Interstitial Lung Disease and Pulmonary Arterial Hypertension in Overlap Syndrome: A Case Report

Abstract

Case Presentation: A 42-year-old female was diagnosed with overlap syndrome consisting of systemic lupus erythematous (SLE), systemic sclerosis (SSc), and rheumatoid arthritis (RA); serologic profile included positive anti-nuclear antibody (ANA), anti-dsDNA, anti-RNP, anti-Ro, anti-Scl70, anti-Sm, rheumatoid factor and hypocom-plementemia (C3, C4). She had chronic stable interstitial lung disease (ILD) for 17 years maintained on hydroxychloroquine (HCQ), pred-nisone 5 mg/day and indacaterol. The current admission was due to progressive dyspnea and right-sided heart failure over the past month. Chest radiograph showed pulmonary congestion, and 2-dimensional echocardiography (2DE) disclosed severe pulmonary arterial hypertension (PAH) with systolic pulmonary arterial pressure (SPAP) of 76 mmHg by tricuspid regurgitation (TR) jet, dilated right ventricle (RV) with poor systolic function, moderate pericardial effu-sion with no signs of tamponade. She received furosemide, pred-nisone increased to 20 mg/day, beraprost and sildenafil. Two weeks following discharge, there was complete resolution of symptoms and repeat 2DE showed non-dilated RV with good systolic function, normal SPAP of 21.4 mmHg and minimal pericardial effusion. Pred-nisone was tapered to 5 mg/day; beraprost, sildenafil and HCQ were continued. Conclusion: Overlap syndrome was diagnosed by the combination of clinical features and serology distinctive of SLE, SSc and RA. Her illness, particularly ILD, was adequately controlled over several years while maintained on prednisone 5 mg/day and HCQ, until the recent onset of PAH complicated by right-sided heart failure. The dramatic response to high-dose steroids is more consistent with inflammatory vasculitis of SLE activity rather than fibrosis typical of SSc.