Localized and systemic scleroderma Treatment

Scleroderma is a chronic autoimmune connective tissue disease characterized by excessive collagen deposition leading to fibrosis of the skin and, in systemic disease, internal organs. The exact cause is unknown, but genetic susceptibility, immune dysregulation, and vascular injury contribute to disease development.

Two major forms are recognized:

• Localized scleroderma (morphea), which primarily affects the skin.

• Systemic scleroderma (systemic sclerosis), which involves the skin, blood vessels, and internal organs such as the lungs, kidneys, gastrointestinal tract, and heart.

Pathophysiology (Brief)

The disease is characterized by:

• Autoimmune activation

• Microvascular injury

• Excessive collagen production

• Progressive fibrosis of the skin and internal organs

Signs & Symptoms

Localized scleroderma

• Shiny, thickened plaques

• Skin hardening

• Lesions over the extremities

• Scalp involvement

• Hand involvement

Systemic scleroderma

• Skin thickening and tightening

• Raynaud phenomenon

• Finger ulcers

• Joint stiffness

• Gastroesophageal reflux

• Difficulty swallowing

• Pulmonary involvement

• Renal involvement

• Cardiac involvement

Diagnostic Criteria

Diagnosis is based on:

• Characteristic skin thickening

• Raynaud phenomenon

• Internal organ involvement in systemic disease

Investigations

Recommended investigations include:

• Full blood picture (FBP)

• ESR

• Liver function tests

• Renal function tests

• Urinalysis

• ANA

• Anti-centromere antibody

• Anti-Scl-70 antibody

• Skin biopsy when diagnosis is uncertain

Monitoring

• Liver, renal function, and full blood count every 3 months

• Random blood glucose every 3 months in patients receiving prolonged corticosteroid therapy

Treatment

Non-pharmacological treatment

• Keep extremities warm

• Physiotherapy

• Monitor blood pressure

• Involve a physician

• Deworm with Albendazole 400 mg stat before initiating prolonged immunosuppression where appropriate

Pharmacological Treatment

Localized scleroderma

• Clobetasol propionate cream/ointment (topical) 0.05% — apply every 12 hours

Systemic scleroderma

• Prednisolone (PO) 0.5–1 mg/kg once daily until disease stabilizes, then taper slowly

AND

• Methotrexate (PO) 7.5–20 mg once weekly

AND

• Omeprazole (PO) 20 mg once daily when needed for gastritis or peptic ulcer disease

Complications

• Permanent skin fibrosis

• Digital ulcers

• Pulmonary fibrosis

• Pulmonary hypertension

• Renal crisis

• Cardiac disease

Patient counselling

• Scleroderma is a chronic autoimmune disease.

• Treatment controls disease progression but does not cure it.

• Regular follow-up is essential.

• Keep hands warm to reduce Raynaud attacks.