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Flashcards in Scleroderma Deck (14):
1

Scleroderma

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.

2

What is diffuse scleroderma

widespread and rapidly progressive skin thickening
proximal to elbows, knees; early visceral disease (lung, heart, kidney)

3

What is limited scleroderma

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

4

Definition of Scleroderma

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

5

6 Symptoms of Scleroderma

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

6

Labs for Scleroderma

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

7

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

Raynauds Phenomenon

8

General Measure treatments for Raynauds

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

9

Medication treatment for Raynauds

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

10

Major complications of Diffuse Scleroderma

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

11

Major complications of Limited Scleroderma

Pulmonary hypertension
40% have Interstitial lung disease (ILD)

12

Disease modifying agents for systemic sclerosis

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

13

CREST syndrome (variant of Scleroderma)

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

14

Limited Cutaneous Systemic Sclerosis

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