Scleroderma

Question 1

Clinical features of scleroderma

Answer 1

.best evaluated using a combination of endoscopy and barium x-ray study.
scleroderma = endoscopic findings relate to a hypotensive lower esophageal sphincter.
Symptoms include heartburn, dysphagia due to stricture or esophageal dysmotility, and pain due to diffuse spasm

Question 2

Clinical features of sjogren’s syndrome

Answer 2

.

Question 3

Distinctive autoantibodies that are associated with SSc and sjogren’s syndrome

Answer 3

.Diffuse = antibodies against topoisomerase 1 (scl-70)
Limited = antibodies against centromere

Question 4

Histopathology distinguishing SSc and Sjogren’s from other connective diseases

Answer 4

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Question 5

Types of scleroderma and distributions

Answer 5

Diffuse = all over the body
Limited = head, hands, legs below the thighs
Sine Sclerosis = internal organs, no hard skin

Question 6

Skin findings in scleroderma

Answer 6

• early = swelling of fingers and hands (puffy/edematous phase) with erythema and pruritis
• later = shiny, tight and thick with pigment changes, sclerodactyly and digital ulcers/pitting at the fingertips

Question 7

Characteristics of sine sclerosis

Answer 7

• 5% of people with scleroderma have sine sclerosis
• Raynaud’s, GI abnormalities, autoantibodies and telengiectasia
• diagnosis often delayed
• prognosis is the same as limited disease

Question 8

Characteristics of limited systemic sclerosis

Answer 8

CREST

• calcinosis - can appear in fingers, sodium thiosulfate can help them come to the surface and be removed, late manifestation, never occurs in diffuse disease
• raynaud’s phenomenon - can be the first symptom by months or years
• esophageal dysmotility
• sclerodactyly
• telengiectasia

Question 9

x-ray findings in fingers with calcinosis in Ssc

Answer 9

.

Question 10

Pattern of Raynaud’s phenomenon in SSc

Answer 10

• cold induced vasospasm with blanching/pallor, cyanosis, reactive hyperremia
• present in 95% of patients with SSc
• vasospasm and structural changes in the small blood vessels (vasculopathy)

Question 11

What are the structural changes seen in vasculopathy associates with SSc

Answer 11

• reduction in finger pad, tapering of fingers
• fingertip ischemia/necrosis
• necrosis over PIP/MCP/Ulnar styloid/Elbow
• Abnormal nail fold capillaries
• blood vessel wall thickening = actual lumen is blocked
  Can proceed to gangrene if blood flow is not restored

Question 12

most specific finding in making a diagnosis of CTD in patient with Raynaud’s

Answer 12

dilated nailfold capillaries

Question 13

Variation in nailfold capillary pathology in SSc

Answer 13

1. Dilated
2. Drop out and hemorrhage
3. Significant drop out of blood vessels = bad
   This is a helpful finding in a patient in whom you suspect raynaud’s secondary to a CTD

Question 14

Appearance of esophageal dysmotility on barium swallow

Answer 14

.

Question 15

Xray finding in SSc sclerodactyly

Answer 15

Resorption of the tufts of the terminal phalanges: acro-osteolysis
Also see joint deformity due to the skin tightening

Question 16

Appearance of SSc telengiectasia

Answer 16

Appears on fingers, hands and face

• less than 1mm but can grow
• in GI tract, can cause significant blood loss

Question 17

Differences between diffuse vs limited SSc

Answer 17

Diffuse: skin changes more on front than back of torso; early organ involvement; renal crisis; pulmonary fibbrosis; antibodies against topoisomerase 1 (Scl-70)

Limited: CREST, Calcinosis, pulmonary hypertension (10-15%), antibodies against centromere

Question 18

Pathophysiology of scleroderma includes:

Answer 18

• vasculopathy
• activation of cellular immunity
• activation of humoral immunity
• fibrosis
  DOES NOT include vasculitis