Multi-system Autoimmune Diseases Flashcards

1
Q

Name some connective tissue diseases

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

Name some systemic vasculitides

A
  • Giant cell arteritis
  • Granulomatosis polyangiitis (Wegeners)
  • Microscopic polyangiitis
  • Eosinophilic granulomatosis polyangiitis (Churg-Strauss)
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3
Q

Describe the sequence of events in autoimmunity

A
  • Initiating event + genetic susceptibility
  • Breakdown of self-tolerance
  • Autoreactivity
  • Humoral +/- cellular
  • Autoimmune disease
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4
Q

How do you generally diagnose autoimmune diseases?

A

Cardinal clinical features through history and exam

Immunology
Imaging
Tissue
Exclusion of differential diagnosis

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5
Q

What can mimic autoimmune disease?

A

Drugs - cocaine, minocyline, PTU

Infection - HIV, endocarditis, Hepatitis, TB

Malignancy - Lymphoma

Cardiac myxoma
Cholesterol emboli
Scurvey

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6
Q

What is the epidemiology of systemic lupus erythematosis?

prevalence, incidence, M:F, age of onset, ethnicity

A

UK prevalence 28/100,000
UK incidence 4/100,000
M:F = 1:9
Onset = 15-50 years

Significant ethnic diversity
-Afro-caribbeans > asian > caucasian

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7
Q

What can SLE cause?

A

Face:

  • butterfly rash
  • photosensitivity

General:

  • fever
  • depression

Skin:

  • vasculitis
  • purpura
  • urticaria

Chest:

  • pleurisy
  • pleural effusion
  • fibrosis

Raynaud’s phenomenon

Alopecia

Joints:

  • aseptic necrosis of hip
  • arthritis small joints

Nervous system:

  • fits
  • hemiplegia
  • ataxia
  • peripheral neuropathy
  • cranial nerve lesions

Heart

  • pericarditis
  • endocarditis
  • aortic valve lesions

Abdominal pain

Myopathy

Blood

  • anaemia
  • leucopenia
  • thrombocytopenia

CAN CAUSE ANYTHING

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8
Q

What is the differential for a butterfly rash?

A

Rosacae
Mitral stenosis
SLE

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9
Q

What is the key difference between the arthritis in SLE and RA?

A

SLE -> fingers are bent but joints look normal on x-ray

RA -> joints are abnormal on x-ray

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10
Q

Why is urine dipstick so important in SLE?

A

Kidney problems are the things that will kill you

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11
Q

What is the epidemiology of scleroderma?

prevalence, incidence, age of onset, M:F

A

UK prevalence: 24/100,000
UK incidence: 10/100,000
Onset: 30-50 years
M:F = 1:3

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12
Q

What is morphea?

A

Morphea, also known as localized scleroderma, is a disorder characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissues, or both.

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13
Q

What are the 2 types of scleroderma?

A

Limited

Diffuse

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14
Q

What are the complications of the two types of scleroderma?

A

Limited:
-Pulmonary hypertension

Diffuse:

  • Pulmonary fibrosis
  • Renal crisis
  • Small bowel bacterial overgrowth -> malabsorption
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15
Q

What is the epidemiology of sjogren’s syndrome?

prevalence, incidence, age of onset, M:F

A

Prevalence: 1 in 100
Incidence: 4 in 100,000
Onset: 40-50 years
M:F = 1:9

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16
Q

What are sicca symptoms?

A

Dryness of eyes and mouth

17
Q

What does Sjogren’s syndrome effect?

A

Gastrointestinal

  • dysphagia
  • abnormal oesophageal mobility

Respiratory
-intestinal lung disease

Peripheral nervous system

  • sensory neuropathy
  • mononeuritis multiplex

CNS

  • fits
  • hemiplegia
  • ataxia
  • cranial nerve lesions

Kidney
-renal tubular acidosis

Skin

  • palpable purpura
  • Rayneud’s syndrome

joints:
-arthralgia

18
Q

What are the signs and symptoms of Sjogren’s syndrome?

A

Dry eyes and mouth

Parotif gland enlargement

1/3 have systemic upset

  • fatigue
  • fever
  • myalgia
  • arthralgia
19
Q

What are the complications of Sjogren’s syndrome?

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

What are the signs and symptoms of auto-immune myositis?

A

Muscle wakness
-Symmetrical, diffuse, proximal

Polymyositis (younger)

Dermatosyositis (older)

  • Gottron’s papules (80%)
  • Heliotrope rash (30-60%)
21
Q

What are the complications of auto-immune myositis?

A

Polymyositis -> interstitial lung disease

Dermatosyositis -> cancer

22
Q

What are the features of Mixed Connective Tissue Disease?

A

Soft tissue swelling
Raynaud’s
Myositis
Arthralgia

23
Q

What vasculitides effect large vessels?

A

Takayasu Arteritis

Giant cell arteritis

24
Q

What vasculitides effect medium vessels?

A

Polyarteritis nodosa

Kawasaki disease

25
Q

Name 3 ANCA- associated vasculitides effecting small vessels

A

Microscopic Polyangiitis

Granulomatosis with polyangiitis

Eosinophilic Granulomatosis with Polyangiitis

26
Q

Name some immune complex small vessel vasculitides

A

Anti-GBM disease

Cryoglobulinemic Vasculitis

IgA Vasculitis (Henoch-Schonlein)

Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis)

27
Q

What is the classification criteria for giant cell arteritis?

A

3 of the following:

  • Age at onset 50 or over
  • New headache
  • Temporal artery tenderness/ reduced pulsation
  • ESR 50 or above
  • Abnormal temporal biopsy
28
Q

What is Granulomatosis with Polyangiitis (Wegener’s)?

what is it, what does it effect?

A

Necrotising granulomatous inflammation

Usually involving the upper and lower respiratory tract

Affecting predominantly small to medium vessels

Necrotising glomerulonephritis is common

29
Q

What is microscopic polyangiitis?

what is it, what does it effect, what sets it apart?

A

Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels.

Necrotising arteritis involving small and medium arteries may be present

Necrotising glomerulonephritis is very common

Pulmonary capillaritis often occurs

Granulomatous inflammation is absent

30
Q

What is eosinophilic granulomatosis with polyangiitis (Churg Strauss)?

(what is it, what does it effect, what is it associated with)

A

Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract

Necrotising vasculitis predominantly affecting small to medium vessels

Associated with asthma and eosinophilia

ANCA is more frequent when glomerulonephritis is present

31
Q

What is the treatment of multi-system autoimmune diseases?

A

Mild:
-Hydroxychloroquine

Moderate:

  • Azathioprine
  • Methotrexate
  • Mycophenolate

Severe:

  • Cyclophosphamide
  • Rituximab

(often combination- particularly in giant cell arteritis)