Multisystem disease. Localised scleroderma syndromes ( Morphea ) do not involve organs and rarely cause vasospasm.
Etiology/Epidemiology
- SSc has highest mortality in autoimmune diseases
- Female, 30-50y, exposure (vinyl chloride, silica, rapeseed oil. Drugs – bleomycin
Pathology
- Vascular
- Extensive damage to small vessels w/ increased permeability (cytokines)
- Lead to obliterative arterial lesions and chronic ischaemia
- Fibrosis
- Fibroblasts produce collagen (1/3), GAG, fibronectin
- At the lower dermis of the skin – thickening, tightening, induration (Th17)
Clincial features
- Raynauds – seen in all cases
- LcSSc (70%)
- Raynauds > skin changes (after 10-15y) > hands/face/ feet/forearm
- Beak like face, microsomia , telangiectasia, nail dystrophy
- GIT, pulmo HT, Pulmo interstitial disease
- CREST syndrome
- DcSSc (30%)
- Edema > rapid skin sclerosis and raynauds > Skin atrophy
- General – lethargy, weight loss, anorexia
- Heartburn, dysphagia, malabsorption, anal incontinence
- Renal – acute/chronic, hypertensive renal crisis
- Lungs – fibrosis, PHT
- Heart – arrhythmias, pericarditis
- Sine SSc – systemic features w/out skin involvement
Diagnosis
Made clinically
- FBC – normo anaemia, microangiopathic haemolytic anaemia (renal)
- Auto-ab
- LcSSc – ACA (anti centromere ab)
- DcSSc – anti-Scl-70 (topoisomerase I), anti-RNA poly, RF +, ANA +
- Imaging – CXR, barium, CT (lung fibrosis)
Treatment
Organ based tx, corticosteroids and immune modulators are rarely used – unless lung fibrosis.
- Raynauds – calcium blockers, ACE I, lumbar sympathectomy
- Oesophagus – PPI, nutritional supplements (malabsorption)
- Lungs – PHT (oral vasodilators, o2, warfarin), fibrosis (cyclophosphamide, azathioprine, CS)