[CIS PIDD] [cis-pidd] Lung transplantation in CVID

[CIS-PIDD]

Dear All,
I have a 42yo male with CIVD that was diagnosed with "sarcoidosis" and treated with steroids for many years. When he came to our institution, the diagnosis of GLILD was made and we treated him with RTX/MMF (could not tolerate azathioprine). Unfortunately, he had moderate to severe pulmonary fibrosis with severe restrictive lung disease at the time of presentation and is O2 dependent. The immunosuppression has stabilized his lung disease, but with a normal decline in lung function with age he will require lung transplantation at some point in time. I have referred him for evaluation of lung transplantation, but so far two  programs have refused to even evaluate him citing CVID as a contraindication. There is a paucity of literature on this topic and most lung transplants I could find in the literature had other complications at the time of transplantation  (severe bronchiectasis, hematological abnormalities etc.,), which my patient does not have. He has been on immunosuppressive therapy for a number of years w/o serious infections and I believe that the pos-transplant immunosuppression (cyclosporin-MMF) would make a return of the GLILD highly unlikely. Does anyone have any direct experience in lung transplantation for pulmonary fibrosis due to GLILD in CVID.
thanks
Jack



John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874
Phone: Office 414-266-6840
Fax: 414-266-6437
Email: jroutes at mcw.edu<mailto:jroutes at mcw.edu>

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