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10.1016/j.humpath.2015.05.011

http://scihub22266oqcxt.onion/10.1016/j.humpath.2015.05.011
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suck abstract from ncbi


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pmid26138782
      Hum+Pathol 2015 ; 46 (9 ): 1306-14
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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 #MMPMID26138782
  • Rao N ; Mackinnon AC ; Routes JM
  • Hum Pathol 2015[Sep]; 46 (9 ): 1306-14 PMID26138782 show ga
  • 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.
  • |*Immunohistochemistry [MESH]
  • |Adult [MESH]
  • |Biomarkers/analysis [MESH]
  • |Biopsy [MESH]
  • |Common Variable Immunodeficiency/*diagnosis/immunology/pathology [MESH]
  • |Female [MESH]
  • |Granuloma, Respiratory Tract/*diagnosis/immunology/pathology [MESH]
  • |Humans [MESH]
  • |Lung Diseases, Interstitial/*diagnosis/immunology/pathology [MESH]
  • |Lung/*immunology/*pathology [MESH]
  • |Lymphocyte Subsets/*immunology/pathology [MESH]
  • |Male [MESH]
  • |Middle Aged [MESH]


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