Systemic sclerosis (generically known as scleroderma) is a multisystem connective tissue disorder characterized by abnormal, marked deposition of collagen in tissues. The skin, vascular system, gastrointestinal tract, musculoskeletal system, lungs, kidney, and heart are particularly affected.
Systemic sclerosis has a 3:1 female:male distribution.
The lungs are the fourth most commonly affected organ (following the skin, vessels, and esophagus). In established cases, the most common pulmonary symptoms are dyspnea (secondary to interstitial fibrosis or pulmonary arterial hypertension (PAH)), cough, and pleuritic pain. In the diffuse form, restrictive lung disease is most common; in the limited form, PAH is most prevalent. There is often incongruence of findings on radiological exams and pulmonary function testing.
The most common radiographic finding is symmetric, bibasilar reticulonodular interstitial disease. Other findings include low lung volumes, cystic lesions, and traction bronchiectasis. Esophageal dilatation is also frequently seen.
Infection, often secondary to aspiration, eclipses respiratory failure as the most common pulmonary cause of death. Pulmonary disease complications are the most common cause of death in patients with systemic sclerosis.