Pleuroparenchymal fibroelastosis in mycobacterium avium complex lung disease

Sugino, Keishi; Ono, Hirotaka; Ando, Masahiro; Tsuboi, Eiyasu

doi:10.1002/rcr2.1084

Cited by 1 publication

(1 citation statement)

References 2 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Tofacitinib, a Janus kinase inhibitor, has been beneficial in treating cutaneous manifestations in refractory DM. 181 Explorations for its use in anti-MDA-5-related ILD 182,183 and ASyS-ILD 184,185 have been limited and more research is needed. Abatacept, an inhibitor of anti-CD80/CD-86 which prevents T cell activation, was evaluated in a retrospective report of eight patients with ASyS-ILD.…”

Section: Other Therapiesmentioning

confidence: 99%

Review of Pulmonary Manifestations in Antisynthetase Syndrome

Ghanbar,

Danoff

2024

Semin Respir Crit Care Med

View full text Add to dashboard Cite

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.

show abstract