Systemic Sclerosis

Question 1

morphea ≣

Answer 1

localized scleroderma

Question 2

What are the locations of limited vs diffuse systemic scleroderma?

Answer 2

Limited → feet to knees, hands to elbows, face and head

Diffuse → universal

Question 3

What are the early vs later signs of scleroderma?

Answer 3

early edematous phase → erythema, pruritis, swelling of fingers, hands, face
Late → shiny, tight, thick, sclerodactyly, digital ulceration

Question 4

What is Sine Scleroderma?

Answer 4

NIH → raynaud’s + scleroderma of organs and tissues, but not skin
Class → raynaud’s, GI abnormalities, auto-Abs, telangiectasias
5% of patients, Dx delayed, good prognosis

Question 5

CREST →

Answer 5

calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telangectasia

Question 6

How do you determine primary vs secondary Raynaud’s?

Answer 6

Primary → normal nail fold capillaries

Secondary → dilated nail fold capillaries

Question 7

What is Sclerodactyly?

Answer 7

resorption of the tufts of the terminal phalanges → acro-osteolysis
thickening and tightness of the skin of the fingers or toes → ulceration of fingers and atrophy of the underlying soft tissues.

Question 8

What are the differences between Diffuse and Limited scleroderma?

Answer 8

Diffuse → early organ involvement, renal crisis, pulm. fibrosis, Topo 1 (Scl-70) Ab
Limited → CREST, pulmonary HTN, centromere Ab

Question 9

What are the Antibodies for diffuse vs limited scleroderma?

Answer 9

Diffuse → Anti-Topo 1 (Scl-70)

Limited → Anti-centromere

Question 10

What are the influence on the Lungs for diffuse vs limited scleroderma

Answer 10

Diffuse → pulmonary fibrosis

Limited → pulmonary HTN

Question 11

What are the features of vasculopathy in scleroderma?

Answer 11

obliterative and failure to replace blood vessels
Concentric proliferation and thickening of INTIMA, fibrosis of ADVENTITIA, media left alone, smooth muscle hypertrophy
No inflammation

Question 12

What are features of pulmonary artery hypertension in scleroderma?

Answer 12

asymptomatic to rapidly progressive

Dx → ↓ DLCO, doppler, R heart cath

Question 13

What are the features of Scleroderma Renal Crisis?

Answer 13

Accelerated HTN
Oliguria
protinuria and hematuria

Question 14

Who is at risk for Scleroderma Renal Crisis?

Answer 14

diffuse, rapid skin thickening, < 4 years of dz
Steroids
Volume depleion
Anti-RNA pol III Ab

Question 15

What is pulmonary presentation in scleroderma?

Answer 15

Interstitial Lund Dz → 50% pfs
dyspnea, dry cough, velcro crackles
dcSSC is risk
PFTs → ristriction
CT → ground glass and opacities
occurs < 3 years of dz onset

Question 16

What is the GI presentation in scleroderma?

Answer 16

90% of patients
Esophageal hypomobility, LES → GERD, strictures, Gastroparesis,
Lower GI → hypomobility, obstruction, malabsorption, bacteria, wide mouth diverticulae

Gastric Antral Vascular Ectasia (GAVE) ≣ thin mucosa → looks like watermelon stripes

Question 17

What are common articular joint scleroderma effects?

Answer 17

arthralgia, stiffness
Sinovitis → Tx MTX
Tendon Friction Rubs → deposits on tendons → hear with stethoscop (specific finding)
Joint constractures → tendon and periarticualr fibrosis

Question 18

What are muscle effects of scleroderma?

Answer 18

bland myopathy → non-inflammatory muscle weakness with no CK elevation and poor response to steroids
Myositis overlap → dyspnea, dysphagia, CK DDx