Anti-synthetase syndrome with lung involvement associated with primary biliary cirrhosis: A case report

SummaryWe here report a case of association between primary biliary cirrhosis and anti-synthetase syndrome with exclusive interstitial lung involvement. The patient developed exertional dyspnoea 14 years after being diagnosed with primary biliary cirrhosis. Search for auto-antibodies showed positivity for anti-Jo-1. No clinically evident myositis was present. HRCT scan showed bilateral interstitial pulmonary involvement, and the BAL yielded an alveolar lymphocytosis with low CD4/CD8 ratio. Treatment with systemic corticosteroids led to rapid improvement of the clinico-radiological picture. Although primary biliary cirrhosis had been previously found in association with polymyositis, no previous reports of its association with anti-Jo-1 syndrome with prominent pulmonary involvement are known