Systemic Sclerosis (scleroderma)

Question 1

epidemiology

Answer 1

adults (age 30-50 yo), female predominance

Question 2

clinical features

Answer 2

(1) skin thickening of fingers of BOTH hands proximal to MCP joint
(2) sclerodactyly
(3) matted telangiectasia
(4) dilated capillary loops alternating with capillary drop out on nails
(5) Raynaud’s phenomenon
(6) pulmonary findings (hypertension, interstitial lung disease)
(7) beaked nose
(8) calcinosis cutis
(9) dry, itchy skin
(10) salt + pepper sign (hyPOpig of trunk/face with perifollicular sparing)

Question 3

subtypes

Answer 3

limited (anti-centromere Ab) OR diffuse (anti-Scl70/anti-topoisomerase)

Question 4

what is the most common cause of mortality?

Answer 4

lung disease ( #1 interstitial lung disease); yes, believe it due to ACEI use for renal crisis prophylaxis

Question 5

what is the most common extracutaneous site of disease?

Answer 5

GI (esophageal dysmotility, gastroparesis, etc)

Question 6

what is scleroderma renal crisis?

Answer 6

kidney crisis in patients with diffuse scleroderma; starts with rapid rise in creatinine. PPX: ACEinhibitors

Question 7

how do we treat scleroderma?

Answer 7

focus on internal organ damage (e.g. pulm, renal, cardiac, GI, then skin). Skin treatments are often ineffective

Question 8

what is a new biomarker for skin and lung fibrosis, as well as pulmonary HTN?

Answer 8

CXCL4

Question 9

is this a TH1 or TH2 disease?

Answer 9

TH2