Barts Wiki
Advertisement

2. Describe the differences between diffuse and limited scleroderma[]

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.
Advertisement