Systemic sclerosis is an autoimmune condition characterised by thickening and hardening of the skin (sclerosis) and other connective tissues.
90% of patients with any form are ANA positive.
There are 3 distinct patterns of systemic sclerosis, including:
Limited cutaneous SS
Diffuse cutaneous SS
Scleroderma
Limited cutaneous systemic sclerosis
Clinical Features
Typically women aged 30-50 years of age
Sclerosis affects the face & distal limbs
Raynaud’s phenomenon is common
Used to be referred to as CREST syndrome. Features of LCSS therefore include: Calcinosis, Raynaud’s, Esophageal dysfunction, Sclerodactyly, Telangiectasia
Antibody
Anti-centromere antibodies
ANA
Figure 219: Shiny, tight skin on distal phalanges in systemic sclerosis. Maria Sieglinda von Nudeldorf, CC BY-SA 4.0
Diffuse cutaneous systemic sclerosis
Clinical Features
Typically women aged 30-50 years of age
Sclerosis affects the trunk & proximal limbs
Complications
Interstitial lung disease (up to 8/10 patients) - SSc-ILD (Scleroderma-related ILD) - pulmonary fibrosis with progressive respiratory failure
Pulmonary hypertension
Hypertension, CKD
Antibody
SCL-70 antibodies
ANA
Figure 220: HRCT image demonstrating interstitial lung disease in a patient with diffuse cutaneous systemic sclerosis. Nevit Dilmen, CC BY-SA 3.0.
Scleroderma
Clinical Features
Hardened, thickened areas of skin. Tightening of the skin gives a 'shiny' appearance.
Plaque morphoea - oval patches of discoloured, hardened skin which might be shiny
Linear morphoea: thickened lines of skin along the arms and legs
