Scleroderma

Question 1

Scleroderma most commonly present with

Answer 1

1. Female, estrogen dominant phase
2. Fingers - polyarthritis, SAUSAGE FINGERS
3. Raynaud phenonmenon
4. BIRD LIKE facies
5. ROMAN BREASTPLATE chest
6. Lung - fibrosis, Cor pulmonale
7. Heart - Pulm artery hypertension, pericarditis
8. GIT - esophageal dysmobility, GERD, malabsorption
9. Renal - Scleroderma renal crisis, ARF, high creatinine and proteinuria (rx: ACE inhibitors)

Skin/tissue - fibrosed and contacted

Question 2

Leading cause of death in scleroderma

Answer 2

Pulmonary fibrosis
Previously renal failure (creatinine and proteinuria)- now rx by ACE inhibitors

Question 3

Mx for Raynaud phenomenon

Answer 3

CCB
Avoid vasospasm (smoking, B-blockers, ergotamine)

Question 4

Mainstay Rx for scleroderma

Answer 4

D Penicillamine

Question 5

Clinical variants of scleroderma

Answer 5

1. Morphea- only skin involved
2. CREST syndrome
3. Diffuse multisystem involved

Question 6

CREST syndrome (elaborate)/Limited cutaneous systemic sclerosis

Answer 6

C- Calcinosis (Calcium deposits on skin)
R- Raynauds
E- Esophageal dysmobility
S- Sclerodactyly
T- Telangiectasia

Question 7

Mxof scleroderma

Answer 7

1. ESR raises
2. ANA, Anti centromere, Anti topoisomerase +
3. Skin biopsy - dermal collagen
4. D Penicillamine