Background Interstitial lung disease (ILD) is the most characteristic feature and prognosis determinant of patients with antisynthetase (AS) and anti-MDA5 syndromes. Despite immunosuppressive treatment, ILD sometimes progresses to an end-stage lung disease, for which lung transplantation (LT) is the only therapeutic option. There is scarce data about post-LT outcome in this group of patients.
Objectives To describe clinical characteristics and post-LT outcome of patients with ILD associated to AS and anti-MDA5 syndromes included in the LT program of the Vall d'Hebron Hospital of Barcelona.
Methods We performed a review of patient records listed in the LT program.
Results From 1990 to 2016 ten patients (5 women) with ILD related to AS or anti-MDA5 syndromes were included in the LT program (3% of patients with ILD accepted for LT). Nine patients (2 anti-MDA5, 4 anti-Jo1, 2 anti-PL12 and 1 anti-PL7) received LT while 1 patient (anti-Jo1) was still in list at the end of this study. Median age (range) of disease diagnosis was 39 years (25–55). Six patients had clinical myopathy [2 dermatomyositis (DM) and 4 polymyositis] whereas 1 patient was diagnosed with amyopathic DM. Four patients had associated pulmonary hypertension. Time between disease diagnosis and patient inclusion in LT list was higher in anti-Jo1 patients [median (range) 8.8 years (8.3–17.6)] than in the rest of the cohort [anti-MDA5 <1 year; antiPL12 0.7 and 3.9 years; anti-PL7 1.3 years] (p<0.05). Four patients underwent bilateral LT and 5 unilateral LT. Three patients received an urgent LT (2 anti-MDA5 and 1 anti-Jo1). Six patients presented an histologic pattern of UIP and 3 of NSIP. Early complications (<45 days) included: primary graft disfunction in 3 patients; phrenic paresis in 2 patients and infection in 6 patients. One patient suffered an acute rejection (AR). Six patients presented late complications (>45 days): one patient developed an acute cellular rejection (ACR) and an autoantibody mediated rejection 8 and 10 months from LT respectively; 4 patients suffered chronic lung allograft dysfunction (CLAD), 2 of whom are still alive; Infection was diagnosed in 6 patients. One patient developed a squamous skin carcinoma. No flare of myopathic or lung disease was observed after LT in any case. Four patients (44%) died: 2 of an acute respiratory failure in the immediate postoperatory period (1 caused by suture dehiscence and 1 by refractory ACR); 1 of an invasive aspergillosis, 17 months after LT; and 1 of CLAD after 54 months. Median follow-up (range) of the rest of the cohort was 35 months (8–70). All patients who survived more than 45 days recovered an optimal functional capacity for daily activities with no request for long-term oxygen therapy.
Conclusions LT should be considered a valid option to treat patients with end-stage or severe and rapidly progressive ILD associated to AS and anti-MDA5 syndromes. An early remission to LT referral centers for evaluation should be considered especially in non-Jo1 patients. No relapse of myositis or ILD was observed after LT. Mortality could not be attributed to the primary disease.
Disclosure of Interest None declared
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