Scleroderma

Question 1

Scleroderma

Answer 1

The term scleroderma is used to describe thickened or hardened skin

When the characteristic skin disorder is associated with internal organ involvement it is considered diffuse.

Question 2

What is diffuse scleroderma

Answer 2

widespread and rapidly progressive skin thickening

proximal to elbows, knees; early visceral disease (lung, heart, kidney)

Question 3

What is limited scleroderma

Answer 3

restricted and non-progressive skin thickening (distal extremities); later visceral disease EXCEPTION-ILD
not above the elbow and not above the knees

Question 4

Definition of Scleroderma

Answer 4

Fibrosis that can exhibit in the skin
Other locations that can be affected include:
kidneys
lungs (ILD)
cardiac (right sides heart failure)
GI tract with esophageal dysmotility
MS system

Question 5

6 Symptoms of Scleroderma

Answer 5

Fatigue 					76 % 
Stiff joints 				74 % 
Loss of strength			68 %
Pain					67%
Sleep					66%
Skin discoloration

Question 6

Labs for Scleroderma

Answer 6

95% of patients have + ANA
95% of patients have sclerodactly
90% have Raynaud’s phenomenon
Think of scleroderma-like skin disease if 2 of 3 are present

Question 7

What can be the first symptom/sign seen in patient with SSc or CREST Syndrome

Answer 7

Raynauds Phenomenon

Question 8

General Measure treatments for Raynauds

Answer 8

Avoidance of cold exposure,
dressing warmly
winter gloves, chemical hand warmers, and heavy wool stockings
hands under warm water
Under axilla
Avoidance of smoking
Avoidance of sympathomimetic drugs (ADHD dexamphetamine)
Control or limitation of emotional stress

Question 9

Medication treatment for Raynauds

Answer 9

Calcium channel blockers (nifedipine 30-180mg amlodopine 5-20mgs)
Watch for pressure drop and light headedness
Phosphodiesterase 5 inhibitor: sildenafil (Viagra™)
Topical nitrates

Question 10

Major complications of Diffuse Scleroderma

Answer 10

Scleroderma renal crisis
Pulmonary complications
Cardiac disease– r sided heart failure

Question 11

Major complications of Limited Scleroderma

Answer 11

Pulmonary hypertension

40% have Interstitial lung disease (ILD)

Question 12

Disease modifying agents for systemic sclerosis

Answer 12

Used only in early diffuse SSc
None yet proved beneficial in double-blind, placebo-controlled trials
Organ-based strategies (referral to rheumatologists)
Symptomatic treatments
Need antifibrotic agent.

This is a rheumatology referral

Question 13

CREST syndrome (variant of Scleroderma)

Answer 13

C calcinosis	
R raynaud’s
E esophageal dysmotility
S sclerodactyly
T telangestasia

Question 14

Limited Cutaneous Systemic Sclerosis

Answer 14

Also known as CREST

Skin changes limited to symmetrical change of fingers (sclerodactyly), distal arms, legs and face/neck

Progression of disease after the onset of Raynaud’s phenomenon
Later visceral disease compared to diffuse variant

Also associated with autoantibodies

Relatively good prognosis

Survival >70% at 10 years