Systemic sclerosis (SSc), also known as scleroderma, is a (non contagious, chronic) multi-system, autoimmune inflammatory and fibrotic connective tissue disease, characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, and production of auto-antibodies.

Aetiology and pathogenesis are unknown.

There are 2 main subtypes:

• Limited cutaneous SSc
• Diffuse cutaneous SSc

Risk factors:

• Positive family Hx
• Immune dysregulation (presence of ANA)
• Exposure to environmental substances and toxins (e.g. silica dust or solvents)

PC

*Presence of risk factors

• Scleroderma - Hardening of skin, shiny tight skin without normal skin folds
• Sclerodactyly - Skin tightening restricts ROM in hands
• Digital pits and ulcers - (50%), distal digits ulcers - originate from ischaemia
• Telangiectasia
• Calcinosis - calcium deposits under fingertips
• Raynaud’s phenomenon