8.1. Mulit-System Autoimmune Disease - Connective Tissue Diseases

Question 1

What are the 2 broad categories of Mulit-System Autoimmune Disease?

Answer 1

1. Connective Tissue Diseases

2. Systemic Vasculitis Disease

Question 2

What are the common Connective Tissue Mulit-System Autoimmune Disease?

Answer 2

1. Systemic Lupus Erythematous (SLE)
2. Scleroderma
3. Sjogren’s Syndrome
4. Auto-Immune Myositis
5. Mixed Connective Tissue Disease

Question 3

What are common Systemic Vasculitis Mulit-System Autoimmune Disease?

Answer 3

1. Giant Cell Arteritis
2. Granulomatosis Polyangiitis (Wegner’s Granulomatosis)
3. Microscopic Polyangiitis
4. Eosinophilic Granulomatosis Polyangiitis (Churg-Strauss Granulomatosis)

Question 4

Who commonly presents with Systemic Lupus Erythematous (SLE)?

Answer 4

1. Female (9:1)
2. Young (20% before 16, 65% between 16-55)
3. Afro-Caribbean > Asian > Caucasian

Question 5

What are the Classification Criteria for Systemic Lupus Erythematous (SLE)?

Answer 5

Any 4 of:

1. Malar Rash (Butterfly Rash)
2. Discoid Rash (Raised, Scarring, Permanent, Alopecia)
3. Photosensitivity
4. Oral Ulcers
5. Arthritis (2 Joints at least)
6. Serositis (Pleurisy or Pericarditis)
7. Renal (Significant proteinuria / cellular cast in urine)
8. Neurological (unexplained seizures / psychosis)
9. Haematological (Low WCC, Platelets, Lymphocytes, Haemolytic Anaemia)
10. Immunological (Lupus anticoagulant, Low complement)
11. Anti-Nuclear Antibodies (ANA)

Question 6

Who commonly presents with a Scleroderma Disorder?

Answer 6

1. Female (3:1)

2. Middle Ages (30 - 50 years old)

Question 7

What are the Scleroderma Disorders?

How do they present?

Answer 7

A Heterogenous Group of Conditions linked by the presence of Thickened, Sclerotic Skin Lesion
Note - Other manifestations of these conditions are quite diverse

Question 8

How do Scleroderma Disorders present?

Answer 8

The Presence of Thickened, Sclerotic Skin Lesion

Note - Other manifestations of these conditions are quite diverse

Question 9

What are the 3 types of Scleroderma Disorders?

Answer 9

1. Morphea
2. Limited
3. Diffuse

Question 10

What are some complications of Limited Scleroderma Disorders?

Answer 10

Pulmonary Hypertension

Question 11

What are some complications of Diffuse Scleroderma Disorders?

Answer 11

1. Pulmonary Fibrosis
2. Renal Crisis
3. Small Bowel Bacterial Overgrowth

Question 12

Who presents with Sjogren’s Syndrome?

Answer 12

1. Female (9:1)

2. Middle Aged (40 - 50 years old)

Question 13

What is the common presentation of Sjogren’s Syndrome?

Answer 13

1. Dry eyes and Mouth
2. Parotid Gland Enlargement
3. (1/3 have) Systemic Upset:
4. a) Fatigue
5. b) Fever
6. c) Myalgia (Muscle Pain)
7. d) Arthalgia (Joint Pain)

Question 14

What are some other presentations of Sjogren’s Syndrome?

Answer 14

1. G.I.: Dysphagia / Abnormal Oesophageal Motility
2. CNS: Fits / Hemiplegia / Ataxia / Cranial Nerve Lesions
3. Dermatology: Raynaud’s Syndrome
4. Joints: Arthalgia

Question 15

What are complications of Sjogren’s Syndrome?

Answer 15

1. Lymphoma
2. Neuropathy
3. Purpura
4. Interstitial Lung Disease
5. Renal Tubular Acidosis

Question 16

How does Auto-Immune Myositis present?

Answer 16

Systemic, Diffuse, Proximal Muscle Weakness:
1. Polymyositis
2. Dermatomyositis:
2. a) Gottron’s Papules (80%)
2. b) Heliotrope Rash (30-60%)
Note - The muscle weakness causes difficulty of dialy activities

Question 17

What are the complications of Auto-Immune Myositis?

Answer 17

1. Cancer

2. Interstitial Lung Disease

Question 18

What are some presentations of Mixed Connective Tissue Diseases?

Answer 18

1. Soft Tissue Swelling
2. Raynaud’s Disease
3. Myositis
4. Arthalgia