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SAT0339 Lung transplantation in patients with interstitial lung disease associated with antisynthetase and anti-mda5 syndromes. experience from a reference spanish hospital
  1. E Trallero-Araguás1,
  2. C Berastegui2,
  3. M Lόpez-Corbeto1,
  4. I Bello3,
  5. A Román2,
  6. A Selva-O'Callaghan4
  1. 1Rheumatology Unit
  2. 2Pneumology Department
  3. 3Thoracic Surgery Department
  4. 4Internal Medicine Department, Hospital Vall d'Hebron, Barcelona, Spain

Abstract

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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