Overview
Scleroderma represents a spectrum of disorders characterised by thickened, hardened skin (from the Greek “scleros”). These disorders include:
- Primary/secondary Raynauds Phenomenon
- Localised scleroderma
- Systemic scleroderma
- Chemical induced
When the characteristic skin disorder is associated with internal organ involvement, the disease is termed systemic scleroderma. Systemic scleroderma also known as systemic sclerosis is the most well known and is broadly divided into:
- Limited cutaneous (60%) also known as CREST syndrome
- Diffuse cutaneous
Systemic scleroderma, systemic sclerosis, predominantly affects women and is associated with production of collagen, microvascular damage, and inflammation.
| Definition Scleroderma: represents a spectrum of disorders characterised by thickened, hardened skin (from the Greek “scleros”). Systemic sclerosis: Thickened, hardened skin associated with internal organ involvement. Further divided into limited and diffuse cutaneous Limited cutaneous (CREST syndrome) Diffuse cutaneous Raynouds syndrome: sequential color changes in the digits precipitated by cold, stress, or even change in temperatures. Raynaud phenomenon is due to arterial vasoconstriction in the digits. |
Risk Factors
Signs and Symptoms
This section focuses on systemic scleroderma. Which can be further divided into limited and diffuse sclerosis.
| Case: young or middle-age woman with Raynaud phenomenon and skin changes accompanied by musculoskeletal discomfort and gastrointestinal symptoms. |
General
- Fatigue
- Stiff joints
- Weakness
- Pain
- Sleep problems
- Skin discolouration
Limited cutaneous scleroderma (CREST Syndrome) skin sclerosis is restricted to the hands and face.
- Calcinosis
- Raynouds Phenomenon
- Esophageal reflux
- Sclerodactyly
- Telangectasia
Diffuse cutaneous scleroderma skin sclerosis is involved hands, face, chest and abdomen.
- CREST syndrome
- Internal organ fibrosis and ischaemia
- Pulmonary fibrosis
Differential Diagnosis
- Amyloidosis
- Drug-induced scleroderma (chemotherapy drugs)
- Sclerodactyly (“diabetic cheiroarthropathy”) occurs in individuals with longstanding Type I diabetes
- Myxedema (Hypothyroidism)
Differentials for Raynoud phenomenon
Investigations
- Urinalysis
- FBC
- EUC
- ANA (95%)
- Anti-centromere antibody (75% with limited systemic scleroderma)
- Antitopoisomerase 1 antibody (Anti Scl-70) (60% with diffuse systemic scleroderma)
- Anti-RNA polymerase III antibody
Other
- RF
- Anti-CCP
- Anti-smith antibody
- Anti-dsDNA
- Chest X-ray
- Echocardiogram – heart/lung involvement
- CT chest – pulmonary fibrosis?
Pathophysiology
Three principle pathologies in scleroderma
- Raynoud’s Phenomenon
- Inflammation
- Fibrosis
Management
Complication and Prognosis
Complications
- Vascular
- Digitalis ischaemia
- Pulmonary artery hypertension
- Renal crisis
- Mycocardial infarction
Prognosis
- Systemic sclerosis has the highest case-specific mortality of any autoimmune rheumatic disease.
- Renal crisis (50% mortality)
Discussion