PURPOSE: 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. METHODS: A retrospective chart review of patients with CVID and GLILD who were treated withcombination 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. RESULTS: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. CONCLUSIONS:Combination chemotherapy improved pulmonary function and decreased radiographic abnormalities in patients with CVID and GLILD.
RCT Entities:
PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: Combination chemotherapy improved pulmonary function and decreased radiographic abnormalities in patients with CVID and GLILD.
Authors: J Wanger; J L Clausen; A Coates; O F Pedersen; V Brusasco; F Burgos; R Casaburi; R Crapo; P Enright; C P M van der Grinten; P Gustafsson; J Hankinson; R Jensen; D Johnson; N Macintyre; R McKay; M R Miller; D Navajas; R Pellegrino; G Viegi Journal: Eur Respir J Date: 2005-09 Impact factor: 16.671
Authors: M R Miller; J Hankinson; V Brusasco; F Burgos; R Casaburi; A Coates; R Crapo; P Enright; C P M van der Grinten; P Gustafsson; R Jensen; D C Johnson; N MacIntyre; R McKay; D Navajas; O F Pedersen; R Pellegrino; G Viegi; J Wanger Journal: Eur Respir J Date: 2005-08 Impact factor: 16.671
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Authors: L J Maarschalk-Ellerbroek; P A de Jong; J M van Montfrans; J W J Lammers; A C Bloem; A I M Hoepelman; P M Ellerbroek Journal: J Clin Immunol Date: 2014-06-21 Impact factor: 8.317
Authors: Elise M N Ferré; Timothy J Break; Peter D Burbelo; Michael Allgäuer; David E Kleiner; Dakai Jin; Ziyue Xu; Les R Folio; Daniel J Mollura; Muthulekha Swamydas; Wenjuan Gu; Sally Hunsberger; Chyi-Chia R Lee; Anamaria Bondici; Kevin W Hoffman; Jean K Lim; Kerry Dobbs; Julie E Niemela; Thomas A Fleisher; Amy P Hsu; Laquita N Snow; Dirk N Darnell; Samar Ojaimi; Megan A Cooper; Martin Bozzola; Gary I Kleiner; Juan C Martinez; Robin R Deterding; Douglas B Kuhns; Theo Heller; Karen K Winer; Arun Rajan; Steven M Holland; Luigi D Notarangelo; Kevin P Fennelly; Kenneth N Olivier; Michail S Lionakis Journal: Sci Transl Med Date: 2019-06-05 Impact factor: 17.956