Background/Purpose: Clinical manifestations of anti-synthetase syndrome (ASS) include fever, Raynaud phenomenon, mechanic’s hands, inflammatory arthritis, myositis and interstitial lung disease (ILD). Pulmonary hypertension (PH), although not part of the syndrome, when present may indicate a poor prognosis and has not been well studied.

Methods: We conducted a retrospective chart review of patients with ASS with Jo-1, PL-7, PL-12, EJ and OJ antibodies between 2003 – 2017. ASS patients with PH were selected for further review. PH was diagnosed by right heart catheterization (RHC) in all patients with mean pulmonary arterial pressure (mPAP) ≥25 mmHg, and defined as severe if mPAP was ≥35 mmHg. Patients were classified as having pre-capillary PH if pulmonary capillary wedge pressure (PCWP) was ≤15 mmHg, post-capillary PH if PCWP was >15 mmHg, and combined pre- and post-capillary PH if PCWP was >15 mmHg with diastolic pressure gradient ≥7 mmHg and/or pulmonary vascular resistance (PVR) >3 Wood units. Patient demographics, laboratory data, echocardiogram and RHC parameters, spirometry and DLCO were evaluated. Numerical variables were compared using Mann-Whitney test. Categorical variables were compared using either Pearson’s chi-squared test or Fisher’s exact test. Binary logistic regression analysis was used to identify factors associated with mortality. Survival function was estimated using the Kaplan-Meier method. A p-value of <0.05 was considered significant.

Results: We identified 177 patients with positive anti-Jo-1 antibody, and 36 patients with non-Jo-1 antibodies. Twenty-two (10.33%) had PH and the following antibodies: Jo-1 (18), PL-12 (2), EJ (1) and PL-7 (1). The median age was 51 years and the median follow-up duration was 6.89 years. The median duration from ASS onset to PH diagnosis was 36.17 months. Fifteen patients had pre-capillary PH, 3 had post-capillary PH and 4 had combined PH. Median forced vital capacity (FVC) at PH diagnosis was 48%, of which 3 patients had FVC ≥70%. One patient had PH without ILD. Sixteen patients (72.7%) had severe PH. Sixteen out of 19 patients with pre-capillary PH were treated for pulmonary arterial hypertension and 9 of them received combination therapy. All 22 patients also received immunosuppressive therapy. Twelve patients (54.5%) died. Two patients with lung transplants survived. The deceased group had higher PVR (p= 0.024) and higher rates of severe PH (p=0.05). Right ventricular dilation was associated with mortality (p= 0.046). Survival was not affected by SS-A antibody positivity, presence of Raynaud phenomenon or pericardial effusion, New York Heart Association Functional Class, FVC at PH diagnosis, and duration from ASS onset to PH diagnosis. Survival analysis is shown in Figure 1.

Conclusion: PH is not uncommon in patients with ASS. Severity of PH at diagnosis was a predictor of mortality. Given this dismal prognosis, we recommend screening echocardiography in all ASS patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-hypertension-in-anti-synthetase-syndrome/