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 slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/5310709471138338.
Highlights
Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease that is characterized by progressive suppurative and obstructive airway disease, which, if left untreated, gives rise to bronchiectases, respiratory failure and death [1]
Histologic studies revealed that interstitial lung disease (ILD) in the context of Common variable immunodeficiency (CVID) may manifest as sarcoid-like granuloma, organizing pneumonia, nonspecific interstitial pneumonia (NSIP), follicular bronchiolitis and lymphocytic interstitial pneumonia (LIP)
To the best of our knowledge, the case reported is the first case described in the literature of panbronchiolitis in a patient affected by CVID
Summary
Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease that is characterized by progressive suppurative and obstructive airway disease, which, if left untreated, gives rise to bronchiectases, respiratory failure and death [1]. Diagnosis of CVID had been made when the patient was 22 years old for recurrent respiratory tract infections; he had a complete deficiency of the production of all immunoglobulin classes (IgA, IgM, IgG and IgE). He worked in textile industry until the age of 38 when he developed chronic respiratory failure and started oxygen-therapy. The final diagnosis, according to the radiological and clinical findings and to the clinical history of the patient, was acute necrotizing and chronic fibrosing panbronchitis and panbronchiolitis
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
