Scleroderma

1. Scleroderma

2. The term scleroderma encompasses a spectrum of disorders that includes localisedscleroderma (morphea), which mainly causesdermal fibrosis; juvenile scleroderma, which isusually localised but can present with systemicdisease; and scleroderma-like disorders. Thediagnosis of scleroderma should be doubtedin the absence of Raynaud's phenomenon. There are two main subsets of sclerodermaaccording to the duration of Raynaud'sphenomenon before the start of symptomsand signs suggestive of scleroderma.

3. Raynaud's phenomenon- episodic, clearly demarcated two or three phasecolour change-white (ischaemia), then often blue (stasis), then red(reactive hyperaemia)-of fingers and sometimes toes (rarely nose,tongue, or ears) in response to cold or, less often, emotion. Raynaud's disease refers to the development of trophic changes as a result ofmicrocirculatory damage and prolonged local ischaemia.

4. Raynaud's phenomenon

5. Subsets of scleroderma 1/ Limited cutaneous scleroderma 2/ Diffuse cutaneous scleroderma 3/ "Scleroderma sine scleroderma"

6. 1/ Limited cutaneous scleroderma * Raynaud's phenomenon for years (sometimes decades) before start of scleroderma * Skin is affected only at the extremities (hands, face, feet, and forearms) or is not affected * Substantial proportion of patients develop pulmonary hypertension oflate onset (after 10-15 years) with or without interstitial lung disease, skin calcifications, telangiectasia, and gastrointestinal symptoms * High prevalence of anticentromere antibodies (70-80%) * Dilated capillary loops of nail folds, usually without capillary dropout

7. 2/ Diffuse cutaneous scleroderma • * Start of skin changes (puffy or hidebound) within one year of start of Raynaud's phenomenon • * Skin of trunk and extremities affected • * Presence of tendon friction rubs • * Substantial proportion of patients have early onset of interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myocardial disease • * Dilated capillaries of nail folds and capillary dropout • * Antibodies to scleroderma-70 (topoisomerase-1) in 30% of patients

8. Limited cutaneous scleroderma Patients who develop this condition(previously called CREST) may haveRaynaud's phenomenon for years before theappearance of the condition's characteristicsymptoms: • calcium deposits in the skin, • painful digital scars and ulcers, • dilated blood vessels (telangiectasia), • oesophageal dysmotility and reflux. • There are few if anyconstitutional symptoms, and skin fibrosis isoften restricted to sclerodactyly and microstomia, with minimal progression Raynaud's phenomenon, pitting scars, digital ulcers, and telangiectasia can all betroublesome, and oesophageal symptoms arecommon. • Patients may not be aware of thethickening of their fingers, but unsightlypuckering, wrinkling and tightening of theskin around the mouth are soon noticed

9. There are few if anyconstitutional symptoms, and skin fibrosis isoften restricted to sclerodactyly and microstomia, with minimal progression Raynaud's phenomenon, pitting scars, digital ulcers, and telangiectasia can all betroublesome, and oesophageal symptoms arecommon. Patients may not be aware of thethickening of their fingers, but unsightlypuckering, wrinkling and tightening of theskin around the mouth are soon noticed

10. Diffuse cutaneous scleroderma • Patients destined to develop this conditionoften have a short history and abrupt onset ofRaynaud's phenomenon, and their skin isoedematous and itchy.During the first fiveyears of diffuse disease (early phase), patientsare weary and ill and lose weight. Arthritis,myositis, and tendon involvement arecommon. • The fibrotic phase rapidly followsand can extend to affect most areas of skinexcept for the middle and lower back and buttocks. Hyperpigmentation and hypopigmentation may occur, the latter beingmore obvious in non-white patients.

11. Rapidprogression of skin disease is accompanied byincreased risks of renal failure (oftenpresenting as hypertensive renal crisis) and ofpulmonary interstitial, early cardiac, and gastrointestinal disease. After about five years (late phase) theconstitutional symptoms usually subside. Thisatrophic phase may last for many years;musculoskeletal problems lead to deformityand wasting and existing visceral disease oftenprogresses, though the risk of new organs being affected is reduced.

12. Scleroderma sine scleroderma • Some patients have scleroderma withouttheir skin being affected, although they may have Raynaud's phenomenon. • Patients present with complications of an internal organ such as restrictive pulmonary disease, cardiac failure, hypertensive renal crisis, or malabsorption and pseudo-obstruction. • The presence of anticentromere, scleroderma-70, or antinucleolar antibodies can be helpful in making a definitive diagnosis.

13. Treatment of scleroderma • Therapy is directed towards the individual features affecting different areas of the body • - aggresive treatment of elevated blood pressure to prevent kidney failure especially ACE inhibitors • Raynaud’s phenomenon- nifedipine • Heartburn- omeprazole • Pulmonary hipertension: i.v. prostacyclin- Iloprost or bosentan, sildenafil or tadanafil