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.

1. Scleroderma
systemic sclerosis
Dr / Hytham Nafady

2. Definition
Multisystem collagen vascular disease of
unknown etiology characterized by
• fibrosis of the skin with
• involvement of the internal organs.

3. Scleroderma
Diffuse
systemic sclerosis
Localized
scleroderma
Diffuse cutaneous
systemic sclerosis
limited cutaneous
systemic sclerosis
Overlap syndromes Morphea Linear scleroderma
CREST syndrome
Scleroderma +
RA or SLE or
dermatomyositis
Early visceral
involvement

4. Demographics
• Age: Middle age (30-50).
• Sex: F > M (3:1).

5. CREST syndrome

6. Pulmonary manifestations of
scleroderma
Bibasilar interstitial lung disease:
• Ground glass opacification.
• Reticular opacities & interlobular septal
thickening.
• Honey combing & traction bronchiectasis.
NO pleural effusion (DD with Rh & SLE).

8. GIT manifestations of scleroderma
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 muscle atrophy
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
coin sign
= accordion
sign

17. Colonic scleroderma
Motility:
Reduced colonic transit 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.

21. Acro-osteolysis
terminal phalanx resorption