Scleroderma Lung Disease – Other Lung Complications in Systemic Sclerosis

Maria Do Socorro Teixeira Moreira Almeida

doi:10.4172/2161-1149.s1-009

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.

Highlights

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
Pulmonary involvement is second in frequency only to oesophagus involvement as a visceral complication of systemic sclerosis and has surpassed renal involvement as the most common cause of death
Pulmonary involvement is common and occurs in all SSc subsets, including limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis and SSc sine scleroderma [4]

Summary

Introduction

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 [1]. Mortality from scleroderma renal crisis has been significantly reduced with the use of angiotensin converting enzyme inhibitors beginning in the 1980s and lung disease has emerged as the leading cause of mortality. The face may be affected in lcSSc and dcSSc. ILD and pulmonary arterial hypertension (PAH) are the leading causes of mortality in SSc [5]. The identification and staging of pulmonary manifestation is of paramount importance to the management of patients [2]

10. Spontaneous pneumothorax

Findings

Conclusion