Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency--histologic and immunohistochemical analyses of 16 cases. Hum Pathol 2015 Sep;46(9):1306-14
Date
07/04/2015Pubmed ID
26138782Pubmed Central ID
PMC4554947DOI
10.1016/j.humpath.2015.05.011Scopus ID
2-s2.0-84940460372 (requires institutional sign-in at Scopus site) 86 CitationsAbstract
Common variable immunodeficiency is a primary immunodeficiency of unknown etiology characterized by low serum immunoglobulin G, a decreased ability to make specific antibodies, and variable T-cell defects. Approximately 10-30% of patients with common variable immunodeficiency develop clinical evidence of a diffuse parenchymal lung disease with a constellation of histopathologic findings termed granulomatous and lymphocytic interstitial lung disease. In this study, we characterized the histologic and immunohistochemical features in a series of 16 cases diagnosed by open lung biopsy. Peribronchiolar and interstitial lymphocytic infiltration, granulomatous inflammation, and organizing pneumonia were consistent features; interstitial fibrosis with architectural remodeling was also found in a subgroup of patients. By immunohistochemistry, a predominance of CD4+ T lymphocytes with variable numbers of CD8+ T cells and B cells was present, with a striking absence of FOXP3-positive T-regulatory cells. This heretofore unrecognized immunohistochemical finding needs further investigation for a potential role in the pathogenesis of the condition. The presence of interstitial fibrosis with or without architectural remodeling in a subset of patients also needs additional study, for effect on prognosis.
Author List
Rao N, Mackinnon AC, Routes JMMESH terms used to index this publication - Major topics in bold
AdultBiomarkers
Biopsy
Common Variable Immunodeficiency
Female
Granuloma, Respiratory Tract
Humans
Immunohistochemistry
Lung
Lung Diseases, Interstitial
Lymphocyte Subsets
Male
Middle Aged
Young Adult
