• Skip to primary navigation
  • Skip to main content

Stanford Alliance for Primary Immunodeficiency

Stanford University

  • SAPI
  • Stanford PI Clinic
  • Patient Support
    • Diagnosis
    • Treatment and Complications
    • School
    • Work
    • Parenting
    • Sibling
    • Lifestyle
    • Mentorship Program
    • PI Resources
      • Immune Deficiency Foundation (IDF)
      • Jeffrey Modell Foundation
      • Painted Turtle Camp
      • Make-A-Wish
      • Baxter IVIG
      • CSL Behring IVIG
  • Kids’ Zone
    • Kids’ Zone
    • Pre-Teen FAQ
    • Teen FAQ
  • PID Research
    • Butte Lab Immunology Research Projects
    • PID Research blog
  • Local Events
  • Donate
You are here: Home / Archives for Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency-histologic and immunohistochemical analyses of 16 cases.

Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency-histologic and immunohistochemical analyses of 16 cases.

Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency-histologic and immunohistochemical analyses of 16 cases.

July 4, 2015 By Manish Butte

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 Jun 1;

Authors: Rao N, Mackinnon AC, Routes JM

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.

PMID: 26138782 [PubMed – as supplied by publisher]

Powered by WPeMatico

Filed Under: Research Tagged With: 5, closed, Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency-histologic and immunohistochemical analyses of 16 cases., post, publish

Copyright © 2025 · Genesis Framework by StudioPress · WordPress · Log in