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Systemic Sclerosis (Scleroderma): Symptoms, Causes, Treatment

التصلب الجهازي (تصلب الجلد)

Quick summary

Systemic sclerosis is an autoimmune disease affecting blood vessels and connective tissues. Skin thickening and internal-organ involvement vary between patients. Early lung, heart and kidney assessment helps guide individualized treatment.

Medical disclaimer: This content is for educational purposes only and is not a substitute for consulting a qualified physician. Do not use this information for self-diagnosis or self-treatment.

What is Systemic Sclerosis (Scleroderma)?

It is a systemic form of scleroderma, distinct from localized forms with different courses. Limited and diffuse cutaneous patterns describe skin distribution but do not capture every organ risk. Raynaud’s phenomenon may precede other features.

Related topic: Sjögren’s disease: another autoimmune cause of dryness.

Symptoms

Fingers may change colour in the cold, become swollen or tight, and lose flexibility. Reflux, swallowing difficulty, dry cough or breathlessness may occur. Symptoms depend on the affected organ, and new changes should be reported.

Causes

Immune and vascular abnormalities interact with excess collagen deposition. The full cause is unknown; genetic and environmental influences may contribute. It is not contagious or caused by eating collagen, and dry skin alone does not establish the diagnosis.

Risk factors

The condition is more common in women and adults, with genetic and environmental influences on susceptibility. Raynaud’s alone does not mean systemic sclerosis will develop. Disease pattern, antibodies, symptom history and treatment affect complication risk.

Diagnosis

Diagnosis combines history, examination and immune tests, sometimes with nailfold-capillary or skin assessment. Blood pressure, urine and blood tests, lung function and imaging assess organ involvement as appropriate. A positive antibody alone is not a definitive diagnosis.

Treatment

Care targets the affected systems: vascular treatment for Raynaud’s, reflux and skin care, mobility support, and selected immune-directed or antifibrotic treatment for lung disease. No single drug addresses every feature. Specialist monitoring is essential; do not start corticosteroids yourself.

Complications

Complications may include digital ulcers, lung fibrosis or pulmonary hypertension, heart or oesophageal problems, and kidney crisis in some patients. They do not affect everyone; regular surveillance supports timely detection and treatment.

Prevention

There is no guaranteed prevention. Protecting extremities from cold, avoiding smoking, caring for skin and teeth, and suitable movement exercises support health. Keep organ-surveillance visits and prescribed blood-pressure monitoring even when skin symptoms seem stable.

When to see a doctor

Seek urgent care for newly elevated blood pressure with headache, blurred vision or reduced urine, or sudden breathlessness or chest pain. A painful ulcer or markedly cold, discoloured finger needs prompt assessment. Internal-organ symptoms should not await a routine skin visit.

Scientific references

  1. Scleroderma: types, symptoms and causes — NIAMS
  2. Scleroderma: diagnosis, treatment and self-care — NIAMS
  3. Scleroderma — NHS
  4. Scleroderma: patient information — American College of Rheumatology