Management of granulomatous lymphocytic interstitial lung disease in a patient with common variable immune deficiency

Mohini Pathria, Daniel Urbine, Marc Stuart Zumberg, Juan Guarderas

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

A 61-year-old woman presented with longstanding cough and progressive dyspnoea. She underwent an extensive evaluation and was diagnosed with common variable immunodeficiency (CVID) with granulomatous lymphocytic interstitial lung disease (GLILD). She was initially treated with subcutaneous immunoglobulin therapy, having declined intravenous immunoglobulin (IVIG) therapy. She also declined treatment with oral glucocorticoids. Over several months, she became increasingly symptomatic and developed increased pulmonary infiltrates, pleural effusions, mediastinal adenopathy, splenomegaly, pancytopenia and ascites. An interdisciplinary team composed of an immunologist, pulmonologist and haematologist deliberated over a therapeutic management approach. The patient received a recently reported immunotherapy regimen with azathioprine and rituximab. The therapy led to rapid improvement of her constitutional and respiratory symptoms, with clinical and radiographic improvement in her interstitial lung disease, lymphadenopathy, pleural effusions and ascites. This case report reviews the literature surrounding the diagnosis and management of GLILD.

Original languageEnglish (US)
Article number215624
JournalBMJ Case Reports
Volume2016
DOIs
StatePublished - 2016
Externally publishedYes

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Common Variable Immunodeficiency
Interstitial Lung Diseases
Passive Immunization
Pleural Effusion
Ascites
Pancytopenia
Intravenous Immunoglobulins
Splenomegaly
Azathioprine
Cough
Dyspnea
Immunotherapy
Glucocorticoids
Therapeutics
Lung
Lymphadenopathy

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Management of granulomatous lymphocytic interstitial lung disease in a patient with common variable immune deficiency. / Pathria, Mohini; Urbine, Daniel; Zumberg, Marc Stuart; Guarderas, Juan.

In: BMJ Case Reports, Vol. 2016, 215624, 2016.

Research output: Contribution to journalArticle

Pathria, Mohini ; Urbine, Daniel ; Zumberg, Marc Stuart ; Guarderas, Juan. / Management of granulomatous lymphocytic interstitial lung disease in a patient with common variable immune deficiency. In: BMJ Case Reports. 2016 ; Vol. 2016.
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