scleroderma
SCLERODERMA
Scleroderma is a connective tissue disorder characterized by skin thickening and fibrosis. It is rare, autoimmune and chronic.
It has an idiopathic cause and not contagious.
Early microvascular damage, mononuclear cell infiltrates and slowly developing fibrosis are the important features of the tissue lesions.
The leading causes of death are pulmonary fibrosis and pulmonary arterial hypertension.

Surgical Intervention

  • An option for patients who does not respond to medical therapy
  • Considered for pain reduction, severe fixed deformities, ulceration & calcinosis
  • If there is severe digital gangrene & debridement & removal of the nails did not caused relief, amputation of the affected limb may be done

Lumbar Sympathectomy

  • Provides long term beneficial effect to severe vasoconstriction of the feet

Radical Microarteriolysis (Digital Sympathectomy)

  • Relieves severe pain, heal digital ulcers & often reduces the severity of the attacks of Raynaud phenomenon
  • Although complication rates are relatively high

Organ Transplant

  • Renal & lung transplantation is done in severe cases of renal crisis or pulmonary fibrosis, respectively
  • Some studies have shown that non-myeloablative stem cell transplantation are beneficial for patients w/ systemic sclerosis when compared to treatment w/ Cyclophosphamide
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