Therapeutic plasma exchange for steroid refractory idiopathic inflammatory myopathies with interstitial lung disease

Thompson, Thomas Z.; Bobr, Aleh; Juskewitch, Justin E.; Winters, Jeffrey L.

doi:10.1002/jca.22034

Cited by 4 publications

(4 citation statements)

References 44 publications

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“…The overwhelming majority of the data are for its use in clinically amyopathic DM and specifically anti-MDA5 disease. 188,189 However, there are case reports [190][191][192] and one case series 193 showing favorable outcomes in those with ASyS-ILD. Other therapies that have been trialed for IIM-ILD include antitumor necrosis factor-α, 194,195 tocilizumab, [196][197][198] and anakinra.…”

Section: Other Therapiesmentioning

confidence: 99%

Review of Pulmonary Manifestations in Antisynthetase Syndrome

Ghanbar,

Danoff

2024

Semin Respir Crit Care Med

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Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad of myositis, arthritis, and interstitial lung disease (ILD), its presentation can be diverse. Additional common symptoms experienced by patients with ASyS include Raynaud's phenomenon, mechanic's hand, and fever. Although there is a significant overlap with polymyositis and dermatomyositis, the key distinction lies in the presence of antisynthetase antibodies (ASAs). Up to 10 ASAs have been identified to correlate with a presentation of ASyS, each having manifestations that may slightly differ from others. Despite the proposal of three classification criteria to aid diagnosis, the heterogeneous nature of patient presentations poses challenges. ILD confers a significant burden in patients with ASyS, sometimes manifesting in isolation. Notably, ILD is also often the initial presentation of ASyS, requiring pulmonologists to remain vigilant for an accurate diagnosis. This article will comprehensively review the various aspects of ASyS, including disease presentation, diagnosis, management, and clinical course, with a primary focus on its pulmonary manifestations.

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Section: Other Therapiesmentioning

confidence: 99%

Review of Pulmonary Manifestations in Antisynthetase Syndrome

Ghanbar,

Danoff

2024

Semin Respir Crit Care Med

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“…Therapeutic Apheresis Apheresis involves the extracorporeal removal, return, or exchange of large substances from the blood or plasma. Two case reports have demonstrated its use in ASyS refractory to steroids and other therapeutic agents [143,144]. 9.9.2.…”

Section: Intravenous Immunoglobulinmentioning

confidence: 99%

A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease

Patel,

Marinock,

Ajmeri

et al. 2024

IJMS

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Our objective in this review article is to present a clinical case of a patient with antisynthetase syndrome (ASyS) and provide an overview of the pathogenesis, classification criteria, antibody profiles, clinical features, and current knowledge of treatment options, focusing on interstitial lung disease (ILD). ASyS is an uncommon autoimmune disease with a heterogenous clinical presentation characterized by the presence of autoantibodies against an aminoacyl-tRNA synthetase and manifested by myositis, fever, inflammatory arthritis, Raynaud’s phenomenon, mechanics hands, and ILD. ASyS-associated ILD (ASyS-ILD) is the most serious complication of ASyS, which may evolve to rapidly progressive ILD; therefore, it often requires thorough clinical and radiologic evaluation including recognition of a specific clinical phenotype associated with the antisynthetase antibodies (ASAbs) to guide therapeutic interventions.

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“…Although there is biologic plausibility for the treatment of IIM-ILD/AS-ILD or anti-MDA5+ ILD with IVIG and PEX, there is limited evidence [62–64]. However, they may have less risk of infection compared with other regimens.…”

Section: Treatmentmentioning

confidence: 99%

“…Although there is biologic plausibility for the treatment of IIM-ILD/AS-ILD or anti-MDA5þ ILD with IVIG and PEX, there is limited evidence [62][63][64].…”

Section: Intravenous Immunoglobulin and Plasma Exchangementioning

confidence: 99%

Myositis-associated interstitial lung disease

Trang

Brown

Solomon

2023

Current Opinion in Pulmonary Medicine

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Purpose of review In idiopathic inflammatory myopathies (IIMs), interstitial lung disease (ILD) is common and the autoantibody profile, made up of myositis-specific and myositis-associated (MSA and MAA) antibodies, can predict the clinical phenotype and progression over time. This review will focus on the characteristics and management of antisynthetase syndrome related ILD and anti-MDA5 positive ILD, which are the most clinically relevant subtypes. Recent findings The prevalence of ILD in IIM has been estimated in Asia, North America and Europe at 50, 23 and 26%, respectively, and is increasing. In antisynthetase syndrome related ILD, the clinical presentation, progression and prognosis varies among anti-ARS antibodies. ILD is more common and severe in patients with anti-PL-7/anti-PL-12 antibodies when compared with anti Jo-1 patients. The prevalence of anti-MDA5 antibodies is higher in Asians (11–60%) than in whites (7–16%). Sixty-six percent of antisynthetase syndrome patients had ‘chronic ILD’ compared with the more rapidly progressive ILD (RP-ILD) seen in 69% of patients with anti-MDA5 antibodies. Summary ILD is most common in the antisynthetase subtype of IIM and can be a chronic indolent or RP- ILD. The MSA and MAAs are associated with different clinical phenotypes of ILD. Treatments typically involve combinations of corticosteroids and other immunosuppressants.

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Therapeutic plasma exchange for steroid refractory idiopathic inflammatory myopathies with interstitial lung disease

Cited by 4 publications

References 44 publications

Review of Pulmonary Manifestations in Antisynthetase Syndrome

Review of Pulmonary Manifestations in Antisynthetase Syndrome

A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease

Myositis-associated interstitial lung disease

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