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Systems: MSK AB > Multi-System Auto-Immune Disease > Flashcards

Multi-System Auto-Immune Disease Flashcards

1
Q

What is the diagnosis for someone presenting with:

  • Mylagia/arthralgia
  • Episcleritis
  • Sinusitis
  • Renal failure
A

Wegner’s granulomatosis

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2
Q

What is the diagnosis for someone presenting with:

  • Mylagia/arthralgia
  • Skin rash
  • Pleurisy
  • CVA
A

Lupus

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3
Q

What is the diagnosis for someone presenting with:

  • Mylagia/arthralgia
  • Skin thickening
  • GORD
  • Pulmonary hypertension
A

Scleroderma

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4
Q

What is the diagnosis for someone presenting with:

  • Mylagia/arthralgia
  • Sicca
  • Skin rash
  • Neuropathy
A

Sjorgen’s syndrome

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5
Q

Give examples of autoimmune connective tissue diseases.

A
  • Systemic Lupus Erythematosus
  • Scleroderma
  • Sjogren’s syndrome
  • Auto-immune myositis
  • Mixed connective tissue disease
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6
Q

Give examples of autoimmune systemic vasculitis?

A
  • Giant cell arteritis
  • Granulomatosis polyangiitis (Wegeners)
  • Microscopic polyangiitis
  • Eosinophilic granulomatosis polyangiitis (Churg-Strauss)
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7
Q

How are multisystem autoimmune diseases diagnosed?

A
  • Cardinal clinical features: History & Exam
  • Immunology
  • Imaging
  • Tissue
  • Exclusion of differential diagnosis
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8
Q

What can mimic multisystem autoimmune diseases?

A
  • Drugs such as cocaine, minocycline, PTU
  • Infections including HIV, endocarditis, Hepatitis and TB
  • Malignancy, in particular lymphoma
  • Cardiac myxoma
  • Cholesterol emboli
  • Scurvey
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9
Q

What is the epidemiology of SLE?

A
  • UK Prevalence: 28/100,000
  • UK incidence: 4/100,000
  • F:M 9:1
  • Onset: 15-50 years
  • Significant ethnic diversity: Afro-Caribbean> Asian> Caucasian
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10
Q

How can SLE affect the skin and hair?

A
  • Butterfly rash
  • Photosensitivity
  • Alopecia
  • Vasculitis
  • Purpura
  • Urticaria
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11
Q

How can SLE affect the nervous system?

A
  • Fits
  • Hemiplegia
  • Ataxia
  • Peripheral neuropathy
  • Cranial nerve lesions
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12
Q

How can SLE affect the heart and chest?

A
  • Pericarditis
  • Endocarditis
  • Aortic valve lesions
  • Pleurisy
  • Pleural effusion
  • Lung fibrosis
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13
Q

How can SLE affect the MSK system?

A
  • Myopathy
  • Aseptic necrosis of the hip
  • Arthritis in small joint
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14
Q

How can SLE affect the blood?

A
  • Anaemia (nomochromic normocytic Coombs test positive)
  • Leukopenia
  • Thrombocytopenia
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15
Q

How can SLE affect a person in general?

A
  • Fever
  • Depression
  • Raynaud’s phenomenon
  • Abdominal pain
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16
Q

What is the classification criteria for SLE?

A

Any 4

  • Malar rash (butterfly rash)
  • Discoid rash (raised, scarring, permanent marks, alopecia)
  • Photosensitivity
  • Oral ulcers
  • Arthritis (2 joints at least)
  • Serositis (pleurisy or pericarditis)
  • Renal (significant proteinuria or cellular casts in urine)
  • Neurological (unexplained seizures or psychosis)
  • Haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
  • Immunological (anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement)
  • ANA
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17
Q

What is the earliest sign of kidney involvement in SLE?

A

Blood and protein on urine dipstick

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18
Q

What is scleroderma characterised by?

A

Thickening of the skin

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19
Q

What is the epidemiology of scleroderma?

A
  • UK Prevalence: 24/100,000
  • UK Incidence: 10/1,000,000
  • Onset: 30-50 years
  • F:M 3:1
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20
Q

What is the name for scleroderma limited?

A

Crest syndrome

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21
Q

What are the features of Crest syndrome?

A
  • Calcinosis
  • Raynaud’s
  • Oesophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
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22
Q

What is a possible complication of limited scleroderma?

A

Pulmonary hypertension

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23
Q

What are the possible outcomes of diffuse scleroderma?

A
  • Pulmonary fibrosis
  • Renal crisis
  • Small bowel bacterial overgrowth
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24
Q

What is the epidemiology of Sjogren’s syndrome?

A
  • Prevalence: 1 in 100
  • Incidence: 4 in 100,000
  • Onset: 40-50yrs
  • F:M 9:1
25
Q

How can sjogren’s syndrome affect the GI system?

