Successful Treatment for Refractory Interstitial Lung Disease and Pneumomediastinum With Multidisciplinary Therapy Including Tofacitinib in a Patient With Anti-MDA5 Antibody-Positive Dermatomyositis

Kato, Masumi; Ikeda, Kei; Kageyama, Takahiro; Kasuya, Tadamichi; Kumagai, Toru; Furuya, Hiroki; Furuta, Shunsuke; Tamachi, Tomohiro; Suto, Akira; Suzuki, Kotaro; Nakajima, Hiroshi

doi:10.1097/rhu.0000000000000984

Cited by 52 publications

(32 citation statements)

References 14 publications

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“…The anti-MDA5 autoantibody subgroup of JDM is characterized by increased cutaneous ulceration and interstitial lung disease (ILD) with less muscle disease [1][2][3], also observed in our cohort, with more overlap with these vasculopathic features seen in SAVI than CANDLE in general [15][16][17], also specifically observed in our cohort. JAK inhibitor therapy has been noted to show improved outcomes in refractory anti-MDA5 autoantibody adult and juvenile dermatomyositis, including with rapidly progressive ILD [36][37][38].…”

Section: Discussionmentioning

confidence: 99%

Expression of interferon-regulated genes in juvenile dermatomyositis versus Mendelian autoinflammatory interferonopathies

et al. 2020

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Background: Juvenile dermatomyositis (JDM) is a systemic autoimmune disease with a prominent interferon (IFN) signature, but the pathogenesis of JDM and the etiology of its IFN signature remain unknown. The Mendelian autoinflammatory interferonopathies, Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temperature (CANDLE) and STING-Associated Vasculopathy with onset in Infancy (SAVI), are caused by genetic mutations and have extremely elevated IFN signatures thought to drive pathology. The phenotypic overlap of some clinical features of CANDLE and SAVI with JDM led to the comparison of a standardized interferon-regulated gene score (IRG-S) in JDM and myositis-specific autoantibody (MSA) JDM subgroups, with CANDLE and SAVI. Methods: A peripheral 28-component IRG-S assessed by NanoString™ in 57 JDM patients subtyped by MSA was compared with IRG-S in healthy controls (HC) and CANDLE/SAVI patients. Principal component analysis (PCA) was performed, and individual genes were evaluated for their contribution to the score. IRG-S were correlated with disease assessments and patient characteristics. Results: IRG-S in JDM patients were significantly higher than in HC but lower than in CANDLE or SAVI. JDM IRG-S overlapped more with SAVI than CANDLE by PCA. Among MSA groups, anti-MDA5 autoantibody-positive patients' IRG-S overlapped most with SAVI. The IFI27 proportion was significantly higher in SAVI and CANDLE than JDM, but IFIT1 contributed more to IRG-S in JDM. Overall, the contribution of individual interferon-regulated genes (IRG) in JDM was more similar to SAVI. IRG-S correlated moderately with JDM disease activity measures (r s = 0.33-0.47) and more strongly with skin activity (r s = 0.58-0.79) in anti-TIF1 autoantibody-positive patients. Weakness and joint disease activity (multinomial OR 0.91 and 3.3) were the best predictors of high IRG-S.

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Section: Discussionmentioning

confidence: 99%

Expression of interferon-regulated genes in juvenile dermatomyositis versus Mendelian autoinflammatory interferonopathies

et al. 2020

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“…Successful use of tofacitinib has recently been described for ILD associated with anti-melanoma differentiation-associated protein 5 (anti-MDA5)-positive amyopathic dermatomyositis [172,173].…”

Section: Targeted Synthetic Disease-modifying Antirheumatic Drugs (Tsmentioning

confidence: 99%

Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Lights and Shadows

Cassone

Manfredi

Vacchi

et al. 2020

JCM

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Rheumatoid arthritis (RA) is a chronic and systemic inflammatory disease affecting 0.5–1% of the population worldwide. Interstitial lung disease (ILD) is a serious pulmonary complication of RA and it is responsible for 10–20% of mortality, with a mean survival of 5–8 years. However, nowadays there are no therapeutic recommendations for the treatment of RA-ILD. Therapeutic options for RA-ILD are complicated by the possible pulmonary toxicity of many disease modifying anti-rheumatic drugs (DMARDs) and by their unclear efficacy on pulmonary disease. Therefore, joint and lung involvement should be evaluated independently of each other for treatment purposes. On the other hand, some similarities between RA-ILD and idiopathic pulmonary fibrosis and the results of the recent INBIULD trial suggest a possible future role for antifibrotic agents. From this perspective, we review the current literature describing the pulmonary effects of drugs (immunosuppressants, conventional, biological and target synthetic DMARDs and antifibrotic agents) in patients with RA and ILD. In addition, we suggest a framework for the management of RA-ILD patients and outline a research agenda to fill the gaps in knowledge about this challenging patient cohort.

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“…141,142 Its benefit as an additional therapy to CYC or RTX in refractory ILD was also demonstrated in recent case reports and case series. [143][144][145] Abatacept is currently being assessed in a randomized controlled phase II trial to evaluate its efficacy, safety, and tolerability in ASSD associated ILD.…”

Section: Treatment In Assd: Focus On Ildmentioning

confidence: 99%

Antisynthetase syndrome: A distinct disease spectrum

Huang

Aggarwal

2020

Journal of Scleroderma and Related Disorders

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The discovery of novel autoantibodies related to idiopathic inflammatory myopathies (collectively referred to as myositis) has not only advanced our understanding of the clinical, serological, and pathological correlation in the disease spectrum but also played a role in guiding management and prognosis. One group of the myositis-specific autoantibodies is anti-aminoacyl-tRNA synthetase (anti-ARS or anti-synthetase) which defines a syndrome with predominant interstitial lung disease, arthritis, and myositis. Autoantibodies to eight aminoacyl-tRNA synthetases have been identified with anti-Jo1 the most common in all of idiopathic inflammatory myopathies. Disease presentation and prognosis vary depending on which anti-aminoacyl-tRNA synthetase antibody is present. In this review, we will discuss the clinical characteristics, overlap features with other autoimmune diseases, prognostic factors, and management of the antisynthetase syndrome.

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Successful Treatment for Refractory Interstitial Lung Disease and Pneumomediastinum With Multidisciplinary Therapy Including Tofacitinib in a Patient With Anti-MDA5 Antibody-Positive Dermatomyositis

Cited by 52 publications

References 14 publications

Expression of interferon-regulated genes in juvenile dermatomyositis versus Mendelian autoinflammatory interferonopathies

Expression of interferon-regulated genes in juvenile dermatomyositis versus Mendelian autoinflammatory interferonopathies

Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Lights and Shadows

Antisynthetase syndrome: A distinct disease spectrum

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