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J Allergy Clin Immunol. 2014 Feb;133(2):535-42. doi: 10.1016/j.jaci.2013.08.022. Epub 2013 Oct 13.

Tertiary lymphoid neogenesis is a component of pulmonary lymphoid hyperplasia in patients with common variable immunodeficiency.

Author information

1
Immunology Institute, Mount Sinai School of Medicine, New York, NY; Department of Medicine, Mount Sinai School of Medicine, New York, NY.
2
Department of Pathology, Mount Sinai School of Medicine, New York, NY.
3
Department of Medicine, Mount Sinai School of Medicine, New York, NY; Department of Pathology, Mount Sinai School of Medicine, New York, NY.
4
Immunology Institute, Mount Sinai School of Medicine, New York, NY; Department of Medicine, Mount Sinai School of Medicine, New York, NY; Department of Pediatrics, Mount Sinai School of Medicine, New York, NY. Electronic address: Charlotte.Cunningham-Rundles@mssm.edu.

Abstract

BACKGROUND:

Despite reducing pneumonia and other infections, antibody replacement does not appear to treat pulmonary lymphoid hyperplasia (PLH) in patients with common variable immunodeficiency (CVID). The pathogenesis and optimal treatments remain to be clarified.

OBJECTIVE:

We aimed to better understand the pathology of CVID-associated lung disease. Tertiary lymphoneogenesis, although a component of interstitial lung disease associated with autoimmune diseases, has not previously been explored in patients with CVID.

METHODS:

We examined the clinical characteristics and pathologic findings of 6 patients with CVID with nodular/infiltrative lung disease who had biopsy specimens demonstrating PLH.

RESULTS:

In these subjects regions of PLH contained distinct B- and T-cell zones, with B-cell predominance in 1 patient and T-cell predominance in the others. Colocalization of Ki67, Bcl6, and CD23 within this ectopic lymphoid architecture demonstrated tertiary lymphoneogenesis with active centers of cellular proliferation. One patient received rituximab with improved pulmonary radiologic findings.

CONCLUSION:

Ectopic lymphoid tissue forming germinal centers suggest tertiary lymphoneogenesis in CVID-associated lung disease. B cell-targeted therapy might disrupt CVID-associated lymphoid hyperplasia.

KEYWORDS:

CT; CVID; Common variable immunodeficiency; Computed tomography; Diffusing capacity of the lung for carbon monoxide; Dlco; GLILD; Granulomatous-lymphocytic interstitial lung disease; LIP; Lymphocytic interstitial pneumonia; PLH; Pulmonary lymphoid hyperplasia; chronic lung disease; ectopic follicle; germinal center; lymphoid neogenesis; pulmonary hyperplasia

PMID:
24131823
PMCID:
PMC4109033
DOI:
10.1016/j.jaci.2013.08.022
[Indexed for MEDLINE]
Free PMC Article

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