Lecture 20 - Scleroderma + Myositis

Question 1

The three main areas of the body affected by Systemic Scleroderma (SCL) are the ______ tract, _____, and spleen.

Answer 1

GI tract

Kidney

Spleen

Question 2

There are two flavors of SCL:

1. Diffuse cutaneous
2. Limited cutaneous (limited to the hand, face, and feet)

CREST is the acronym to describe the common symptoms of Limited Cutaneous SCL. What do the letters stand for?

Answer 2

C - Calcinosis
R - Raynaud’s
ES - Esophageal dysmotility (frequent heartburn)
T - Telangiectasia (spider veins)

Question 3

For SCL patients that present with Raynaud’s, anti-hypertensive _____ channel blockers are prescribed during cold months.

Answer 3

Calcium

Question 4

The most common cause of death in SCL patients is _____ disease (either fibrosis or hypertension).

Answer 4

Lung disease (pulmonary fibrosis or Pulmonary hypertension)

Question 5

SCL patients can also present with Scleroderma _____ crisis. These patients should be treated with ______ inhibitors, bc it is severe HTN that causes the problem in the first place.

Answer 5

Scleroderma Renal Crisis

ACE inhibitors

Question 6

The most common autoAbs tested for in an SCL ANA test are ____-70 and anti-______ Abs.

Answer 6

SCL-70

anti-Centromere

Question 7

Inflammatory muscle diseases present as ______ (symmetric or asymmetric?) _______ (proximal or distal?) muscle weakness. Lab tests should show an increase in _____ kinase. The most common types are polymyositis and dermatomyositis.

Answer 7

Symmetric

Proximal

Creatine

Question 8

Dermatomyositis patients present with a characteristic _______ rash around the eyes and _______ papules on the hands.

Answer 8

Heliotrope rash

Gottron’s papules

Question 9

The autoAb present in Myositis diseases is ___-1, an anti-tRNA synthetase Ab.

Answer 9

Jo-1