Exam 1 Material : Scleroderma

Question 1

What is Scleroderma?

Answer 1

Connective Tissue Disorder that causes collagen overproduction, which leads to fibrotic, degenerative changes to skin, blood vessels, muscle, synovial membranes and internal organs

Question 2

True or false? Scleroderma is most common in African Americans?

Answer 2

True

Question 3

True or false? The usual onset age for Scleroderma is from 18 to 30?

Answer 3

False. It is from ages 30-50.

Question 4

What is the cause of Scleroderma?

Answer 4

Unknown

Question 5

True or false? Scleroderma is more common in women than in men?

Answer 5

True

Question 6

What is a key clinical manifestation of scleroderma?

Answer 6

Tightening of skin and organs due to sclerosis and thickening.

Question 7

What are the two different types of Scleroderma?

Answer 7

Localized and Diffuse Scleroderma.

Question 8

What is difference between Localized and Diffuse Scleroderma?

Answer 8

Localized only has symptoms limited to the face, hands, lower arms, feet and lower legs. 80% of patients have localized

Diffuse scleroderma has symptoms all of the above and has progressed to the trunk thus affecting all organs. Lung, heart and renal disease are prominent.

Question 9

What does CREST stand for?

Answer 9

Calcinosis
Raynaud's phenomenon 
Esophageal Dysfunction 
Sclerodactyly 
Telangiestasia

Question 10

What is Calcinosis?

Answer 10

Painful calcium deposits within the subcutaneous tissues

Question 11

What is Raynaud;s phenomenon?

Answer 11

Restricted extremity blood flow in response to cold or stress

Question 12

What is Sclerodactyly?

Answer 12

Tightening of the skin on the fingers and toes. Skin becomes shiny and tihgt. Facial skin appears tight and mask like.

Question 13

What is Telangiectasia?

Answer 13

Red spots on the hands/palms, forearms, face and lips

Question 14

What is Esophageal Dysfunction?

Answer 14

Scarring leading to strictures of the esophagus.

Question 15

True or False? CREST is the list of signs for Diffuse Scleroderma?

Answer 15

True

Question 16

What are the diagnostic tests for Scleroderma?

Answer 16

Anticentromere antibody. (60-80% of patients have these)

UA for proteinuria and elevated creatinine

CXR, Pulmonary Function test and ECG

Question 17

What drugs are used for drug therapy for Scleroderma?

Answer 17

Diltiazem to treat A-Fib

Lisinopril to treat HTN and Pulmonary HTN due to sclerosis of Blood vessels.

Vasoactive drugs in order to improve symptoms of Raynaud’s Phenomenon

Immunosuppressants to slow progression

Question 18

What cardiac disorders can come from Scleroderma?

Answer 18

Heart muscles become fibroses.

Pericarditis

Pericardial Effusion

Dysrhythmias

HF

Question 19

What pulmonary disorders can come from Scleroderma?

Answer 19

Pulmonary HTN and interstitial Lung disease (main cause of death for scleroderma patients)

Question 20

What non-pharm care can be done for Scleroderma Patients?

Answer 20

Protect hands and feet from cold and injury

Small Frequent meals and avoid lying down 1 to 2 hours after eating due to esophageal dysfunction

Good Oral hygiene

Body image support