Answer:
I do not have personal experience with any of my CVID patients needed lung transplant for bronchiectasis. I have included below abstract and references for case reports and series for this patient population. While lung transplant for severe bronchiectasis in CVID is rare it has occurred and can be successful. I question I would have is the experience of the transplant center with lung transplants in general and transplant in PIDD patients.
Successful Lung Transplantation in a CVID Patient with Recalcitrant Bronchiectasis
Heena Shah, MD, Prem Kumar, MD, FAAAAI; Louisiana State University, New Orleans, LA.
Rationale: It is well established that common variable immunodeficiency (CVID) can predispose affected people to frequent infections resulting in irreversible pulmonary disease. Treatment is primarily replacement therapy for hypogammaglobulinemia; however, morbidity is higher when organ dysfunction has already developed at the time of presentation. Literature regarding lung transplantation for refractory respiratory failure in CVID has variable results and it is currently not a commonly considered treatment modality.
Methods: A review of clinical and laboratory findings was performed both pre- and post-lung transplantation.
Results: A 52-year-old woman presented with a lifetime of frequent, severe sinopulmonary infections and CVID was confirmed. Treatment with intravenous immune globulin (IVIG) reduced infections but severe inflammatory damage to lung parenchyma persisted. Pulmonary function testing revealed an FEV1 of 33% and radiography showed bronchiectasis. Repeated antibiotic courses, oxygen supplementation and multiple rounds of pulmonary rehabilitation were required. After a severe bout of pneumonia and prolonged hospital stay led to a rapid deterioration in quality of life, transplant evaluation was initiated. Months later, bilateral allogenic lung transplantation was performed. Several months post-transplant, on immunosuppresive therapy, prophylactic antibiotics and IVIG replacement, our patient remains free of supplemental oxygen without dyspnea or evidence of rejection.
Conclusions: Underrecognition of CVID often leads to delay in diagnosis, and therefore, a delay in management of these patients. Awareness of respiratory complications and aggressive treatment can slow disease progression. However, evaluation of lung transplantation should be considered as a long-term therapeutic option for advancing respiratory failure with exhaustion of other treatment modalities.
Scand J Infect Dis. 2007;39(4):362-7.
Common variable immune deficiency and lung transplantation.
Burton CM1, Milman N, Andersen CB, Marquart H, Iversen M.
Abstract
We report on a male patient with bronchiectasis secondary to common variable immune deficiency (CVID) receiving lung transplantation. The patient had been diagnosed with CVID many y prior to right-sided single lung transplantation and was receiving appropriate immunoglobulin substitution therapy. He received antithymocyte globulin induction and maintenance triple therapy with cyclosporine, azathioprine and prednisolone. The early post-operative course was complicated by the development of severe acute cellular rejection and organizing pneumonia. Despite immunoglobulin replacement and antifungal prophylaxis and treatment, Aspergillus fumigatus was repeatedly cultured from bronchoalveolar lavage fluid, 18 months after transplantation. The patient died following a protracted period of repeated hospital admissions, 46 months after transplantation. A review of the literature suggests that many CVID patients appear to have had a complicated post-operative course after lung- and other solid-organ transplantation, and highlights the need for the establishment of international registries for transplanted patients with uncommon conditions.
1. M. Yeatman, K. NcNeil, J.A. Smithetal.,“Lung Transplantation in patients with systemic diseases: an eleven-year experience at Papworth Hospital,” e Journal of Heart and Lung Transplantation, vol. 15, no. 2, pp. 144–149, 1996.
2. A. T. Hill, R. A. Thompson, J. Wallwork, and D. E. Stableforth, “Heart lung transplantation in a patient with end stage lung disease due to common variable immunodeciency,” Thorax, vol. 53, no. 7, pp. 622–623, 1998.
3. C. M. Burton, N. Milman, C. B. Andersen, H. Marquart, and M. Iversen, “Common variable immunedeciency and lung transplantation,” Scandinavian Journal of Infectious Diseases, vol. 39, no. 4, pp. 362–367, 2007.
4. C. Cunningham-Rundles and C. Bodian, “Common variable immunodeciency: clinical and immunological features of 248 patients,” Clinical Immunology, vol. 92, no. 1, pp. 34–48, 1999.
5. P. A. Beirne, N. R. Banner, A. Khaghani, M. E. Hodson, and M. H. Yacoub, “Lung transplantation for non-cystic brosis bronchiectasis: Analysis of a 13-year experience,” Journal of Heart and Lung Transplantation, vol. 24, no. 10, pp. 1530–1535, 2005.
I hope this has been helpful
Andrew Murphy MD FAAAI