Refractory anti-synthetase syndrome treated with rituximab

Ball, Elisabeth; Savage, Eimear; Pendleton, Adrian

doi:10.1093/rheumatology/kep438

Cited by 17 publications

(12 citation statements)

References 3 publications

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“…Recently its potential usefulness has been demonstrated in various autoimmune diseases, and several case reports and case series of rituximab have also been reported in myositis-ILD [93][94][95]. Sem et al reported 11 refractory ILD in anti-synthetase syndrome patients treated with rituximab and showed that rituximab stabilized and/or improved the ILD in 7 of 11 patients [93].…”

Section: ) Rituximabmentioning

confidence: 99%

Interstitial Lung Disease in Myositis: Clinical Subsets, Biomarkers, and Treatment

2012

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Interstitial lung disease (ILD) is the most frequent organ involvement (found in nearly half) of myositis patients, but it reveals various clinical courses and therapeutic responsiveness according to clinical and serological subsets. Autoantibodies, as well as imaging and histopathological studies, are useful for the classification of ILD in myositis and provide useful information for predicting prognosis and determining treatment. Antisynthetase antibodies are correlated with chronic and recurrent ILD, whereas anti-CADM-140 (MDA5/IFIH1) antibodies are a marker of acute progressive ILD in clinically amyopathic dermatomyositis. Serum KL-6, SP-D, and ferritin are useful biomarkers for monitoring the activity and severity of ILD. Regarding treatment, glucocorticoids are the first-line drug, but additional immunomodulating drugs are also used in refractory patients. Cyclophosphamide and calcineurin inhibitors (cyclosporine and tacrolimus) appear to be the key drugs in the treatment of refractory myositis-ILD. Rituximab may become another candidate if these drugs are not effective.

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Section: ) Rituximabmentioning

confidence: 99%

Interstitial Lung Disease in Myositis: Clinical Subsets, Biomarkers, and Treatment

2012

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“…We use cyclophosphamide for cases with clinically severe or rapidly progressive ILD. For patients in whom ILD worsens despite aggressive conventional therapy, rituximab has been used with moderate success, (83,84) with the stabilization or improvement of 7 of the 11 patients evaluated in one case series (Chart 3). (85) …”

Section: Therapy and Prognosismentioning

confidence: 99%

Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase

2011

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In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies marks the presence or predicts the development of interstitial lung disease (ILD). A distinct clinical entity—antisynthetase syndrome—is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud’s phenomenon, and mechanic’s hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.

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“…Favorable responses to RTX have been reported in case reports and retrospective uncontrolled studies, in particular ASSD-ILD [118][119][120][121][122][123][124]. Keir et al described four anti-Jo-1 patients with a good response to RTX in terms of respiratory function [122].…”

Section: Antisynthetase Syndrome (Assd)mentioning

confidence: 99%

Therapeutic Options for the Treatment of Interstitial Lung Disease Related to Connective Tissue Diseases. A Narrative Review

Vacchi

Sebastiani

Cassone

et al. 2020

JCM

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Interstitial lung disease (ILD) is one of the most serious pulmonary complications of connective tissue diseases (CTDs) and it is characterized by a deep impact on morbidity and mortality. Due to the poor knowledge of CTD-ILD’s natural history and due to the difficulties related to design of randomized control trials, there is a lack of prospective data about the prevalence, follow-up, and therapeutic efficacy. For these reasons, the choice of therapy for CTD-ILD is currently very challenging and still largely based on experts’ opinion. Treatment is often based on steroids and conventional immunosuppressive drugs, but the recent publication of the encouraging results of the INBUILD trial has highlighted a possible effective and safe use of antifibrotic drugs as a new therapeutic option for these subjects. Aim of this review is to summarize the available data and recent advances about therapeutic strategies for ILD in the context of various CTD, such as systemic sclerosis, idiopathic inflammatory myopathy and Sjogren syndrome, systemic lupus erythematosus, mixed connective tissue disease and undifferentiated connective tissue disease, and interstitial pneumonia with autoimmune features, focusing also on ongoing clinical trials.

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Refractory anti-synthetase syndrome treated with rituximab

Cited by 17 publications

References 3 publications

Interstitial Lung Disease in Myositis: Clinical Subsets, Biomarkers, and Treatment

Interstitial Lung Disease in Myositis: Clinical Subsets, Biomarkers, and Treatment

Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase

Therapeutic Options for the Treatment of Interstitial Lung Disease Related to Connective Tissue Diseases. A Narrative Review

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