Long-term experience with rituximab in anti-synthetase syndrome-related interstitial lung disease.
OBJECTIVE
To retrospectively evaluate the efficacy and safety of rituximab (Rtx) treatment in patients with anti-synthetase syndrome (ASS) and severe interstitial lung disease (ILD).


METHODS
Patients with severe ILD and >12 months follow-up post-Rtx were identified from the Oslo University Hospital ASS cohort (n = 112). Clinical data, including pulmonary function tests (PFTs), were retrospectively collected from medical reports. Extent of ILD pre-, and post-Rtx was scored on thin-section high-resolution CT (HRCT) images and expressed as a percentage of total lung volume. Muscle strength was evaluated by manual muscle testing of eight muscle groups (MMT8).


RESULTS
Altogether, 34/112 ASS patients had received Rtx; 24/34 had severe ILD and >12 months follow-up post-Rtx (median 52 months). In these 24 patients, the median percentage of predicted forced vital capacity, forced expiratory volume in 1 s (FEV1) and diffusing capacity of the lungs for carbon monoxide (DLCO) increased by 24%, 22% and 17%, respectively, post-Rtx. Seven patients (all with disease duration <12 months and/or acute onset/exacerbation of ILD) had >30% improvement in all three PFTs. HRCT analysis showed a median 34% reduction in ILD extent post-Rtx. MMT8 score increased post-Rtx. During follow-up, 7/34 (21%) Rtx-treated ASS patients died; 6/7 deaths were related to infections. The mortality rate in the Rtx-treated group was comparable to that of the remaining ASS cohort (25/78 deceased; 32%).


CONCLUSIONS
This study, which included 24 Rtx-treated ASS patients with severe ILD, reports improved PFTs after a median 52 months follow-up post-Rtx. The best outcome was observed in patients with a disease duration <12 months and/or acute onset/exacerbation of ILD. The study indicates that Rtx could be a treatment option for selected ASS patients, but infections should be given attention.
Institute of Clinical Medicine, Department of Rheumatology, helena.andersson@medisin.uio.no.
Helena
H
Andersson
Department of Rheumatology.
Marthe
M
Sem
Department of Respiratory Medicine.
May Brit
MB
Lund
Department of Radiology and.
Trond Mogens
TM
Aaløkken
Department of Radiology and.
Anne
A
Günther
Department of Orthopaedic Surgery, Oslo University Hospital, Oslo, Norway.
Ragnhild
R
Walle-Hansen
Department of Rheumatology.
Torhild
T
Garen
Institute of Clinical Medicine, Department of Rheumatology.
Øyvind
Ø
Molberg
D000328:Adult; D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008137:Longitudinal Studies; D008168:Lung / Q000000981:diagnostic imaging; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength / Q000502:physiology; D009220:Myositis / Q000150:complications* / Q000503:physiopathology; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D000069283:Rituximab; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D058846:Antibodies, Monoclonal, Murine-Derived; D018501:Antirheumatic Agents; D000069283:Rituximab
anti-Jo1; anti-aminoacyl tRNA synthetase; anti-synthetase syndrome; interstitial lung disease; myositis; rituximab