RDS 2026 ANNUAL MEETING

What Is Systemic Sclerosis?

Scleroderma, also called systemic sclerosis (SSc), is an autoimmune connective tissue disease characterized by autoantibodies, the thickening and stiffening of skin tissue, and sometimes internal organ involvement. The cause of systemic sclerosis is unknown.

It can occur in systemic form (systemic sclerosis, scleroderma) or in localized form. The localized form, called morphea, only affects the skin and underlying tissue. Read more about morphea here.

The systemic form usually affects skin tissue and internal organs, and has two major subsets: limited and diffuse.

Early Signs and Skin Findings

Scleroderma often presents with Raynaud’s phenomenon (reversible narrowing of blood vessels in the fingers and toes) characterized by white, then blue, then red color changes of the digits. Severe cases may leave sores on the fingers. Most patients first notice a puffiness or swelling of the hands followed by tightening and hardness of the fingers and hands (sclerodactyly).

Internal Organ Involvement

While many patients only have skin symptoms, patients with the systemic form of the condition can develop internal organ involvement that may include the GI tract, kidney, lungs, and heart. The lung involvement, the most common cause of death, must be prevented as damage is very difficult to reverse once it has occurred. Your doctor will likely recommend you be screened for interstitial lung disease (ILD) and/or pulmonary artery hypertension (PAH) via pulmonary function tests (PFTs), a high-resolution CT scan (HRCT) or an echocardiogram.

Disease Course

It is unknown how scleroderma will behave or progress in a particular person. The disease remains relatively mild in some people and progresses in others. Sometimes, the disease stops progressing and patients improve.