Scleroderma Lung Disease – Other Lung Complications in Systemic Sclerosis

Abstract

Scleroderma or systemic sclerosis (SSc) is a clinically heterogeneous, multi-system autoimmune disorder characterized by endothelial dysfunction, dysregulation of fibroblasts resulting in excessive production of collagen and profound abnormalities of the immune system. Pulmonary involvement is common and occurs in all SSc subsets, including limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis and SSC sine scleroderma. Aside from the common complications of pulmonary vasculopathy and interstitial lung disease (ILD], other less frequent pulmonary complications have been reported in SSc. The emphasis of this review will be on other lung complications in systemic sclerosis.

Atypical rapid onset Scleroderma Renal Crisis (SRC) complicated with diffuse alveolar hemorrhage and pleuro-pericardial effusions in a patient with recently diagnosed breast cancer and a positive anti-RNA polymerase III Ab.: A case report

“…Scleroderma or systemic sclerosis (SSc) is a heterogeneous disorder characterized by endothelial dysfunction, dysregulation of fibroblasts resulting in excessive production of collagen and profound abnormalities of the immune system. These changes cause progressive fibrosis of the skin and internal organs, system failure, and death [ 7 ]. Pulmonary-Renal Syndrome characterized by diffuse alveolar hemorrhage and acute renal failure is a rare but devastating complication with high mortality that can be associated with systemic sclerosis.…”