org/10.1016/j.jaci.2023.11.347View

Published (Version of record) Open Access

Abstract

Rationale Granulomatous lymphocytic interstitial lung disease (GL-ILD) is a complication of common variable immunodeficiency (CVID). This study sought to determine key features, evaluation and therapy in a retrospective cohort encompassing patients seen by Pulmonology and Immunology at the University of Michigan from 2018-2022. Methods We used ICD codes for CVID and ILD to review 56 patients and include 34 patients based on clinical symptoms, radiologic features, pulmonary function testing, lab values and lung biopsies. Results GL-ILD was associated with splenomegaly (67.8%), female sex (61.8%), history of immune thrombocytopenia (35.3%), and history of smoking (32.4%). Mean IgG was 276.2 mg/dL. Infections included pneumonia (73.5%), sinusitis (47%), upper respiratory (50%) and urinary tract infection (26%). Infectious agents included rhino-enterovirus (23.5%), SARS-CoV-2 (17.6%), Haemophilus influenzae (14.7%), and Streptococcus pneumoniae (11.7%). Lymphocyte phenotyping showed decreased levels of T (45.8% of patients), B (25%), and NK (25%) cells. Among a variety of genetic testing including PID panels and whole exome sequencing, there were three cases of NOD2 increased risk allele and one patient had CTLA-4 haploinsufficiency, which has been reported in earlier onset GL-ILD. Lung biopsies contained granulomas, lymphoid infiltrates, organizing pneumonia and interstitial fibrosis to varying degrees. Half of patients were treated with rituximab with modest improvements in symptoms and pulmonary function testing. Conclusions GL-ILD is a complication of CVID in up to 20-30% of patients, but optimal non-invasive testing, evaluation and management are not clearly defined. This study elucidates the clinical picture of GL-ILD and its management in an academic institution.

Details

Title: Subtitle: Granulomatous Lymphocytic Interstitial Lung Disease in Common Variable Deficiency: A Retrospective Cohort Study
Creators: Fatema Mollah - University of Michigan–Ann Arbor
Angeline Massett - University of Michigan–Ann Arbor
Kale Bongers - University of Michigan–Ann Arbor
Kristine Konopka - University of Michigan–Ann Arbor
Bonnie Wang - University of Michigan–Ann Arbor
David O'Dwyer
Anna Kovalszki - University of Michigan–Ann Arbor
Resource Type: Abstract
Publication Details: Journal of allergy and clinical immunology, Vol.153(2 Supplement), p.AB106
DOI: 10.1016/j.jaci.2023.11.347
ISSN: 0091-6749
eISSN: 1097-6825
Language: English
Date published: 02/2024
Academic Unit: Internal Medicine
Record Identifier: 9984696678102771

Metrics

1 Record Views