Scleroderma

Question 1

autoantibodies associated with limited systemic sclerosis

Answer 1

anti-centromere, ANCA, rheumatoid factor

Question 2

autoantibodies associated with diffuse sytemic sclerosis

Answer 2

anti-Scl 70 (toposomerase 1)

anti RNA polymerase I, II, III

Question 3

autoantibodies associated with mixed connecrive disase

Answer 3

anti-ribonuclear protien (RN) antibody.

Question 4

CREST syndrome associated with which scleroderma?

Answer 4

limited scleroderma

Question 5

CREST syndrome

Answer 5

calcinosis
raynaud's phenomenon
eosphageal dysmotilgiy
sclerodactyliy
telangiectasia

Question 6

most common cause of mortality in systemic sclerosis

Answer 6

renal failture is the major cause of death.

Question 7

diffuse scleroderma classifed by what features

Answer 7

diffuse involvement of skin, trunk and extremities.

early involvement of lungs, kidneys, gut and heart.

Question 8

limited sceroderma features

Answer 8

skin affected only at the extremieties +/- face
CREST
LATE invovlement of lungs
late developement of pulmonary hypertension
renal crises are rare.

Question 9

What organs are involved in systemic sclerosis

Answer 9

skin: raynauds phenomenon, sclerodactyliy, soft tissue atrophy, calcinosis, nail dystrophy, ulceration, gangrene, flexion deformities, oedema, telangiectasia, vitiligo, morphoea, pigmentation, pruritis.
musculos

Musculoskeletal: arthritis, myosistis, myopathy, intraarticular calcification, osteopenia

Gastrointestinal: dysphagia, GORD, intestinal hypomotility-> bacterial overgrowth-> steatorrhoea and malabsorption, pneumatosis coli, colonic diverticulae, bowel obstruction, primary biliary cirrhosis.

renal: malignant hypertension, glomerulonephritis, renal crises
respiratroy: interstitial lung disease, restrictive lung deffect, pleural effusion

cardiovascular: restrictive cardiopyopathy, pericarditis, pulmonary hypertension, conduction defect.

Question 10

sclerotic skin over face and fingers
smooth, shiny and tight.
perioral puckering, restrictinve mouth opening, pinched nose, telangiectasia, sclerodactly with flexion deformities, dilated nail-fold capillaries with dystrophic nails. atrophy of soft tissues particualry the finger pulps, digital ulcertation and gangrene. Raynod’s pheonomenon.
calcinosis.

Answer 10

systemic sclerosis.

Question 11

raynaud’s phoenomenon management

Answer 11

smoking cessation
handwarmers

vasodilators ( calcium antagonist, ACEI, prostacyclin anagolues)
sympathectomy in severe cases

Question 12

gastrointestinal sx management

Answer 12

low residue diets and nutrional supplements for malabosorption
PPI fror reflus
prokinetic durgs for dysmotility
abx for bacterial overgrowth

Question 13

renla s management

Answer 13

aggressive management of HTN, ACEI

ACE for renal crises

Question 14

respiratory:

Answer 14

vasodilator therapy for pulmonary HTN

anti-fibrotic therapy for pulmonary fibrosis ( D-penicillamine and interferon r)