Study of Lung and Pulmonary Hypertension Issues in Adults with Connective Tissue Diseases Including Systemic Sclerosis

What this Trial Is About

Researchers are investigating the current patterns and progression of interstitial lung disease ILD and pulmonary hypertension PH in patients with connective tissue disease CTD. This observational registry study aims to better understand the prevalence, incidence, and management of these lung-related conditions, focusing on challenges like identifying patients with worsening disease and determining the best timing for treatment. The study particularly explores whether inflammation or fibrosis drives the disease. The study collects detailed clinical data including demographics, disease information, routine lab results such as antibody profiles and iron status, diagnostic procedures like thoracic ultrasound and high-resolution CT scans, and therapeutic management over a 10-year period. For a subgroup of patients with systemic sclerosis, blood samples will be collected over time to study inflammatory and fibrotic disease patterns. This multidisciplinary registry seeks to provide real-world insights into CTD-related lung disease. Participants will undergo routine clinical assessments including pulmonary function tests spirometry or plethysmography, imaging exams thoracic ultrasound, HRCT, echocardiography, and blood sampling during their regular care visits. The study will monitor key outcomes over one year such as lung function decline measured by forced vital capacity and carbon monoxide diffusion capacity, as well as skin involvement measured by the modified Rodnan Skin Score. This long-term observation will help describe disease course and may guide future research and treatments.

Connective Tissue Diseases and Lung Manifestations