Study (reference) | No. pts | Type of study | Refractory manifestations | RTX regimen | Follow up | Outcomes | Safety of RTX |
---|---|---|---|---|---|---|---|
Andersson et al. [12] | 34 | Retrospective | Progressive ILD (70.5%). Two-thirds of the patients also had signs of myositis, with elevated CK levels and/or reduced MMT8 scores | RA scheme | 52 m | Improvement in PFT (CVF increased by 24% and DLCO by 17%) and HRCT (the extent of ILD in HRCT scans decreased by 34%) post-RTX. MMT8 score increased from a median 93% of the maximum score pre-RTX to a median 98% post-RTX (P<0.05) | Serious infection: 18% Mortality: 21% The mortality rate in the RTX-treated group was comparable to that of the remaining ASS cohort |
Doyle TJ et al. [13] | 25 | Retrospective | Recurrent or progressive ILD (84%) Myositis (90.4%) Mechanic’s hands/rashes/arthritis (57%) | RA scheme | 1 to 3 yrs | Stability or improvement in PFT (FVC, TLC and DLCO) and HRCT in 88% and 79% of subjects, respectively. Further, there was a significant steroid-sparing effect (GC dose decreased from 18 ± 9 to 12 ± 12 mg/day) | No serious AE |
Langlois et al. [14] | 28 RTX vs. 32 CYC | Retrospective | ILD (100%) Muscle weakness (71%) Arthralgia/arthritis (76%) Cutaneous involvement (55%) | RA scheme | 2 yrs | Improved PFTs and HRCT score in both groups. RTX and CYC demonstrated similar pulmonary progression-free survival (PFS) at six months after treatment. However, RTX proved superior to CYC at 2 years of treatment (HR 0.263) | Similar AE |
Allenbach et al. [15] | 10 | Prospective Open label, phase II trial | 10 patients with refractory ASS Myositis (100%) ILD (100%) | RA scheme | 1 year | Improvement of PFT in 5 patients, stability in 4, and worsening in 1 Stable HRCT score 8 of 10 patients showed muscular improvement on MMT10 and normalization of creatine kinase levels The steroid dose was decreased in 6 patients | No serious AE |