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Scleroderma

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Scleroderma

  1. 1. SCLERODERMA
  2. 2. Systemic sclerosis is a rare, heterogeneous, slow- motion disease, with (allegedly) a small window of opportunity to fundamentally change the course of the disease.
  3. 3.  derived from greek and means hard skin  Affects about 250/million = 7500 Canadians  Affects mainly women in the prime of their life  women: men ratio 3-5:1,  onset between 30-50 years
  4. 4.  Systemic sclerosis (scleroderma)  a multisystem disorder characterized by  functional and structural abnormalities of blood vessels  fibrosis of the skin and internal organs  immune system activation  autoimmunity  Localized scleroderma  morphea, linear scleroderma
  5. 5. LOCALIZED FORMS  Morphea  Generalized / pansclerotic morphea  Linear scleroderma - En coup de saber  Progressive hemifacial atrophy
  6. 6. MORPHEA  A rare skin condition that causes reddish or purplish patches on your skin.  Tends to affect only the outermost layers of your skin — the dermis and the fatty tissue just beneath the dermis.  Location –  Abdomen, chest and back - %  Face, Arms and legs
  7. 7.  Signs of morphea -  Hardening and thickening of the skin.  Discoloration of the affected skin to look lighter or darker than the surrounding area.  Oval-shaped patches that may change colors and gradually develop a whitish center.  Linear patches, especially when on arms and legs  Loss of hair and sweat glands in the affected area over time.
  8. 8. Generalized
  9. 9. Linear variety
  10. 10. Epidemiology  Prevalence: 19-75 cases per 100,000  Susceptibility: host factor  age - peak occurrence: age 35-65 years  gender - female : male = 7-12 : 1  genetic background  Environmental factors  infection  occupational exposures: silica dust
  11. 11. Pathogenesis  Vasculopathy of small artery and capillary  endothelial cell injury  adhesion and activation of platelet  PDGF, thromboxane A2 release  vasoconstriction & growth of endothelial cell and fibroblast  narrowing or obliteration, increased permeability  Fibrosis  aberrant regulation of fibroblast cell growth  increased production of extracellular matrix (collagen, fibronectin, and glycosaminoglycan)  thickening of the skin & fibrosis of internal organs
  12. 12.  Immunologic mechanism  cell mediated immunity  skin: cellular infiltrates in perivascular region and dermis (T cell, Langerhans cell, plasma cell, macrophage)  Humoral immunity  hypergammaglobulinemia  autoantibody production  antinuclear antibody (+) > 95%
  13. 13. •Environmental factors 1) silica dust 2) organic solvents 3) biogenic amines 4) urea formaldehyde 5) polyvinyl chloride 6) rapeseed oil 7) bleomycin 8) L-tryptophan 9) silicone implant (?)
  14. 14. Classification of systemic sclerosis  Diffuse cutaneous systemic sclerosis  proximal skin thickening  - distal and proximal extremity and often the trunk and face  tendency to rapid progression of skin change  rapid onset of disease following Raynaud’s phenomenon  early appearance of visceral involvement  poor prognosis
  15. 15. Limited cutaneous systemic sclerosis 1) symmetric restricted fibrosis - affecting the distal extremities and face/neck 2) prolonged delay in appearance of distinctive internal manifestation 3) prominence of calcinosis and telangiectasia 4) good prognosis * CREST syndrome
  16. 16. Clinical features  Vascular abnormalities  Raynaud's phenomenon  cold hands and feet with reversible skin color change (white to blue to red)  induced by cold temperature or emotional stress  initial complaint in 3/4 of patients  90% in patients with skin change  (prevalence in the general population: 4-15%)  Digital ischemic injury
  17. 17. Raynaud’s phenomenon
  18. 18. telangiectasia
  19. 19. Terminal digit resorption
  20. 20. Digital ulcers – evidence of vascular disease
  21. 21. Digital necrosis
  22. 22. Skin involvement 1) stage - edematous phase - indurative phase - atrophic phase 2) firm, thickened bound to underlying soft tissue 3) decrease in range of motion, loss of facial expression, inability to open mouth fully
  23. 23. Skin thickening of systemic sclerosis begins on the fingers and hands in nearly all cases. The skin initially appears shiny and taut and may be erythematous at early stages.
  24. 24. Phases of skin thickening  Edematous
  25. 25. Causes of this swelling  Basically this is water in the tissues = edema  Binding of water to increased extracellular connective tissue matrix  Inflammation  Poor lymphatic return  Microvascular injury with fluid extravasation
