[Lung involvement in systemic sclerosis]. / Manifestations pulmonaires de la sclérodermie systémique.

Systemic sclerosis (SSc) is a protean disorder in which prognosis and treatment are tailored on the basis of organ involvement. Among SSc lung manifestations, interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) or the combination of both, are the first cause of SSc mortality and impact heavily on patient quality of life. ILD may begin early in disease and usually progresses slowly. However, approximately 10% of patients with ILD may reach terminal respiratory insufficiency. PAH may be an early or late complication of SSc in which increased blood pressure in pulmonary arteries leads to right heart failure. Current treatments provide some benefit, but both SSc-ILD and PAH still represent an enormous unmet need of more efficacious therapeutic strategies.

[Gastrointestinal features in systemic sclerosis]. / Manifestations digestives de la sclérodermie.

Systemic sclerosis (SSc) is a rare disorder associating vasculopathy, tissue fibrosis and autoimmunity. The gastro-intestinal tract (GIT) is frequently involved with any segment being potentially affected from mouth to anus. The esophagus is the most common localization resulting in reflux and its complications such as erosive esophagitis and Barrett's esophagus. Gastric involvement is less frequent but may be complicated by hemorrhage due to gastric antral vascular ectasia (GAVE or watermelon stomach). Intestinal involvement may lead to malabsorption, intestinal pseudo-obstruction or bacterial overgrowth. Anorectal involvement can cause fecal incontinence and rectal prolapse. GIT involvement greatly affects morbimortality in SSc and therapeutic approaches essentially aim at relieving the symptoms.