Relapse of Sarcoidosis After Lung Transplantation

Abstract

<h3>Introduction</h3> Sarcoidosis is a systemic granulomatous disease of unknown etiology. It most commonly affects the lungs, lymph nodes and skin, but the eyes, liver, heart and central nervous system can also be involved. Lung involvement is present in almost all patients with sarcoidosis. Prognosis is generally benign with more than half of patients entering spontaneous remission. However, a third of patients exhibit progressive disease culminating in organ dysfunction. Although sarcoidosis is not among the most frequent causes for lung transplantation, this can be the only therapeutic alternative in this subset of patients. <h3>Case Report</h3> The authors present the case of a 48-year-old caucasian male patient, with no relevant medical history, who had been diagnosed with sarcoidosis since 2006. Thoracic lymph nodes and lung parenchyma were affected on presentation, and screening showed ocular involvement as well. The patient experienced a flare in 2015, with worsened dyspnea and decline in FEV1 and DLCO values, and was started on prednisolone and methotrexate. Clinical stability for the next three years allowed suspension of methotrexate in 2018, while maintaining low dose steroids. However, new clinical and functional decline, as well as detection of cardiac involvement, prompted a six month course of infliximab in 2019. Due to continued worsening of lung function tests (LFTs) and progression to lung fibrosis, the patient underwent bilateral lung transplantation in June 2020 without major complications, and was discharged a month later. He remained asymptomatic and follow-up LFTs were stable until February 2021, eight months after the transplant, when a new fall in FEV1 was noticed. Flexible bronchoscopy and transbronchial biopsies (TBB) were performed, revealing minimal acute rejection (A1) and granulomas compatible with sarcoidosis. Methylprednisolone pulse therapy was administered, followed by weaning. FEV1 levels improved in subsequent evaluations, although TBB performed three months later continued to show signs of sarcoidosis. <h3>Summary</h3> Allograft relapse of sarcoidosis is relatively common - Schultz et al have reported a recurrence rate of 30%. Research by Ionescu et al has showed the granulomas originate from the recipient. Recurrence of sarcoidosis does not seem to impact survival or lung function of lung transplant recipients. This case illustrates coexistence of both acute rejection and recurrent sarcoidosis.