SCLERODERMA-ASSOCIATED PULMONARY ARTERIAL HYPERTENSION: EARLY DETECTION FOR BETTER OUTCOMES

Course overview

PULMONARY ARTERIAL HYPERTENSION (PAH) IS A RARE BUT POTENTIALLY FATAL COMPLICATION OF SCLERODERMA. EARLY SCREENING AND DIAGNOSIS OF PAH, FOLLOWED BY PROMPT INITIATION OF RISK-BASED TREATMENT, HAS BEEN SHOWN TO IMPROVE OUTCOMES AND DELAY PROGRESSION OF PAH. BECAUSE CLINICAL SYMPTOMS OF PAH ARE NONSPECIFIC, INCLUDING DYSPNEA, FATIGUE, WEAKNESS, CHEST PAIN, LIGHT-HEADEDNESS OR SYNCOPE, AND (INFREQUENTLY) COUGH, IT IS IMPORTANT TO SCREEN ALL PATIENTS WITH SCLERODERMA FOR PAH AT TIME OF DIAGNOSIS AND ANNUALLY THEREAFTER. RIGHT HEART CATHETERIZATION IS THE GOLD STANDARD DIAGNOSTIC TOOL FOR PAH. TREATMENT STRATEGIES ARE INFORMED BY THE PATIENT’S FUNCTIONAL CLASSIFICATION, USING ANY OF THE EXISTING RISK STRATIFICATION APPROACHES. THIS PROGRAM REVIEWS THE SCREENING AND DIAGNOSTIC APPROACHES FOR PAH, RISK STRATIFICATION, AND AVAILABLE THERAPIES FOR TREATMENT OF PAH.