Systemic sclerosis (SSc) is a collagen vascular disease characterized by mircovascular damage and excessive fibrosis of the skin and internal organs including the lungs. The mortality and morbidity of SSc are associated with severe organ involvement of the skin, lung, gastrointestinal tract, heart, and kidney. Survival from SSc has improved over the past two decades because angiotensin converting enzyme inhibitors became available to treat renal crisis. Lung involvement, such as interstitial lung disease (ILD) and pulmonary hypertension, is now the major cause of death in SSc.
ILD complicates in 40-70% of SSc patients. Although it occurs predominantly in SSc patients with diffuse cutaneous involvement (dSSc), it also occurs in patients with limited cutaneous involvement (lSSc). High-resolution computed tomography (HRCT) shows bibasilar pulmonary fibrosis. Steen et al. reported that only 15-20% of SSc patients developed severe restrictive lung disease. This finding implies that the progression of pulmonary fibrosis is much slower or ceases in surviving patients.
Clinical and Radiographic Heterogeneity of Interstitial Lung Disease in Systemic Sclerosis Based on Disease-Specific Autoantibodies: Tohru Takeuchi, Kentaro Isoda, Kenichiro Hata, Shuzo Yoshida, Koji Nagai, Takuya Kotani, Shigeki Makino and Toshiaki Hanfusa
Journal of Pulmonary & Respiratory Medicine welcomes articles related to "Systemic sclerosis"
Last date updated on June, 2014