Systemic Sclerosis with Multiple Pulmonary Manifestations OD16-OD17
Dr. Mohan Rao Kotnur,
Professor and Head, Department of Respiratory Medicine, Rajarajeswari Medical College and Hospital,
Mysore Road, Kengeri Hobli, Bangalore-74, India.
Systemic sclerosis (SSc) is a chronic autoimmune multisystem disorder characterized by endothelial dysfunction and fibroblast dysfunction, which results in progressive fibrosis of the skin and internal organs more frequently the lungs and gastro intestinal tract. Pulmonary involvement is common in the course of SSc, with Interstitial Lung Disease (ILD) and Pulmonary Arterial Hypertension (PAH) being the leading causes of death. Here we report, case of an elderly female patient presenting with Diffuse SSc with multiple uncommon pulmonary manifestations like ILD with Usual Interstitial Pneumonia (UIP) pattern (usually less common), PAH and right sided pleural effusion.