Scleroderma

Scleroderma

Thickening of skin r/t accumulation of connective tissue by structure functional abnormalities of visceral organs, GI, Lungs, Heart and Kidney
1. Uncontrolled Irreversible proliferation of normal connective tissue along with striking vascular changes
  1. Collagen
  2. Proteoglycans
  3. Fibronectin
  4. Laminal
Types
1. Localized
  1. Morphea
    1. LImited to skin
  2. Linear Scleroderma
    1. liinear lesions extend trhoug the dermis SQ muscle underlying bone= unilaterl, limb and trunk 1 or more streak of extrimiites of trunk flexion contracting when lesions extends over joints limbs length discrepancy
  3. En Coup Sabre
    1. Scalp and face can extend to CNS= Neuro Sword, HA, Seizures, uveitis, hemifacial atrophy feel changes
  4. Cant be cured placed on remission no great Treament for Remission Steriod Methotrexate pulse steroid Scleroderma localized burns self out 5-10 years control and prevent inflammation damage
2. Generalized or systemic progressive sclerosis , cant put in remssion gets worse life expectancy 20-30
  1. insidious onset
  2. types
    1. Limited
    2. Diffuse
      1. Pulmonary Arterial
      2. Telagietasis
      3. Digital Pelter
      4. Pylmonary Fibrosis
      5. Raynauds
      6. Sclerodaty
      7. Cardiac Defeccts
      8. Pulmonary Arterial HTN
      9. Renal Disease
  3. Distal to Proximal
  4. Endothelial damage leads Fibroblast dysfunction
Treament
1. Methylprednisone
2. Nifedepine - dilate feet
3. Methotrexate
4. Prednisone
5. UV light
6. Cycloph
7. Mycophenate
DIagnose
1. Chest CT
2. PFT
3. EGG
4. ECHO

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	Criado por Sandra Rodriguez mais de 6 anos atrás