Scleroderma

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Scleroderma

  1. 1. Sclerodermasystemic sclerosisDr / Hytham Nafady
  2. 2. DefinitionMultisystem collagen vascular disease ofunknown etiology characterized by• fibrosis of the skin with• involvement of the internal organs.
  3. 3. SclerodermaDiffusesystemic sclerosisLocalizedsclerodermaDiffuse cutaneoussystemic sclerosislimited cutaneoussystemic sclerosisOverlap syndromes Morphea Linear sclerodermaCREST syndromeScleroderma +RA or SLE ordermatomyositisEarly visceralinvolvement
  4. 4. Demographics• Age: Middle age (30-50).• Sex: F > M (3:1).
  5. 5. CREST syndrome
  6. 6. Pulmonary manifestations ofsclerodermaBibasilar interstitial lung disease:• Ground glass opacification.• Reticular opacities & interlobular septalthickening.• Honey combing & traction bronchiectasis.NO pleural effusion (DD with Rh & SLE).
  7. 7. GIT manifestations of sclerodermaLocation:• Esophagus (most common location).• Stomach.• Small bowel.• Colon.
  8. 8. Esophageal sclerodermaLocation:Lower 2/3 (contain smooth muscles).Pathology:Smooth muscle atrophy.Motility disorder:Decreased peristalsis.Reflux.Radiological:• Dilatation of the lower 2/3 of the esophagus.• Patulous gastro-esophageal junction.• Loss of longitudinal folds.• Esophageal candidiasis.
  9. 9. Gastric sclerodermaLocation:Pathology:Smooth muscle atrophyMotility disorder:• Delayed gastric emptying (fluoroscopy).Radiological manifestations:• Dilated stomach.Endoscopy:• Water melon stomach (gastric antraltelangiectasia)
  10. 10. Small bowel sclerodermaLocation:• Most common duodenum.Pathology:Preferential atrophy of the inner circular smooth muscle layerrelative to the outer longitudinal layerContraction of the longitudinal layer result in foreshortening ofthe bowel & packing of valvulae conniventes.Motility disorder:• Decreased peristalsis (fluoroscopy).• Delayed small bowel transit time.Radiological manifestations:• Hidebound sign (crowding of valvulae conniventes).• Small bowel dilatation (mega-duodenum or mega-jejenum).
  11. 11. Hideboundsign= stack ofcoin sign= accordionsign
  12. 12. Colonic sclerodermaMotility:Reduced colonic transit time.Radiology:Colonic dilatation.Loss of haustration.Pseudo-sacculation.
  13. 13. Skeletal manifestations ofsclerodermaLocation:• The hands are the most common location.Pathology:Radiology:Bone changes:• Acro-osteolysis (resorption of terminal phalanges).• Joint space narrowing.• Erosions.Soft tissue changes:• Subcutaneous & peri-articular calcification.• Atrophy specially at the tips of finger.• Flexion deformities.
  14. 14. Acro-osteolysisterminal phalanx resorption

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