SESSION TITLE: Miscellaneous Cases III
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 03:00 PM - 04:00 PM
INTRODUCTION: Sarcoidosis is a multisystem inflammatory condition. Anti-tumor necrosis factor alpha (anti-TNF-α) therapy, infliximab has been reported to be useful in refractory pulmonary and extra-pulmonary sarcoidosis in some case series. Lung transplant is indicated for select patients with refractory end-stage pulmonary sarcoidosis.
CASE PRESENTATION: 41 year old African-American female with fibrocystic pulmonary sarcoidosis and severe pulmonary hypertension underwent sequential bilateral lung transplantation. Immunosuppression post transplant included induction therapy with Basiliximab and maintenance with Tacrolimus, Mycophenolate and Prednisone. Post transplant course was uncomplicated and she was discharged post-operative day 12. 1 month post transplant surveillance bronchoscopy showed severe, bilateral, diffuse airway obstruction with thick mucopurulent necrotic tissue. FEV1 was 1.53L (47% of predicted). Biopsies were negative for acute rejection. 3 month post transplant transbronchial and endobronchial biopsies showed extensive non-caseating granulomatous inflammation but no acute rejection. Her lung function continued to decline despite high dose corticosteroids. Monthly infusion of infliximab was added with dramatic improvement in FEV1 from 0.47L to 2.03L. Follow-up biopsies were negative for non-caseating granulomas. She is currently 13 month post transplant and on monthly infliximab and prednisone.
DISCUSSION: To our knowledge, this is the first reported case of early recurrent endo-bronchial sarcoidosis post lung transplant refractory to corticosteroids and other immunosuppressive agents successfully treated with infliximab. Sarcoidosis affects the respiratory system in >90% of cases, most commonly intra-thoracic lymph nodes and lung parenchyma with airways being less commonly involved. While histological recurrence of sarcoidosis is common after lung transplant, it is usually not of clinical significance. The treatment of sarcoid bronchostenosis is usually with corticosteroids, but with mixed results at best. Current guidelines recommend alternative immunosuppressive therapy when corticosteroids are not controlling the disease.
CONCLUSIONS: We have described a case of recurrent endobronchial sarcoidosis presenting within 90 days post lung transplant. She had persistent decline in her lung function despite aggressive immunosuppression but had significant improvement with Infliximab (anti-TNF-α therapy). Infliximab should be considered for refractory sarcoidosis not responding to other immunosuppressive agents.
Reference #1: Martinez FJ, et al. Recurrence of sarcoidosis following bilateral allogenic lung transplant . Chest. 1994;106(5):1597-9.
Reference #2: Baughman RP, et al. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med. 2006;174(7):795-802.
DISCLOSURE: The following authors have nothing to disclose: Sadia Shah, Farooq Cheema, Alan Betensley, Sana Quddus
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