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2. Describe the differences between diffuse and limited sclerodermaEdit

Both limited and diffuse scleroderma fall under the category of 'systemic sclerosis', thus separating limited scleroderma from localised scleroderma syndromes such as morphoea.


Limited Cutaneous Scleroderma (LsSSC) - formerly CREST Diffuse Cutaneous Scleroderma (DcSSc) 
70% of cases 30% of cases
Prodrome - Reynaud's precedes disease up to 15 years before disease Prodrome - Occurs shortly before disease onset - Raynaud's and oedema
Skin involvement pattern - distal to knees and elbows Skin involement pattern - can be whole body in severe cases
Characteristic features - puffy fingers, tight skin, microstomia, telangectasia, hypopigmentation, capillary loops Characteristic features - extensive skin involvement, oedematous in onset, later skin atrophy, severe internal organ involvement
Early stage (<5 years from onset) Early stage (<5 years from onset)
Constitutional symptoms - fatigue common

Constitutional symptoms - fatigue, weight loss, pruritis

Skin thickening - no or minimal progression Skin thickening - rapid progression, usually reaching peak involvement within 2 years
Organs affected - Reynaud's, fingertip ulceration, oesophageal symptoms Organs affected - risk of renal (acute or chronic failure), cardiac (conduction defect,arrhythmia, pericarditis), pulmonary fibrosis, pulmonary hypertension, GI (anal incontinence common), articular, muscular damage
Rx - Vascular treatment, digital sympathomimectomy, removal of calcinosis, removal of calcinosis, treat oesophageal problems Rx- vascular therapy, PTOT as appropriate, immunosuppression for lung fibrosis/severe disease, corticosteroids
Late stage Late stage
Constitutional symptoms worsened by vasculopathy, GI symptoms Constitutional symptoms generally diminished
Skin thickening - stable or slow progression Skin thickening - Stable or slow regression
Organs affected - Raynaud's, ulcers of digit tips, calinosis, oesophageal stricture, small bowel malabsorption, pulmonary arterial hypertension, lung fibrosis Organs affected - musculoskeletal deformities, progression of visceral diseases but reduced risk of new complications
Rx - As above, treat midgut problems Rx - treat complications, gradual withdrawal of immunosuppression
Autoimmune Profile Autoimmune Profile
Anti Centromere Antibodies (ACA) - 60%. If present, will almost never have another reactivity associated with SSc.  Anti-topoisomerase 1 (a.k.a Scl-70) -  40%. Predictive of interstitial lung involvement.
Anti-topoisomerase 1 (a.k.a Scl-70) - 15%. Especially predictive of interstitial lung involvement.
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