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.
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).
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.