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Table 6 Main characteristics of the previous studies that have evaluated the efficacy and safety of Rituximab in the treatment of antisynthetase syndrome-related interstitial lung disease (AS-ILD)

From: Rituximab in the treatment of progressive interstitial lung disease associated with the antisynthetase syndrome

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

  1. CYC Cyclophosphamide, GC Glucocorticoids, HRCT thoracic high-resolution computed tomography, ILD Interstitial lung disease, MMT Manual muscle testing, %pFVC predicted forced vital capacity, %pDLCO predicted diffusing capacity for carbon monoxide, corrected for hemoglobin, PFT Pulmonary function tests, RA Rheumatoid arthritis, RTX Rituximab