Volume 20: Issue 3

Systemic sclerosis (SSc) is a disease state characterized by a significant lifetime risk for the development of pulmonary hypertension (PH). SSc-associated PH is comprised of a heterogenous array of phenotypes. SSc patients are at risk for both precapillary and postcapillary PH conditions. These include pulmonary arterial hypertension (PAH), PH secondary to interstitial lung disease (ILD-PH), and left heart disease associated PH. SSc-PAH is a vasculopathy that is distinct from idiopathic PAH and probably PAH associated with other connective tissue diseases. This concise review provides an overview of the various forms of SSc-PH, as well as advances in the screening, therapeutic management, and risk stratification strategies for the various types of SSc-PH. As the same SSc patient often displays multiple overlapping PH groups, treatment decisions are complex and often need to be individualized. This review also attempts to provide a rational framework for the management of SSc-PH patients with overlapping disease phenotypes.

INTRODUCTION

Group 5 pulmonary hypertension (PH) has long remained an elusive entity. Data over the past decade have consistently shown links between hematologic disorders and pulmonary vascular disease. PH due to hematologic disorders exemplifies how pulmonary vascular disease is intertwined with vascular abnormalities at the systemic level.

At the last (6^th) World Symposium on PH in 2018, Group 5 PH was defined as “PH with unclear and/or multifactorial mechanisms,” and PH secondary to hematologic disorders forms Subgroup 5.1 (Table 1).¹ This subgroup includes 2 primary and very important etiologic entities, namely chronic hemolytic anemias

Full Issue PDF

Guest Editor’s Memo

Pulmonary Hypertension in Collagen Vascular Disorders: Systemic Sclerosis: An Overview of Systemic Sclerosis-Associated Pulmonary Hypertension

Hematologic Disorders and Pulmonary Hypertension

INTRODUCTION

PH Special Discussion: Obesity and Pulmonary Hypertension: A Discussion With Deborah Jo Levine and Anna Hemnes

PH Professional Network: Obesity and Pulmonary Hypertension

INTRODUCTION

PH Roundtable: PH and COVID-19 as a Systemic Infection

Conference Abstracts

Guest Editor’s Memo

A Roundtable Discussion Addressing Patient-Centered Care and Quality of Life in Patients With Pulmonary Arterial Hypertension

Editor’s Memo

Pulmonary Hypertension Associated With Parenchymal Lung Disease