AB0225CLINICAL SUBTYPE OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS
Authors: Natalia Yudkina, Alexander Volkov, Ekaterina Nikolaeva, Ildar Kurmukov, Evgeny Nasonov
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Despite the similar pathogenesis and clinical picture, pulmonary arterial hypertension in systemic sclerosis (PAH-SSc) in comparison with idiopathic pulmonary arterial hypertension (IPAH) is characterized by a more severe course, an unsatisfactory response to PAH-specific therapy, a poor survival and a disappointing prognosis. In this connection, before doctors of different specialties, first of all before rheumatologists, cardiologists and pulmonologists, there is a need to highlight the characteristics of the flow of PAH depending on the presence of SSc. <h3>Objectives</h3> To describe specific symptoms of PAH associated with SSc. <h3>Methods</h3> The study included 51 patients with PAH-SSc and 50 patients with IPAH formed the control group. To verify the diagnosis of PAH all patients underwent studies according to the program corresponding to the russian recommendations for the diagnosis and treatment of pulmonary hypertension with mandatory right heart catheterization. The diagnosis of SSc was established in accordance with the classification criteria ACR-EULAR 2013. Univariate logistic regression was used to calculate a probability (odds ratio (OR)) of SSc-associated symptoms. <h3>Results</h3> We identified 29 symptoms that were associated with PAH-SSc. The most significant of them, increasing the risk of detection of SSc in patients with PAH, were age over 45 years (OR 9.7, 95% CI 3.9-24.3, p &lt;0.00001), serum uric acid level&gt; 387 μmol/L (OR 3.8, 95% CI 1.5-9.6, p = 0.003), diffusion lung capacity (DLCO) &lt;60% (OR 13.8, 95% CI 4.3-44.7, p &lt;0.00001), ratio of forced vital capacity to DLCO &lt;1.7 (OR 13.0, 95% CI 3.9-42.9, p &lt;0.00001), level of C-reactive protein &gt;2 mg/l (OR 12.9, 95% CI 3.96-41.8, p &lt;0.00001), the presence of pericardial effusion (OR 2.3, 95% CI 1.0-5.1, p = 0.04), mean right ventricular pressure &gt; 15.5 mm Hg. (OR 8.9, 95% CI 3.4-23.1, p &lt;0.00001), diastolic right ventricular pressure &gt; 4.5 mm Hg. (OR 4.6, 95% CI 1.9-11.2, p = 0.0003). Symptoms that reduce the risk of detecting SSc in patients with PAH include mean pulmonary artery pressure &gt;55 mmHg. (OR 0.4, 95% CI 0.2-0.9, p = 0.03), pulmonary vascular resistance &gt;12 units Wood (OR 0.4, 95% CI 0.2-0.9, p = 0.02), hemoglobin level &gt;146 g/l (OR 0.3, 95% CI 0.2-0.8, p = 0.007), presense of syncope (OR 0.3, 95% CI 0.1-0.9, p = 0.02). <h3>Conclusion</h3> The selection of features of the course of PAH that increase the likelihood of detection of SSc will contribute to the timely diagnosis of SSc, will allow to predict the course of PAH, to approach more carefully the selection and monitoring of the effectiveness of PAH-specific therapy. That will contribute to improving the survival and functional status of patients with PAH-SSc. <h3>Disclosure of Interests</h3> Natalia Yudkina: None declared, Alexander Volkov: None declared, Ekaterina Nikolaeva: None declared, Ildar Kurmukov: None declared, Evgeny Nasonov Speakers bureau: Pfizer, Inc., MSD, Novartis, AbbVie Inc., Celgen Corporation, Biocad, Janssen, UCB, Inc.