Scleroderma HY

Question 1

Nail pitting, digital ischemia in cold weather (Raynaud’s), chronic heartburn (Esophageal Dysmotility)
dx/mccod?

Answer 1

CREST Scleroderma
Pulmonary HTN
────
Calcinosis cutis
Raynaud’s
Esophageal dysmotility
Sclerodactyl
Telangiectasia

Question 2

39F h/o Scleroderma presents with
HTN emergency (250/130) + Schistocytes
↑ Cr (3.0)
↓↓PLTs (25k)
dx/tx?

Answer 2

Sclerodermal Renal Crisis
ACE-I

Question 3

Pulmonary manifestations/pathology
in Limited cutaneous CREST Scleroderma?

Answer 3

Pulmonary Arterial HTN

non-inflammatory blood vessel narrowing s/t scarring & fibroses of the inner lining (endothelium) of the pulmonary vasculature

• CXR → absence of infiltrates (nl lung parenchyma)
• PFTs → all Normal (differentiate from PAH s/t lung or heat dz)
───
U World says → Intimal smooth muscle hyperplasia of the pulmonary arteries)

Question 4

Pulmonary manifestations/pathology
in Diffuse Scleroderma?

Answer 4

1st → Interstitial lung disease
s/t systemic inflammation and progressive scarring & fibrosis of the lung parenchyma!

2nd → Pulmonary Arterial HTN

Question 5

Skin involvement with Limited cutaneous CREST Scleroderma?

Answer 5

Involves half of the extremities
(ex: from elbow/knee to fingers/toes)
± Head

Question 6

Skin involvement in Diffuse Scleroderma?

Answer 6

Involves the full extremity
(ex: shoulder to fingers & hips to toes)
± Truncal
(Chest, Abdomen, Back)

Question 7

Classic rheumatologic drug to avoid in pts with Scleroderma?

Answer 7

Steroids

(increase risk of renal crisis)

Question 8

Don’t focus on details just big picture

Answer 8