  26. 26.  Indurative Phase
  27. 27.  Thick  Shiny  Taught  Tightly adheres to underlying tissue so cannot be easily picked up  Creases disappear  May become hyper- or hypo-pigmented  Hair loss  Decreased sweating
  28. 28. Acrosclerosis (flexion contractures secondary to skin tightening) (sclerodactyly)
  29. 29. Facial changes  Pinched nose (“mauskopf”)  Pursed lips  Cannot evert eyelids  Lip thinning and retraction  Immobile facies
  30. 30. •Atrophic phase  Thick dermis reverts to normal or even becomes thinner (atrophy)
  31. 31. Skin involvement 4) ulceration, loss of soft tissue of finger tip, pigmentation, calcific deposit, capillary change 5) pathology - atrophy of dermal appendages - loss of rete pegs - compact bundles of collagen
  32. 32. Digital pitting scars
  33. 33. calcinosis
  34. 34. Telangiectasia  Face / mucous membrane  blanched by pressure
  35. 35. Musculoskeletal  Generalized arthralgia and morning stiffness  Erosive arthropathy has been demonstrated to occur in some series in as many as 29 percent of patients.  An inexorable loss of hand function is the rule as skin thickening worsens and the underlying joints become tethered and restricted in motion.
  36. 36.  Insidious muscle weakness, both proximal and distal, occurs in many patients with systemic sclerosis secondary to disuse atrophy  Subcutaneous calcinosis occurs in around 40 percent of patients with long-standing limited scleroderma and less frequently in diffuse disease
  37. 37. Calcinosis and acrolysis
  38. 38. Intestinal involvement 1) esophagus: hypomotility and retrosternal pain, reflux esophagitis, stricture 2) stomach: delayed emptying 3) small intestine: pseudo-obstruction, paralytic ileus, malabsorption 4) large intestine: chronic constipation and fecal impaction diverticula
  39. 39. Gastrointestinal  Disordered peristalsis of the lower two thirds of the esophagus presents as dysphagia  Impaired function of the lower esophageal sphincter  chronic esophageal reflux include erosive esophagitis with bleeding, Barrett's esophagus, and lower esophageal stricture  Involvement of the stomach occurs in systemic sclerosis and presents clinically as ease of satiety and on occasion as either functional gastric outlet obstruction or acute gastric dilatation.
  40. 40.  Small bowel involvement  Intermittent bloating with abdominal cramps, intermittent or chronic diarrhea, and presentations suggestive of intestinal obstruction.  Malabsorption occurs  Bacterial overgrowth in areas of intestinal stasis occurs frequently
  41. 41. Diverticula
  42. 42.  Colonic involvement…  is present in the majority of patients with systemic sclerosis  is infrequently a prominent cause of clinical symptoms.  and constipation, obstipation, and pseudo-obstruction may occur and are related to abnormal colonic motility
  43. 43. Lungs 1) 2/3 of patients affected - leading cause of mortality and morbidity in later stage of systemic sclerosis 2) pathology - interstitial fibrosis - intimal thickening of pulmonary arterioles (pulmonary hypertension)
  44. 44.  Pulmonary manifestations  Progressive dyspnea on exertion, limited effort tolerance, and a nonproductive cough
  45. 45. Interstitial thickening and fibrosis and continuing evidence of interstitial inflammation.
  46. 46.  Pulmonary Hypertension  Individuals with limited systemic sclerosis may also develop interstitial disease but are also at risk for progressive pulmonary hypertension in the absence of interstitial change, a complication most typical of long- standing disease
  47. 47. Heart (10%) 1) pericarditis 2) heart failure 3) arrhythmia 4) myocardial fibrosis
  48. 48. Kidney 1) diffuse scleroderma in association with rapid progression of skin involvement 2) pathology - intimal hyperplasia of the interlobular artery - fibrinoid necrosis of afferent arterioles - glomerulosclerosis 3) proteinuria, abnormal sediment, azotemia, microangiopathic hemolytic anemia (scleroderma renal crisis), renal failure
  49. 49. Diagnosis  major criteria: proximal scleroderma  minor criteria:  sclerodactyly  digital pitting scar or loss of substance from the finger pads  bibasilar pulmonary fibrosis  * one major or 2 or more minor criteria for diagnosis
  50. 50. Prognosis  Quite variable and difficult to predict  Cumulative survival  diffuse limited  5 yr 70% 90%  10 yr 50% 70%  Major cause of death  renal involvement  cardiac involvement  pulmonary involvement

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