Sjogren's, Sclerosis, Polymyositis

Question 1

What are autoantibodies?

Answer 1

Antibodies that attack self-antigens

Question 2

What multisystem disease is characterized by fibrosis of skin and internal organs?

Answer 2

Systemic sclerosis/scleroderma

Question 3

What are the initial symptoms of systemic sclerosis?

Answer 3

Raynaud’s phenomenon, fatigue, MS complaints

Question 4

What are the dermatologic symptoms of systemic sclerosis? (three phases)

Answer 4

Phase I: Edematous Phase - Puffy skin, non-pitting edema

Phase II: Fibrotic Phase - skin tightening and fibrosis, salt & pepper appearance

Phase III: Atrophic Phase - no folds in skin, very thin and tight, can lead to ulcers and ischemia

Question 5

What is Raynaud’s phenomenon? What triggers it? What meds can exacerbate it?

Answer 5

Closure of muscular digital vessels leading to color changes-

cold or stress, exacerbated by beta-blockers

Question 6

What organ is primarily affected in systemic sclerosis?

Answer 6

Lungs - pneumonia, pulmonary HTN

Question 7

What crisis do you have to watch out for with systemic sclerosis? What do you treat it with?

Answer 7

Scleroderma renal crisis - accelerated HTN - treat with ACE inhibitors

Question 8

What are CREST symptoms? What type of scleroderma autoantibody type are they associated with

Answer 8

Calcinosis, Raynaud’s phenom, Esophageal dysmotility, Sclerodactyly, Telangectasias

Assoc with anti-centromere autoantibodies

Question 9

Where is muscle weakness first seen in in polymyositis? What other systemic complications can occur?

Answer 9

Shoulder and pelvic girdles

Cardiac, GI (esophageal), pulmonary

Question 10

What skin conditions can be seen with dermatomyositis?

Answer 10

Gottron’s papules - plaques on knuckles

Heliotrope rash - violet discoloration of eyelids

V-neck rash

Nailfold capillaries

Question 11

What type of autoantibodies are often found in patients with dermato or poly-myositis?

Answer 11

Antinuclear antibodies (ANA)

Question 12

What type of T cells infiltrate muscle fibers in polymyositis?

Answer 12

CD8+ (cytotoxic immune response)

Question 13

What type of T cells infiltrate muscle fibers in dermatomyositis?

Answer 13

CD4+ (humoral immune response)

Question 14

What is used to treat dermato or poly-myositis?

Answer 14

Corticosteroids

Question 15

What disorder is associated with lymphocytic infiltration of exocrine glands leading to dysfunction of salivary and lacrimal glands?

Answer 15

Sjogren’s Syndrome

Question 16

What 2 symptoms are seen in most people with people with Sjogren’s Syndrome?

Answer 16

1. Dry eyes - keratoconjunctivitis sicca

2. Dry mouth - xerostomia