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Use of combination chemotherapy for treatment of granulomatous and lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID).


ABSTRACT: A subset of patients with common variable immunodeficiency (CVID) develops granulomatous and lymphocytic interstitial lung disease (GLILD), a restrictive lung disease associated with early mortality. The optimal therapy for GLILD is unknown. This study was undertaken to see if rituximab and azathioprine (combination chemotherapy) would improve pulmonary function and/or radiographic abnormalities in patients with CVID and GLILD.A retrospective chart review of patients with CVID and GLILD who were treated with combination chemotherapy was performed. Complete pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans of the chest were done prior to therapy and >6 months later. HRCT scans of the chest were blinded, randomized, and scored independently (in pairs) by two radiologists. The differences between pre- and post-treatment HRCT scores and PFT parameters were analyzed.Seven patients with CVID and GLILD met inclusion criteria. Post-treatment increases were noted in both FEV1 (p=0.034) and FVC (p=0.043). HRCT scans of the chest demonstrated improvement in total score (p=0.018), pulmonary consolidations (p=0.041), ground-glass opacities (p=0.020) nodular opacities (p=0.024), and both the presence and extent of bronchial wall thickening (p=0.014, 0.026 respectively). No significant chemotherapy-related complications occurred.Combination chemotherapy improved pulmonary function and decreased radiographic abnormalities in patients with CVID and GLILD.

SUBMITTER: Chase NM 

PROVIDER: S-EPMC3557581 | biostudies-literature | 2013 Jan

REPOSITORIES: biostudies-literature

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Use of combination chemotherapy for treatment of granulomatous and lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID).

Chase Nicole M NM   Verbsky James W JW   Hintermeyer Mary K MK   Waukau Jill K JK   Tomita-Mitchell Aoy A   Casper James T JT   Singh Sumit S   Shahir Kaushik S KS   Tisol William B WB   Nugent Melodee L ML   Rao R Nagarjun RN   Mackinnon A Craig AC   Goodman Lawrence R LR   Simpson Pippa M PM   Routes John M JM  

Journal of clinical immunology 20120829 1


<h4>Purpose</h4>A subset of patients with common variable immunodeficiency (CVID) develops granulomatous and lymphocytic interstitial lung disease (GLILD), a restrictive lung disease associated with early mortality. The optimal therapy for GLILD is unknown. This study was undertaken to see if rituximab and azathioprine (combination chemotherapy) would improve pulmonary function and/or radiographic abnormalities in patients with CVID and GLILD.<h4>Methods</h4>A retrospective chart review of patie  ...[more]

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