Systemic autoimmune diseases

Question 1

What multisystem diseases affect connective tissue?

Answer 1

Systemic Lupus Erythematosus
Scleroderma
Sjogren's syndrome
Auto-immune myositis
Mixed connective tissue disease

Question 2

What are the 3 main categories of systemic vasculitis?

Answer 2

Large vessel disease
Medium vessel disease
Small vessel disease

Question 3

What is used to diagnose multisystem autoimmune conditions?

Answer 3

History/exam
Immunology
Imaging
Tissue biopsy
Exclusion of differential diagnosis

Question 4

What can mimic systemic autoimmune disease?

Answer 4

Drugs - cocaine
Infection - HIV, endocarditis, hepatitis
Malignancy - lymphoma

Question 5

What is the male:female ratio for systemic lupus erythematosus?

Answer 5

1:9 - much more common in females

Question 6

What is the age of onset of systemic lupus erythematosus?

Answer 6

15-50 years

Question 7

How many of the lupus classification criteria is needed to make a diagnosis?

Answer 7

4

Question 8

What are the 11 classification criteria for systemic lupus eryhtematosus?

Answer 8

Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis of 2+ joints
Serositis
Significant proteinuria - renal
Unexplained seizures - neurological
Low WCC, platelets, haemolytic anaemia - haematological
Immunological
Antinuclear antibodies

Question 9

What is a discoid rash?

Answer 9

Raised, scarring, permanent marks

Question 10

What is scleroderma?

Answer 10

Collection of autoimmune diseases affecting the skin, blood vessels, and internal organs

Question 11

What is the male:female ratio of scleroderma?

Answer 11

1:3

Question 12

What is the age of onset of scleroderma?

Answer 12

30-50 years

Question 13

What is sclerodermal morphea?

Answer 13

Thickening and hardening of skin and subcutaneous tissue due to collagen deposition

Question 14

What is the main complication of limited scleroderma?

Answer 14

Pulmonary hypertension

Question 15

What are complications of diffuse scleroderma?

Answer 15

Pulmonary fibrosis
Renal crisis
Small bowel bacteria overgrowth

Question 16

What is Sjogren’s syndrome?

Answer 16

Inflammation of moisture producing glands

Question 17

Is Sjogren’s more common in men or women?

Answer 17

Women

Question 18

What are features of Sjogren’s syndrome?

Answer 18

Dry eyes and mouth
Parotid gland enlargement
1/3 have systemic upset - fatigue, fever, myalgia, arthalgia

Question 19

What are complications of Sjogren’s syndrome?

Answer 19

Lymphoma
Neuropathy
Purpura
Interstitial lung disease
Renal tubular acidosis

Question 20

What is the main symptom of auto-immune myositis?

Answer 20

Muscle weakness

Question 21

What are complications of auto-immune myositis?

Answer 21

Cancer

Interstitial lung disease

Question 22

What are forms of large cell vasculitis?

Answer 22

Takayasu arteritis

Giant cell arteritis

Question 23

What are forms of medium vessel vasculitis?

Answer 23

Polyarteritis nodosa

Kawasaki disease

Question 24

What are forms of ANCA associated small vessel vasculitis?

Answer 24

Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophillic granulomatosis with polyangiitis

Question 25

How many of the giant cell arteritis criteria must be met to make a diagnosis?

Answer 25

3

Question 26

What are the giant cell arteritis criteria?

Answer 26

``` Age at onset>50 New headache Temporal artery tenderness/reduced pulsation ESR<50 Abnormal temporal biopsy ```

Question 27

What does ANCA stand for?

Answer 27

Anti-neutrophil cytoplasmic antibody

Question 28

What are features of granulomatosis with polyangiitis?

Answer 28

Necrotising granulomatous inflammation Usually involves upper and lower resp tract Affecting predominantly small vessels Necrotising glomerulonephritis is common

Question 29

What are features of microscopic polyangiitis?

Answer 29

Necrotising vasculitis with few immune deposits affecting small vessels Necrotising glomerulonephritis is common Pulmonary capillaritis is common Granulomatous inflammation is absent

Question 30

What are features of eosinophilic granulomatosis with polyangiitis?

Answer 30

Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract Necrotising vasculitis affecting small vessels Associated with asthma and eosinophilia ANCA more frequent with glomerulonephritis

Question 31

How is mild multi-system autoimmune disease treated?

Answer 31

Hydroxylchloroquine

Question 32

How is moderate multi-system autoimmune disease treated?

Answer 32

Azathioprine, methotrexate, mycophenolate

Question 33

How is severe multi-system autoimmune disease treated?

Answer 33

Cyclophosphamide, rituximab