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Pulmonary hypertension in Spanish patients with systemic sclerosis. Data from the RESCLE registry

AuthorsGarcía-Hernández, Francisco José; Castillo-Palma, María J.; Tolosa-Vilella, Carles; Guillén-del Castillo, Alfredo; Rubio-Rivas, Manuel; Freire, Mayka; Vargas-Hitos, José A.; Todolí-Parra, José A.; Rodríguez-Carballeira, Mónica; Espinosa, Gerard; Colunga-Argüelles, Dolores; Ortego-Centeno, N.; Trapiella-Martínez, Luis; Rodero-Roldán, María M.; Pla-Salas, Xavier; Perales-Fraile, Isabel; Pons-Martín del Campo, Isaac; Chamorro, Antonio J.; Fernández-de la Puebla Giménez, Rafael A.; Madroñero-Vuelta, Ana B.; Ruíz-Muñoz, Manuel; Fonollosa-Pla, Vicent; Simeón-Aznar, Carmen P.
KeywordsAnti-centromere antibodies
Pulmonary hypertension
Systemic sclerosis
Issue Date2019
PublisherSpringer Nature
CitationClinical Rheumatology 38: 1117-1124 (2019)
Abstract[Introduction]: Our objective was to evaluate the pulmonary hypertension (PH) data for Spanish patients with systemic sclerosis (SSc), define the PH types and determine the associated factors.
[Method]: Descriptive study of PH-related data from the multicentre RESCLE registry. Estimated systolic pulmonary artery pressure (esPAP), measured via echocardiogram was considered elevated if ≥ 35 mmHg. Left heart disease (LHD) and interstitial lung disease (ILD) were identified. When performed, data from right heart catheterisation (RHC) were collected.
[Results]: esPAP was elevated in 350 of 808 patients (43.3%). One hundred and forty-four patients (17.8%) were considered to have PH (88 via RHC and the rest due to elevated esPAP along with evidence of significant LHD or ILD): PAH 3.7%, secondary to ILD 8.3%, secondary to LHD 2.8% and unclassified 3%. Prevalence of elevated esPAP was greater in diffuse SSc (dSSc) than in limited scleroderma (lSSc) (50.5 vs. 42.2%, p 0.046). In the group with elevated esPAP, a lower prevalence of anti-centromere antibodies (41.9% vs. 52.3%, p 0.006) and a greater prevalence of anti-topoisomerase-1 antibodies (ATA) (25.1% vs. 18.6%, p 0.04) were observed compared to the group with normal esPAP. Patients with elevated esPAP had a lower rate of digital ulcers (50.6% vs. 60.2%, p 0.007) and esophageal involvement (83.6% vs. 88.7%, p 0.07) and higher rate of renal crisis (4.6% vs. 1.8%, p 0.066).
[Conclusions]: Prevalence of PAH was lower than expected (3.7%). Probability of having elevated esPAP was higher among patients with dSSc and among those with ATA.
Publisher version (URL)http://dx.doi.org/10.1007/s10067-018-4390-x
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