Antiphospholipid antibodies in chronic thromboembolic pulmonary hypertension

Zhu, Rui; Cheng, Gang-Yi; Denas, Gentian; Pengo, Vittorio

doi:10.1016/j.ejim.2023.01.012

Cited by 4 publications

(1 citation statement)

References 49 publications

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“…54 These antibodies are present in approximately 40% of patients with SLE, leading to a higher risk of developing APS in these patients. Furthermore, the prevalence of CTEPH is estimated to occur in 1.8 to 3.8% of patients with APS, 55,56 while the prevalence of positive aPLs in CTEPH has been estimated at 12% (8-17%) in a recent systematic review. 57 These data support the recent ESC/ERS guideline recommendation 8 to screen for aPLs in patients diagnosed with CTEPH.…”

Section: Other Mechanisms Of Ph In Non-ssc Ctdmentioning

confidence: 99%

Pulmonary Hypertension in Connective Tissue Diseases Other than Systemic Sclerosis

Budhram,

Weatherald,

Humbert

2024

Semin Respir Crit Care Med

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Pulmonary hypertension (PH) is a known complication of certain connective tissue diseases (CTDs), with systemic sclerosis (SSc) being the most common in the Western world. However, PH in association with non-SSc CTD such as systemic lupus erythematous, mixed connective tissue disease, and primary Sjögren's syndrome constitutes a distinct subset of patients with inherently different epidemiologic profiles, pathophysiologic mechanisms, clinical features, therapeutic options, and prognostic implications. The purpose of this review is to inform a practical approach for clinicians evaluating patients with non-SSc CTD-associated PH.The development of PH in these patients involves a complex interplay between genetic factors, immune-mediated mechanisms, and endothelial cell dysfunction. Furthermore, the broad spectrum of CTD manifestations can contribute to the development of PH through various pathophysiologic mechanisms, including intrinsic pulmonary arteriolar vasculopathy (pulmonary arterial hypertension, Group 1 PH), left-heart disease (Group 2), chronic lung disease (Group 3), chronic pulmonary artery obstruction (Group 4), and unclear and/or multifactorial mechanisms (Group 5). The importance of diagnosing PH early in symptomatic patients with non-SSc CTD is highlighted, with a review of the relevant biomarkers, imaging, and diagnostic procedures required to establish a diagnosis.Therapeutic strategies for non-SSc PH associated with CTD are explored with an in-depth review of the medical, interventional, and surgical options available to these patients, emphasizing the CTD-specific considerations that guide treatment and aid in prognosis. By identifying gaps in the current literature, we offer insights into future research priorities that may prove valuable for patients with PH associated with non-SSc CTD.

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