Syndrome des antisynthétases compliqué d’une hypertension pulmonaire : 4 observations originales

Lecomte, R.; Perrin, F; Journeau, L.; Espitia, O.; Piriou, N.; Horeau-Langlard, D; Néel, A.; Masseau, A.; Hamidou, M.; Agard, C.

doi:10.1016/j.revmed.2015.03.013

Cited by 10 publications

(4 citation statements)

References 30 publications

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“…[9,18,19,26–33] Recently, our team identified 16 cases of hemodynamically-proven PH among 203 consecutive patients presenting with ASS, a condition characterized by the presence of anti-tRNA synthetase antibodies and associated with IIM and ILD. [9] Almost all of them had extensive ILD according to Goh criteria, [34] with marked limitation of functional capacities (NYHA functional class II–III; mean 6MWT distance ± standard deviation: 59% ± 19% of predicted value).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary arterial hypertension in idiopathic inflammatory myopathies

et al. 2016

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Section: Discussionmentioning

confidence: 99%

Pulmonary arterial hypertension in idiopathic inflammatory myopathies

et al. 2016

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“…103 Regardless, PH in ASyS is often attributed to group 3 disease owing to the extensive lung involvement in many patients and limited reports of group 1 (connective tissue disease [CTD]-related) disease exists. [104][105][106][107][108] In reports where pulmonary arterial hypertension (PAH) is diagnosed, this is often due to either limited parenchymal disease and/or hypoxemia, or when the PH is out of proportion to the degree of ILD. The prevalence of PH in ASyS can be overestimated when patients are solely screened with transthoracic echocardiography (TTE), as demonstrated by Abel et al 104 In their cohort, there was TTE evidence of PH in 23%.…”

Section: Pulmonary Vascular Diseasementioning

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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“…Nous avons lu avec intérêt l'article de Lecomte et al [1] rapportant 4 nouveaux cas d'hypertension pulmonaire (HTP) survenant dans le contexte de syndrome des antisynthétases et soulignant l'importance de dépister cette complication rare mais sévère. Cependant, cet article entretient une confusion majeure sur la classification et la prise en charge des HTP associées au syndrome des antisynthétases qu'il semble important de commenter.…”

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“…Comme cela est rapporté dans l'analyse de la littérature faite par Lecomte et al [1], tous les cas d'HTP associée au syndrome des antisynthétases, surviennent dans un contexte de pneumopathie interstitielle diffuse, souvent évoluée. L'existence d'une atteinte vasculaire pulmonaire isolée (sans maladie respiratoire) n'a pas été rapportée à ce jour, à la différence de ce qui est observé dans l'HTAP associée à la sclérodermie systémique.…”

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