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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Hum+Pathol
2015 ; 46
(9
): 1306-14
Nephropedia Template TP
gab.com Text
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English Wikipedia
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.