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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

07/04/2015

Pubmed ID

26138782

Pubmed Central ID

PMC4554947

DOI

10.1016/j.humpath.2015.05.011

Scopus ID

2-s2.0-84940460372   72 Citations

Abstract

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 JM

Author

John M. Routes MD Chief, Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adult
Biomarkers
Biopsy
Common Variable Immunodeficiency
Female
Granuloma, Respiratory Tract
Humans
Immunohistochemistry
Lung
Lung Diseases, Interstitial
Lymphocyte Subsets
Male
Middle Aged
Young Adult