Connective Tissue Disorders

Question 1

What does SLE stand for?

Answer 1

Systemic Lupus Erythematosus

Question 2

What is SLE?

Answer 2

Auto-Antibodies vs components of Cell Nucleus

Question 3

What is pathophysiology of SLE?

Answer 3

Incomplete understanding
Cell apoptosis ⇒ Nuclear Ag’s released
Nuclear Self-Antigen’s presented to Immune cells
B cell mediated Autoimmunity
Healthy tissue damage by 
Complement activation
Fc receptor engagement

Question 4

What is the demographic of SLE?

Answer 4

1. Px : 1 – 40 yo
2. F:M = 9:1
3. ↑ prevalence in African & Asian ancestry population

Question 5

What is the management goal of SLE?

Answer 5

Remission or ↓ Disease activity & Prevention of flares

Question 6

What is the treatment for SLE?

Answer 6

1. Hydroxychloroquine
2. Symptomatic Relief
3. Cyclophosphamide & B cell MAb’s

Question 7

What is the Risk Factor Modification in SLE?

Answer 7

Antiphospholipid Syndrome – Anticoagulants
Assess Infection + Cardiovascular risk
Planned pregnancy

Question 8

What are the symptomatic relief treatments for SLE?

Answer 8

1. Glucocorticoids – Symptomatic Relief (Caution LT use)

2. Immunomodulatory agents (e.g. methotrexate, azathioprine, mycophenolate) to help ↓ GC use

Question 9

What are the two types of scleroderma?

Answer 9

1. Diffuse Scleroderma

2. Limited Scleroderma

Question 10

What is the aetiology of Sjogen’s syndrome?

Answer 10

Can be 1° or 2° to another CT disorder

Question 11

What is the investigation for Sjogen’s syndrome?

Answer 11

Schirmer’s Test

Question 12

What is Schirmer’s Test?

Answer 12

Add filter paper under the eye, measure wetness for 5 min

Question 13

What is the pathophysiology of Sjogen’s syndrome?

Answer 13

Autoimmune Exocrinopathy:

-Lymphocytic infiltration of exocrine glands + sometimes extra glandular involvement

Question 14

What are the symptoms for Sjogen’s syndrome?

Answer 14

1. Glands:
   - Dry eyes (xeropthalmia)
   - Dry mouth (xerostoma)
   - Parotid enlargement
2. Extra glandular involvement:
   - Non erosive arthritis
   - Raynaud’s phenomenon

Question 15

What is medical term for dry eyes?

Answer 15

Xeropthalmia

Question 16

What is the medical term for dry mouth?

Answer 16

Xerostoma

Question 17

What is xeropthalmia?

Answer 17

Dry eyes

Question 18

What is xerostoma?

Answer 18

Dry mouth

Question 19

What is the aetiology for Autoimmune Muscle Disorders?

Answer 19

2 main types – Polymyositis, Dermatomyositis

= Proximal Muscle weakness ± Rash

Question 20

What are the symptoms for Polymyositis?

Answer 20

1. Proximal Muscle weakness

Question 21

What are the symptoms for Dermatomyositis?

Answer 21

1. Proximal Muscle Weakness
2. Heliotrope Rash
3. Gottron’s papules
4. Subcutaneous calcinosis
5. Mechanic Hands

Question 22

What investigations are done for Autoimmune Muscle Disorders?

Answer 22

1. ↑ Serum Creatinine Kinase
2. Abnormal EMG
3. Abnormal Muscle Biopsy
   - Polymyositis = CD8
   - Dermatomyositis = CD4

Question 23

What are the associations with Autoimmune Muscle Disorders?

Answer 23

Malignancy (10-15%)

Pulmonary Fibrosis

Question 24

What abnormal muscle biopsy is found in Polymyositis?

Answer 24

CD8

Question 25

What abnormal muscle biopsy is found in Dermatomyositis?

Answer 25

CD4

Question 26

What Autoimmune Muscle Disorder is CD8 found in an abnormal muscle biopsy?

Answer 26

Polymyositis

Question 27

What Autoimmune Muscle Disorder is CD4 found in an abnormal muscle biopsy?

Answer 27

Dermatomyositis