Pulmonary Scleroderma: How the Lungs Are Affected and What the Prognosis Is

Scleroderma means "hard skin," but in the systemic form the disease extends far beyond the skin and can affect the lungs, heart, and kidneys. Most people with systemic scleroderma have some degree of loss of lung function. We explain why this happens and how it affects the prognosis.

What scleroderma is and how it affects the lungs

Scleroderma is an autoimmune disease: part of the immune system becomes overactive, and connective tissue cells begin producing too much collagen. This causes scarring and thickening of tissues — fibrosis. Systemic scleroderma (systemic sclerosis) can be limited or diffuse; in the limited form, the skin is affected mainly on the hands, forearms, feet, and face, but over time the lungs and digestive system may become involved. The diffuse form more often affects internal organs. Imagine the lungs as a sponge with the thinnest walls between air sacs; in fibrosis, the walls thicken and the "sponge" lets oxygen through less well. The disease usually develops in women aged 30–50.

What changes occur in the lungs

In scleroderma, fibrosis develops in the lungs: the alveolar walls thicken, and an X-ray may reveal bilateral basal pulmonary fibrosis — reticular or linear-nodular opacities, most pronounced in the lower regions, up to a "honeycomb lung" pattern. A possible complication is pulmonary hypertension (high pressure in the vessels of the lungs), which can cause shortness of breath.

Symptoms of lung involvement in scleroderma

Lung problems can be suspected from shortness of breath. Other manifestations of systemic scleroderma include:

• shortness of breath and loss of some lung function, which occurs in most people with systemic scleroderma;
• Raynaud's syndrome — fingers turn white, blue, or red in the cold or from stress;
• thickened, tight skin on the fingers, hands, and face;
• fatigue, joint pain and stiffness;
• difficulty swallowing and heartburn.

How lung involvement is detected

There is no single test for scleroderma. The doctor asks about symptoms, examines the skin and joints, and may order a blood test for antinuclear antibodies (ANA), a skin biopsy, and imaging to assess the organs. The lungs are checked with X-rays and other studies, and blood pressure is measured regularly and organ function is monitored. Follow-up is usually managed by a rheumatologist, and other specialists are involved when organs are affected.

How long people live with scleroderma and what the prognosis depends on

Scleroderma is a chronic, slowly progressive disease; modern medicine cannot fully eliminate its cause, and treatment is aimed at relieving symptoms, slowing progression, and controlling complications. The prognosis is conditionally unfavorable: over time, the ability to work may be lost. The diffuse form more often affects internal organs, while the limited form usually has a milder course. Reference materials do not provide exact survival times: the prognosis depends on the form of the disease and the organs affected, so it is assessed by the attending physician.

When to see a doctor urgently

Scleroderma itself is not a reason to call an ambulance; 103 or 112 — if a life-threatening condition appears, such as severe difficulty breathing.

This material is for reference and does not replace consultation with a doctor.

This content is for reference and does not replace a doctor's consultation. Medical disclaimer