Systemic sclerosis

Question 1

Presentation

Answer 1

Sclerotic skin over the face and fingers - smooth, shiny, tight
Loss of facial wrinkles, perioral puckering, restrictive mouth opening, nose appears pinched
Telangectasia of the face
Sclerodactyly flexion deformities
Dilated nail fold capillaries with dystrophic nails
Atrophy of soft tissue - digital ulceration
Rayndaud’s phenomenon
Palpable nodes of calcinosis

Question 2

Organ system involvement

Answer 2

Skin - Raynaud’s, sclerodactyly, soft tissue atrophy, calcinosis, oedema, telangectasia, vitiligo, morphoea, pigmentation, pruritis
MSK - arthritis, myositis, myopathy, osteopenia
GI - dysphagia, GORD, intestinal hypomobility - bacterial overgrowth, colonic diverticulae, SBO
Renal - malignant hypertension, glomerulonephritis, renal crisis
Resp - restrictive lung defects, ILD, pleural effusions, atelectasis
CV - restrictive cardiomyopathy, pericarditis, pericardial effusions, pulmonary hypertension, conduction defects

Question 3

Diffuse versus limited

Answer 3

Diffuse - involvement of trunk as well as extremities, early involvement of lungs, kidney, gut, heart
Limited - below elbow and face - late involvement of lungs

Question 4

CREST

Answer 4

Calcinosis
Raynaud’s
oEsophageal dysmotility
Sclerodactyly
Telangectasia

Question 5

Major cause of death

Answer 5

Renal failure

Question 6

Abs

Answer 6

Limited - anti-centromere, ANA, RF
Diffuse - Anti- ScL 70 (topoisomerase 1), anti-RNA polymerase

Question 7

Management

Answer 7

Patient education
PT
Analgesia
Raynaud’s - stop smoking, hand warmers, vasodilators (Ca channel blockers, ACEi, prostacyclin analogues)
GI - low residue diets, ABx for bacterial overgrowth, PPIs, prokinetic drugs
Renal - ACEi
Resp - calcium channel blockers, endothelin receptor antagonists, phosphodiesterase 5 inhibitors, prostaglandins