A
  • Dysphagia

- Abnormal oesophageal motility

26
Q

How can sjogren’s syndrome affect the respiratory system?

A

Interstitial lung disease

27
Q

How can sjogren’s syndrome affect the CNS?

A
  • Fits
  • Hemiplegia
  • Ataxia
  • Cranial nerve lesions
28
Q

How can sjogren’s syndrome affect the renal system?

A

Renal tubular acidosis

29
Q

How can sjogren’s syndrome affect the skin?

A
  • Palpable purpura

- Raynaud’s syndrome

30
Q

How can sjogren’s syndrome affect the joints?

A

Arthralgia

31
Q

How can sjogren’s syndrome affect the PNS?

A
  • Sensory neuropathy

- Mononeuritis multiplex

32
Q

How does sjogren’s syndrome present?

A
  • Dry eyes and mouth
  • Parotid gland enlargement
  • 1/3 have systemic upset including fatigue, fever, myalgia and arthralgia
33
Q

What are the possible complications of sjogren’s syndrome?

A
  • Lymphoma
  • Neuropathy
  • Purpura
  • Interstitial lung disease
  • Renal tubular acidosis
34
Q

What is the epidemiology of auto-immune myositis?

A
  • Rare

- 6/ million incidence

35
Q

What is auto-immune myositis characterised by?

A

-Symmetrical, diffuse, proximal muscle weakness

36
Q

What are the 2 forms of auto-immune myositis?

A
  • Polymyositis

- Dermatomyositis

37
Q

How do the populations affected by polymyositis and dermatomyositis differ?

A
  • Polymyositis usually affects younger people

- Dermatomyositis usually affects older people

38
Q

Name 2 skin signs associated with auto-immune myositis.

A
  • Gottron’s papules (80%)

- Heliotrope rash (30-60%)

39
Q

What are the possible complications of auto-immune myositis?

A
  • Cancer

- Interstitial lung disease

40
Q

What syndromes does auto-immune myositis overlap with?

A

Mixed Connective Tissue Disorders

  • Soft tissue swelling
  • Raynauds
  • Myositis
  • Arthalgia
41
Q

Give examples of large vessel vasculitis.

A
  • Takayasu arteritis

- Giant cell arteritis

42
Q

Give examples of medium vessel vasculitis.

A
  • Polyarteritis nodosa

- Kawasaki disease

43
Q

Give examples of ANCA-associated small vessel vasculitis?

A
  • Microscopic polyangitis
  • Granulomatosis with polyangitis
  • Eosionophilic granulomatosis with polyangitis
44
Q

Give examples of immune complex small vessel vasculitis

A
  • Anti GBM disease
  • Cryoglobulinemic vasculitis
  • IgA vasculitis (Henoch-Schonlein)
  • Hypocomplementemic urticarial vasculitis (Anti C1q vasculitis)
45
Q

What is the criteria for giant cell arteritis?

A

3 of the following

  • Age at onset ≥50 years
  • New headache
  • Temporal artery tenderness/reduced pulsation
  • ESR≥50
  • Abnormal temporal biopsy
46
Q

Give examples of ANCA associated vasculitis.

A
  • Granulomatosis with polyangiitis (Wegener’s)
  • Microscopic polyangiitis
  • Eosiniphilic granulomatosis with polyangiitis
47
Q

What is the epidemiology of ANCA associated vasculitis?

A
  • Overall incidence 15/million

- Overall Prevalence 150/million

48
Q

What is the presentation of Wegner’s granulomatosis?

A
  • Necrotising granulomatous inflammation
  • Usually involving the upper and lower respiratory tract
  • Affecting predominantly small to medium vessels
  • Necrotising glomerulonephritis is common
49
Q

What is the presentation of microscopic polyangiitis?

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
50
Q

What is the presentation of eosinophilic granulomatosis with polyangiitis

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
51
Q

What conditions is a positive ANA unhelpful?

A
  • RA
  • MS
  • Infection
52
Q

What is the specific ANA profile associated with SLE?

A
  • dsDNA
  • Ro
  • Sm
53
Q

What is the specific ANA profile associated with scleroderma?

A
  • Scl-70

- Centromere

54
Q

What is the specific ANA profile associated with polymyositis?

A

Jo-1

55
Q

What is the specific ANA profile associated with Sjorgren’s disease?

A
  • Ro

- La

56
Q

What are the different classes of lupus nephritis on biopsy?

A
  • I: minimal mesangial
  • II: mesangial proliferative
  • III: focal
  • IV: diffuse
  • V: membranous
  • VI: advanced sclerosing
57
Q

What is the treatment for mild multi-system autoimmune disease?

A

Hydroxychloroquine

58
Q

What is the treatment for moderate multi-system autoimmune disease?

A
  • Azathioprine
  • Methotrexate
  • Mycophenolate
59
Q

What is the treatment for severe multi-system autoimmune disease?

A
  • Cyclophosphamide

- Rituximab

Systems: MSK AB (43 decks)

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