Rituximab as the first-line therapy in anti-synthetase syndrome-related interstitial lung disease.

Abstract

Anti-synthetase syndrome (ASS) is an idiopathic inflammatory myopathy (IIM). In comparison to interstitial lung disease (ILD) in polymyositis and dermatomyositis (PM/DM), ILD in ASS is more frequent, has a more aggressive phenotype, a greater involvement of the lungs, and a more rapid onset of pulmonary symptoms. Continuous declines in predicted forced vital capacity (FVC) and dyspnea were the main features of patients who developed end-stage ILD. The severity of ASS at diagnosis dictates when and which immunosuppressant will be started. There is an experience for the usage of RTX in the first, second, and subsequent lines, as well as for reintroduction and salvage therapies. Not all ASS patients will develop severe illness and require intense immunosuppression. Some features associated with poor prognosis include older age, acute or subacute onset, lack of response to steroids, and lower baseline values for FVC and DLCO. Here we hypothesize that RTX should be the first line of treatment for high-risk ILD in ASS to preserve lung function and then maintenance therapy should be continued with the same or another drug depending on the recovery of lung function.