scleroderma

Question 1

• small discreet spots or larger patches of skin thickening.

Answer 1

Morphea

Question 2

– linear pattern of skin thickening.

Answer 2

Linear Scleroderma

coup de sabre – when localized to the forehead

Question 3

definition of scleroderma

Answer 3

fibrosis/hardening of the skin

Question 4

Three pathologic processes

Answer 4

1. Tissue fibrosis
2. Inflammation / Autoimmunity
3. Vascular damage

Question 5

localized scleroderam

Answer 5

only in skin

Question 6

is it common to see scleroderma with genetics in RA?

Answer 6

yep but RA is more common

Question 7

scleroderma increases/decreases with age

Answer 7

increases

Question 8

Environmental Factors with scleroderma

Answer 8

Have been explored but none have been universally found in all people with scleroderma.
Silica / Silicone
Organic solvents
Drugs (e.g. tryptophan, bleomycin, and analgesic abuse)

Question 9

Antibody
Specific for limited scleroderma
Not sensitive
Good PPV

Answer 9

Anti-centromere

Question 10

Antibody / Anti-topoisomerase Ab
Specific for diffuse scleroderma
Not sensitive
Good PPV

Answer 10

Anti-Scl-70

Question 11

Using titer of 1:160
85-90% sensitive in Scleroderma
Good NPV

Answer 11

Anti-nuclear Antibody (ANA)

Question 12

hallmark of scleroderma

Answer 12

fibrosis/ thickening of the dermis caused by excessive accumulation of extracellular matrix in the dermis. specifically, monotonously similar collagen fibers are present in the reticular dermis

Question 13

other pathologic findings of scleroderma:

1. _________of papillary dermis and epidermis
2. __________of the papillarity of the dermoepidermal junction
3. ______ of the rete peg formation
4. _______ of the distinction of the papillary and reticular dermis

Answer 13

1. Thinning of papillary dermis and epidermis
2. Loss of the papillarity of the dermoepidermal junction
3. Loss of the rete peg formation
4. Loss of the distinction of the papillary and reticular dermis

Question 14

skin disease starts distally or proximal?

Answer 14

distal and then progresses proximanl

Question 15

In the early stages of the skin disease there is an infiltration of

Answer 15

mononuclear inflammatory cells

Mostly T cells
Surrounding dermal blood vessels
Concentrated at the border between the reticular dermis and subcutaneous fat

Question 16

Extent of involvement of the skin defines whether a person has limited or diffuse skin disease.

1. involvement of areas only distal to the elbows and knees.
2. involvement of areas proximal to the elbows and/or knees.

Answer 16

1. Limited disease: involvement of areas only distal to the elbows and knees.
2. Diffuse disease: involvement of areas proximal to the elbows and/or knees.

Question 17

Why distinguish between limited and diffuse disease?

Answer 17

The distinction is useful prognostically

Diffuse disease has a worse prognosis
Cardiac abnormalities
Interstitial lung disease
Renal crisis

Higher mortality rate
Especially in the first 3 years of disease

Question 18

scleroderma is the most active in

Answer 18

the first three years of the disease

Question 19

CREST

Current utility: good way to remember many of the manifestations of systemic sclerosis – in both limited and diffuse scleroderma.

Answer 19

Acronym
Calcinosis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly
Telangectasias

Question 20

raynaud’s phenomenon

Answer 20

russia flag- from white to blue to red

Clinical manifestation of microvascular compromise

Pallor is caused by the constriction of digital arteries.
Cyanosis is caused by the de-oxygenation of static venous blood.
Hyperemia is related to reperfusion of the affected regions.

Question 21

raynaud’s phenomenon:

• Digital arterial _________ proliferation
• Collagenous hyperplasia/hypoplesia
• Obliteration of the ___________lumen

Answer 21

• Digital arterial intimal proliferation
• Collagenous hyperplasia
• Obliteration of the arterial lumen

Question 22

treatment of raynaud’s phenomenon

Answer 22

1. conservative therapy by keeping core temperature warm

2. vasodilators- nifedipine, sildenafil and epoprostenol (CCB, cGMPP5 inhibitro and prostaglandin agonist)

Question 23

Arterial-venous malformations (AVMs) 
Often occur on:
Face (including lips)
Upper chest, palms
Fingers
Mucosal surfaces (anywhere in the GI tract) 

Helpful in making a diagnosis of scleroderma

Treatment is often cosmetic
Exception: when AVM occur in the GI tract  
Tendency to bleed and in the GI tract  
It is difficult to control hemostasis
Treatment 
Laser photocoagulation
Argon plasma coagulation

Answer 23

telangectasias

Question 24

renal crisis is more common in those with ____ disease

Answer 24

diffuse

10 – 15% of Scleroderma patients

Characterized by triad of:
Acute onset of renal failure
Abrupt onset of moderate or marked HTN
Microangiopathic hemolytic anemia

If left untreated, invariably go on to have renal failure and/or die.

Question 25

renal crisis results from a __________ thickening of renal arteriolar vessels

Answer 25

intimal

Question 26

treatment of the renal crisus

Answer 26

ace inhibitors- most useful because of the high renin state in renal crisis

Question 27

cardiac involvement is more common in those with ____ disease

Answer 27

diffuse- most common is pericardial effusion

Question 28

lung involvement is more common in those with ____ disease

Answer 28

diffuse- ILD and fibrosis

Question 29

pulmonary HTN in scleroderma?

Answer 29

yep due to intimal fibrosis

Question 30

treatment of Pulm HTN

Answer 30

1. vasodilation
2. endothelial receptor antagonist- action of endothelin molecules that would otherwise promote narrowing of the blood vessels and lead to high blood pressure

Question 31

What common histologic finding occurs in Raynaud’s phenomenon, pulmonary hypertension and renal crisis?

1. Vasculitis of the arterioles
2. Fibrosis of the vascular intimal with excess collagen accumulation
3. Fibrosis of the vascular media with excess collagen accumulation
4. Fibrosis of the vascular adventitia with excess collagen accumulation
5. Capillaritis

Answer 31

2. Fibrosis of the vascular intimal with excess collagen accumulation