"Tofacitinib Treatment in Refractory Systemic Lupus Erythematosus with Shrinking Lung Syndrome: A Case-Based Review"

Abstract

Shrinking Lung Syndrome (SLS) is a rare pulmonary complication of Systemic Lupus Erythematosus (SLE), which mainly manifests as dyspnea, pleural chest pain, and restrictive ventilation dysfunction, with or without an elevated hemi-diaphragm. Currently, there is no unified diagnostic criteria for SLS. SLS is a diagnosis of exclusion and can be diagnosed only in the absence of interstitial, alveolar and pulmonary vascular disease. The treatment is based on high-dose glucocorticoids and immunosuppressants. Here, we report a case of a 35-year-old woman who was diagnosed with SLE in 2008. Four years later, she was diagnosed with SLS due to chest pain, dyspnea, cough, and restrictive ventilatory dysfunction. Her condition improved after treatment with glucocorticoids and immunosuppressants but relapsed many times. She was treated with tofacitinib after relapse in May 2019, and then her condition improved and remains in remission with a four-year followup. We searched for and summarized 15 cases of patients with refractory SLE combined with SLS. They were all treated with biological agents. Tofacitinib is a new treatment choice for patients with refractory SLE with SLS.