Literature DB >> 28583689

Granulomatous-Lymphocytic Interstitial Lung Disease in a Patient With Common Variable Immunodeficiency.

Jehan L Shah1, Sagar B Amin2, Nupur Verma2, Tan-Lucien Mohammed2.   

Abstract

Common variable immunodeficiency is the most common primary immunodeficiency and consists of impaired immunoglobulin production causing recurrent sinopulmonary infections. The most common cause of mortality for this disorder, however, is from the development of malignancy and autoimmune disorders. One common entity that develops is a systemic granulomatous and lymphoproliferative disorder that can cause an interstitial lung disease more formally referred to as granulomatous-lymphocytic interstitial lung disease (GL-ILD). We discuss a case of a 25-year-old woman with common variable immunodeficiency and GL-ILD and review the literature to summarize the most common radiological findings to raise the suspicion for GL-ILD on high-resolution computed tomography and delineate this from infection and other mimickers. We will also review key histopathological characteristics for diagnosis and the clinical approach and treatment options for this rare disease.
Copyright © 2018 Elsevier Inc. All rights reserved.

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Year:  2017        PMID: 28583689     DOI: 10.1067/j.cpradiol.2017.04.007

Source DB:  PubMed          Journal:  Curr Probl Diagn Radiol        ISSN: 0363-0188


  1 in total

1.  Respiratory manifestations in LPS-responsive beige-like anchor (LRBA) protein-deficient patients.

Authors:  Oded Shamriz; Bella Shadur; Adeeb NaserEddin; Irina Zaidman; Natalia Simanovsky; Orly Elpeleg; Eitan Kerem; Joel Reiter; Polina Stepensky
Journal:  Eur J Pediatr       Date:  2018-05-18       Impact factor: 3.183

  1 in total

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