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MSK > Systemic autoimmune diseases > Flashcards

Systemic autoimmune diseases Flashcards

1
Q

What multisystem diseases affect connective tissue?

A 
Systemic Lupus Erythematosus
Scleroderma
Sjogren's syndrome
Auto-immune myositis
Mixed connective tissue disease
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2
Q

What are the 3 main categories of systemic vasculitis?

A

Large vessel disease
Medium vessel disease
Small vessel disease

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3
Q

What is used to diagnose multisystem autoimmune conditions?

A 
History/exam
Immunology
Imaging
Tissue biopsy
Exclusion of differential diagnosis
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4
Q

What can mimic systemic autoimmune disease?

A

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

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5
Q

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

A

1:9 - much more common in females

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6
Q

What is the age of onset of systemic lupus erythematosus?

A

15-50 years

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7
Q

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

A

4

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8
Q

What are the 11 classification criteria for systemic lupus eryhtematosus?

A 
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
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9
Q

What is a discoid rash?

A

Raised, scarring, permanent marks

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10
Q

What is scleroderma?

A

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

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11
Q

What is the male:female ratio of scleroderma?

A

1:3

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12
Q

What is the age of onset of scleroderma?

A

30-50 years

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13
Q

What is sclerodermal morphea?

A

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

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14
Q

What is the main complication of limited scleroderma?

A

Pulmonary hypertension

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15
Q

What are complications of diffuse scleroderma?

A

Pulmonary fibrosis
Renal crisis
Small bowel bacteria overgrowth

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16
Q

What is Sjogren’s syndrome?

A

Inflammation of moisture producing glands

17
Q

Is Sjogren’s more common in men or women?

A

Women

18
Q

What are features of Sjogren’s syndrome?

A

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

19
Q

What are complications of Sjogren’s syndrome?

A 
Lymphoma
Neuropathy
Purpura
Interstitial lung disease
Renal tubular acidosis
20
Q

What is the main symptom of auto-immune myositis?

A

Muscle weakness

21
Q

What are complications of auto-immune myositis?

A

Cancer

Interstitial lung disease

22
Q

What are forms of large cell vasculitis?

A

Takayasu arteritis

Giant cell arteritis

23
Q

What are forms of medium vessel vasculitis?

A

Polyarteritis nodosa

Kawasaki disease

24
Q

What are forms of ANCA associated small vessel vasculitis?

A

Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophillic granulomatosis with polyangiitis

25
Q

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

A

3

26
Q

What are the giant cell arteritis criteria?

A 
Age at onset>50
New headache
Temporal artery tenderness/reduced pulsation
ESR<50
Abnormal temporal biopsy
27
Q

What does ANCA stand for?

A

Anti-neutrophil cytoplasmic antibody

28
Q

What are features of granulomatosis with polyangiitis?

A

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

29
Q

What are features of microscopic polyangiitis?

A

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

30
Q

What are features of eosinophilic granulomatosis with polyangiitis?

A

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

31
Q

How is mild multi-system autoimmune disease treated?

A

Hydroxylchloroquine

32
Q

How is moderate multi-system autoimmune disease treated?

A

Azathioprine, methotrexate, mycophenolate

33
Q

How is severe multi-system autoimmune disease treated?

A

Cyclophosphamide, rituximab

34
Q

What are the CREST features of scleroderma?

A 
Calcinosis
Raynauds 
oEsophageal dismotility
Sclerodactyly
Telangiectasia

MSK (30 decks)

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