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Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
Scleroderma
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Scleroderma

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  • 1. Sclerodermasystemic sclerosisDr / Hytham Nafady
  • 2. DefinitionMultisystem collagen vascular disease ofunknown etiology characterized by• fibrosis of the skin with• involvement of the internal organs.
  • 3. SclerodermaDiffusesystemic sclerosisLocalizedsclerodermaDiffuse cutaneoussystemic sclerosislimited cutaneoussystemic sclerosisOverlap syndromes Morphea Linear sclerodermaCREST syndromeScleroderma +RA or SLE ordermatomyositisEarly visceralinvolvement
  • 4. Demographics• Age: Middle age (30-50).• Sex: F > M (3:1).
  • 5. CREST syndrome
  • 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. GIT manifestations of sclerodermaLocation:• Esophagus (most common location).• Stomach.• Small bowel.• Colon.
  • 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. Gastric sclerodermaLocation:Pathology:Smooth muscle atrophyMotility disorder:• Delayed gastric emptying (fluoroscopy).Radiological manifestations:• Dilated stomach.Endoscopy:• Water melon stomach (gastric antraltelangiectasia)
  • 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. Hideboundsign= stack ofcoin sign= accordionsign
  • 12. Colonic sclerodermaMotility:Reduced colonic transit time.Radiology:Colonic dilatation.Loss of haustration.Pseudo-sacculation.
  • 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. Acro-osteolysisterminal phalanx resorption

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