Open Access Case Report

Diffuse panbronchiolitis in a patient with common variable immunodeficiency: a casual association or a pathogenetic correlation?

Alessandro Ginori1*, Aurora Barone1, David Bennett2, Marie Aimée Gloria Munezero Butorano1, Maria Grazia Mastrogiulio1, Antonella Fossi2, Paola Rottoli2 and Donatella Spina1

Author Affiliations

1 Department of Medical Biotechnologies, Pathology Unit, University of Siena, strada delle Scotte 6, Siena 53100, Italy

2 Respiratory Diseases and Lung Transplantation Unit, Regional Center for Sarcoidosis and other Interstitial Lung Diseases, University of Siena, viale Bracci 16, Siena 53100, Italy

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Diagnostic Pathology 2014, 9:12  doi:10.1186/1746-1596-9-12

Published: 20 January 2014

Abstract

Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease that seems to have an immunological pathogenesis and that causes a severe progressive suppurative and obstructive respiratory disorder. Common variable immunodeficiency (CVID) is the most common serious primary immunodeficiency and it is often associated with respiratory diseases. Herein, we describe a case of DPB in a 41-year-old man affected by CVID. We examined the patient’s lungs, focusing on the characteristics of the inflammatory cells and of the foamy macrophagic nodules typical of DPB. Immunohistochemical typing of the lymphocytic infiltrate showed that B-cells were almost absent, matching the immunological profile of CVID. The case described is the first case reported in the literature of DPB in a patient affected by CVID. Moreover it seems to confirm the correlation between an immunodeficiency status and the development of DPB and provides more information on the accumulation of nodules of foamy macrophages in DPB.

Virtual slides

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/5310709471138338 webcite.

Keywords:
Diffuse panbronchiolitis; Common variable immunodeficiency; Foamy macrophages; Lymphocytes; Interstitial lung disease