Diagnosing and managing scleroderma-related pulmonary arterial hypertension.

JAAPA

Katherine Alexis Athanasiou practices rheumatology at the Arthritis Institute of Long Island in Hicksville, N.Y. At Northwell Health System in New Hyde Park, N.Y., Sonu Sahni is a clinical research physician, Amrinder Rana is a research volunteer, and Arunabh Talwar is a physician in the Department of Pulmonary, Critical Care, and Sleep Medicine and director of the Advanced Lung Disease Center. The authors have disclosed no potential conflicts of interest, financial or otherwise.

Published: September 2017

Scleroderma is an uncommon autoimmune disease of unknown cause that may affect any organ system in the body. Patients with scleroderma are prone to developing pulmonary complications, including pulmonary arterial hypertension (PAH), that are the leading cause of death in this population. This article describes scleroderma-related PAH and its diagnosis and management.

Download full-text PDF

Source
http://dx.doi.org/10.1097/01.JAA.0000522129.93995.13DOI Listing

Publication Analysis

Top Keywords

pulmonary arterial
8
arterial hypertension
8
diagnosing managing
4
managing scleroderma-related
4
scleroderma-related pulmonary
4
hypertension scleroderma
4
scleroderma uncommon
4
uncommon autoimmune
4
autoimmune disease
4
disease unknown
4

Similar Publications

Pulmonary hypertension (PH) is a term used to describe a complex heterogenous group of conditions defined by a mean pulmonary arterial pressure of more than 20 mmHg at rest on right-heart catheterization. PH in pregnancy is associated with high rates of maternal morbidity and mortality and poor fetal outcomes. Currently, pregnancy in these women is classified as modified WHO class IV (pregnancy contraindicated).

View Article and Find Full Text PDF

Pulmonary arterial hypertension in Latin America. The age and comorbidity paradox.

Int J Cardiol Congenit Heart Dis

March 2025

From the Cardiopulmonary Department, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico City, Mexico.

Pulmonary arterial hypertension (PAH) has been classically described as a disease in young adults, predominantly females with no comorbidities. However, in recent registries, the epidemiology has changed to older patients with comorbidities such as obesity, diabetes, systemic hypertension, and coronary heart disease. Nevertheless, there is not enough inclusion of these patients in clinical trials.

View Article and Find Full Text PDF

Background: Despite the development of effective pulmonary vasodilators, the prognosis for patients with pulmonary hypertension (PH) remains poor, particularly in medication-refractory patients. Catheter-based pulmonary artery denervation (PADN) is an emerging therapeutic strategy targeting the sympathetic nervous system in various types of PH. However, data on its safety and efficacy in refractory patients with PH who truly require non-pharmacotherapy are lacking.

View Article and Find Full Text PDF

Background: Because Regnase-1, encoded by , suppresses the development of pulmonary arterial hypertension (PAH) by controlling pro-inflammatory cytokines, we aimed to identify variants in patients with PAH.

Methods And Results: We analyzed whole-genome sequence data of patients with PAH to search for disease-associated variants. The Regnase-1 p.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!