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Scleroderma
Scleroderma is a multisystem collagen vascular disease characterized by fibrosis of the skin and involvement of internal organs. It can be classified as diffuse or localized systemic sclerosis. Common manifestations include pulmonary fibrosis, esophageal dysmotility causing reflux, delayed gastric emptying, and small bowel involvement seen as "hidebound sign". Skeletal involvement causes acro-osteolysis, joint space narrowing, and flexion deformities of the hands.
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Scleroderma
- 1. Scleroderma systemic sclerosis Dr /Hytham Nafady
- 2. Definition Multisystem collagen vasculardisease of unknown etiology characterized by • fibrosis of the skin with • involvement of the internal organs.
- 3. Scleroderma Diffuse systemic sclerosis Localized scleroderma Diffuse cutaneous systemicsclerosis limited cutaneous systemic sclerosis Overlap syndromes Morphea Linear scleroderma CREST syndrome Scleroderma + RA or SLE or dermatomyositis Early visceral involvement
- 4. Demographics • Age: Middleage (30-50). • Sex: F > M (3:1).
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- 6. Pulmonary manifestations of scleroderma Bibasilarinterstitial lung disease: • Ground glass opacification. • Reticular opacities & interlobular septal thickening. • Honey combing & traction bronchiectasis. NO pleural effusion (DD with Rh & SLE).
- 8. GIT manifestations ofscleroderma Location: • Esophagus (most common location). • Stomach. • Small bowel. • Colon.
- 9. Esophageal scleroderma Location: 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.
- 12. Gastric scleroderma Location: Pathology: Smooth muscleatrophy Motility disorder: • Delayed gastric emptying (fluoroscopy). Radiological manifestations: • Dilated stomach. Endoscopy: • Water melon stomach (gastric antral telangiectasia)
- 14. Small bowel scleroderma Location: •Most common duodenum. Pathology: Preferential atrophy of the inner circular smooth muscle layer relative to the outer longitudinal layer Contraction of the longitudinal layer result in foreshortening of the 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).
- 15. Hidebound sign = stack of coinsign = accordion sign
- 17. Colonic scleroderma Motility: Reduced colonictransit time. Radiology: Colonic dilatation. Loss of haustration. Pseudo-sacculation.
- 20. Skeletal manifestations of scleroderma Location: •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.
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