Rituximab on lung, skin, and joint involvement in patients with systemic sclerosis: A case series study and review of the literature

Objectives To evaluate the effectiveness of rituximab (RTX) in systemic sclerosis (SSc) patients. Methods Data were collected from patient charts before and after RTX administration for 1 year of follow-up time. An updated review of the literature was also done. Results Of 8 patients enrolled (mean age: 62.4 years; mean disease duration: 16.7 years), 2 patients with pulmonary arterial hypertension (PAH) died after the first RTX cycle. The follow-up data of the remaining 6 patients were evaluated. There was a significant improvement in arthritis of Disease Activity Score of 28 joints - C-reactive protein and Clinical Disease Activity Index compared with baseline. The median change in modified Rodnan Skin Score (mRSS), forced vital capacity (FVC), and carbon monoxide diffusing capacity between baseline and 12 months were similar. Lung involvement was detected in 5/6 of survivor patients, FVC was improved in 2/5, worsened in 1/5, and remained stable in 2/5 at the end of 1 year. Among the 5 diffuse cutaneous SSc patients, none of the patients' mRSS deteriorated by more than 5 points, while one patient's mRSS improved by greater than 5 points. Conclusion This study suggests that RTX is effective for arthritis in patients with SSc. Also, the effectiveness of RTX in skin and lung involvement of SSc was predominantly toward stable disease or improvement. Despite the long disease duration, the presence of patients who showed improvement in skin and lung involvement after RTX treatment suggests the need to investigate predictors of RTX response.