Systemic Sclerosis Associated Pulmonary Arterial Hypertension (SSc-PAH) is a rare and severe complication of systemic sclerosis (SSc), a connective tissue disorder characterized by fibrosis and vasculopathy. SSc-PAH is a progressive disease that affects the pulmonary arteries, leading to increased blood pressure and resistance in the lungs, which can ultimately result in right heart failure and death.
Although SSc-PAH affects only a small percentage of patients with systemic sclerosis, it is a major cause of morbidity and mortality in this population. Studies have shown that the prevalence of PAH in SSc patients ranges from 7% to 12%, and that the mortality rate of SSc-PAH is higher than that of idiopathic PAH.
The pathogenesis of SSc-PAH is complex and not fully understood. It is believed to be related to the underlying vasculopathy of systemic sclerosis, which causes narrowing and obliteration of the small pulmonary arteries. This results in increased pulmonary vascular resistance and ultimately right ventricular dysfunction. Other factors that may contribute to the development of SSc-PAH include inflammation, autoimmunity, and genetic predisposition.
Diagnosing SSc-PAH can be challenging, as symptoms such as dyspnea and fatigue are common in both SSc and PAH. Screening for SSc-PAH is recommended in all patients with systemic sclerosis, using a combination of clinical assessment, echocardiography, and pulmonary function tests. Further evaluation may include right heart catheterization, which is the gold standard for diagnosing PAH.
Treatment options for SSc-PAH include vasodilator therapy, immunosuppressive therapy, and lung transplantation. However, the management of SSc-PAH is complex and requires a multidisciplinary approach, involving rheumatologists, pulmonologists, and cardiologists. The goal of treatment is to improve symptoms, hemodynamics, and quality of life, and to prolong survival.
On Friday, May 5th, 2023, a talk on SSc-PAH will be given at the 39th annual Congress of Clinical Rheumatology by Dr. Virginia D. Steen, MD from Washington DC. Dr. Steen is a renowned rheumatologist and an expert in the field of systemic sclerosis and pulmonary hypertension. Her talk will focus on the diagnosis and management of SSc-PAH, and will provide insights into the latest advances in the field.
Dr. Steen’s talk is expected to be of great interest to rheumatologists, pulmonologists, and cardiologists who manage patients with SSc-PAH. It will provide an opportunity for clinicians to learn about the latest research findings, treatment options, and best practices for managing this challenging complication of systemic sclerosis.
In addition, the talk will highlight the importance of early diagnosis and aggressive management of SSc-PAH, which can significantly improve outcomes for patients with this condition. It is hoped that the talk will raise awareness of SSc-PAH among healthcare professionals and encourage greater collaboration between different specialties in the management of this complex disease.
In conclusion, SSc-PAH is a rare but serious complication of systemic sclerosis that requires prompt diagnosis and management. Dr. Steen’s talk at the 39th annual Congress of Clinical Rheumatology will provide valuable insights into the diagnosis and management of SSc-PAH and is expected to be of great interest to healthcare professionals who manage patients with this condition. It is hoped that the talk will raise awareness of SSc-PAH and encourage greater collaboration between different specialties in the management of this complex disease.