Characteristics and risk factors for pulmonary arterial hypertension associated with primary Sjögren's syndrome: 15 new cases from a single center

International Journal of Rheumatic Diseases
Na ZhangW Wei

Abstract

This study aimed to retrospectively describe 15 new primary Sjögren's syndrome-pulmonary arterial hypertension (pSS-PAH) cases confirmed by right heart catheterization (RHC). Demographic and clinical characteristics were analyzed and risk factors for PAH in pSS were explored. We retrospectively described 15 new pSS-PAH cases confirmed by RHC referred to our institution between January 2013 and March 2018. We present PAH and pSS characteristics, hemodynamic evaluations, medical management, and outcomes. A matched case control study was carried out to determine the risk factors of PAH in pSS compared with pSS-non-PAH patients. All patients were female with a mean age at PAH diagnosis of 52.9 ± 14.6 years. The delay between the first symptom and PAH diagnosis was 18.7 ± 19.7 months. The most common primary manifestation at PAH onset was exertional dyspnea (13/15). At diagnosis of PAH, PAH was severe with a mean pulmonary artery pressure of 48.8 ± 13.7 mm Hg (range, 27-72 mm Hg) and a mean cardiac index of 2.3 ± 0.6 L/min/m2 (range, 1.47-3.41 L/min/m2 ). Compared with the pSS-PAH without pericardial effusion, pSS-PAH with pericardial effusion had larger right arterial (53 [45-56.75] vs 38 [35.5-46.5], P = .018) and right ventricula...Continue Reading

References

Mar 30, 2002·Journal of the American College of Cardiology·Ronald J RaymondWalker Long
May 15, 2002·Annals of the Rheumatic Diseases·C VitaliUNKNOWN European Study Group on Classification Criteria for Sjögren's Syndrome
Dec 14, 2002·The American Journal of Cardiology·George T LauLeonard Kritharides
Nov 3, 2005·The European Respiratory Journal·M M HoeperJ Niedermeyer
May 15, 2008·Respiration; International Review of Thoracic Diseases·Demosthenes BourosArgyris Tzouvelekis
Oct 23, 2010·Revue des maladies respiratoires·O SanchezG Meyer
Dec 2, 2010·European Respiratory Review : an Official Journal of the European Respiratory Society·O Sitbon, N Galiè
Jun 22, 2012·The Canadian Journal of Cardiology·Avi ShimonyLawrence G Rudski
Sep 4, 2012·European Respiratory Review : an Official Journal of the European Respiratory Society·Olivier SitbonPilar Escribano
Dec 21, 2013·Journal of the American College of Cardiology·Michael D McGoonMarc Humbert
Dec 21, 2013·Journal of the American College of Cardiology·Vallerie V McLaughlinOlivier Sitbon
Mar 13, 2014·Pulmonary Circulation·Sandeep Sahay, Adriano R Tonelli
Apr 18, 2014·Annals of the Rheumatic Diseases·Ronald F van VollenhovenMatthias Schneider
May 6, 2014·The European Respiratory Journal·Yan-Jie HaoZhuo-Li Zhang
Nov 30, 2014·Annals of the Rheumatic Diseases·P Brito-ZerónUNKNOWN SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI)
May 27, 2015·The Turkish Journal of Gastroenterology : the Official Journal of Turkish Society of Gastroenterology·Kumral ÇağlıÖmer Başar
Apr 7, 2018·International Journal of Rheumatic Diseases·Shumin YanXiaofeng Zeng
Jan 13, 2019·The European Respiratory Journal·Junyan QianUNKNOWN following investigators were collaborators in the CSTAR-PAH study:

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Citations

Aug 14, 2021·Rheumatology International·Li QinHan Wang

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