scleroderma

Question 1

What is the general definition of scleroderma?

Answer 1

autoimmune disease of excessive fibrosis and collagen deposition throughout the body. also see widespread damage to small vessels. often manifest as skin that is puffy and taut without wrinkles. distal to proximal spread of skin tightening

Question 2

What are some common sites that can be affected by scleroderma? Emergencies?

Answer 2

renal, pulmonary, CV, and GI. renal problems can cause hypertensive crises. you can also get RBC fragmentation as blood vessels are sheared when whipped through blood vessels

Question 3

Epidemiology of scleroderma, risk factors

Answer 3

75% female
high prevalence in choctaw native americans
occupational exposure to silica and organic solvents

Question 4

diffuse scleroderma

Answer 4

widespread skin involvement with rapid progression and early visceral invovlement. ascotiated with anti-Scl-70 ab (anti-DNA topoisomerase I antibody)

Question 5

limited scleroderma

Answer 5

limited skin involvement confined to fingers and face. CREST invovlement. more benign clinical course. associated with anti-centromere antibody

Question 6

CREST

Answer 6

symptoms that may be seen in limited scleroderma

Calcinosis, raynaud’s, esophageal dysmotility, sclerodactlyly, telangiectasia

Question 7

Systemic sclerosis: vascular diseases

Answer 7

this is a microvascular disease with intimal proliferation.
raynauds and peripheral ischemia. see weird capillary findings (loops and drop offs) in nail beds
telangectasias
renal crises
pulmonary HTN (can cause SOB)

Question 8

Systemic sclerosis: fibrosis involvement. location and pathogenesis

Answer 8

multiple factors: fibrogenic cytokines, hyperresposniveness to cytokines, scarring after ischemic damage
skin, muscles, tendons
visceral invovlement of GI tract, lungs, and heart
GI tract: watermelon stomach, esophageal fibrosis with strictures and food sticking and GERD; risk of poor absorption

Question 9

Raynaud’s: causes, description

Answer 9

episodic reversible digital skin color change (white to blue to red)- well demarcated
due to vasospasm
usually cold induced
may be primary or secondary

Question 10

primary vs. secondary raynaud’s

Answer 10

primary: onset in teens and 20s; mild-moderate, symmetric, rapidly reversible, to PIPs but spares thumb.
secondary: onset in 40s, 50s, and 60s., may be severe with ulcers, abnormal capillaries, and ANA+

Question 11

How do you differentiate between different pulmonary diseases of systemic sclerosis?

Answer 11

pulmonary fibrosis: looks different on chest CT, shows decreased FEV1 and normal FEV1/FVC
pulmonary HTN: normal FEV1 but decreased DLCO because you are missing much of the capillary bed

Question 12

life threats in scleroderma

Answer 12

renal crisis, pulmonary HTN, pulmonary fibrosis, heart failure

Question 13

types of localized scleroderma

Answer 13

linear scleroderma and morphea

Question 14

linear scleroderma and morphea: problems

Answer 14

linear: can cause limb shortening in a kid; can cause facial deformities
morphea: skin patches look like scleroderma on biopsy but no systemic involvement

Question 15

nephrogenic systemic fibrosis

Answer 15

induced by gadolinium in pts who get MRIs. now we won’t give gadolinium to pts with poor kidney function. gives mottled skin appearance

Question 16

eosinophilic fascititis

Answer 16

hx of bizarre physical activity (unusual exertion after inactivity)
contraction of the skin and tissue in an extremity. skin feels normal. tethering is below the skin. may have peau d’orange. involves the fascia rather than the dermis.
must treat with steroids.