EJ-antisynthetase syndrome presenting as severe acute respiratory distress syndrome

Ralki, Mike; Langhe, Ellen De; Wilmer, Alexander; Hermans, Greet; Wauters, Joost; Meersseman, Philippe; Vos, Robin; Raemdonck, Dirk Van; Ceulemans, Laurens J.; Neyrinck, Arne; Peetermans, Marijke

doi:10.5588/ijtld.21.0115

Cited by 1 publication

(1 citation statement)

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“…Several cases have demonstrated its successful use in ASyS-ILD. [224][225][226][227][228][229][230][231] We consider this modality in patients that fulfil our center's selection criteria, especially for those awaiting lung transplantation. Additionally, we believe that the lung healing benefits of ECMO 232,233 complement immunosuppression therapy and provides sufficient time for it to take effect.…”

Section: Severe Disease and Critically Ill Patientsmentioning

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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