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MSK Systems > 31: Multi-system Autoimmune Conditions > Flashcards

31: Multi-system Autoimmune Conditions Flashcards

1
Q

list examples of connective tissue diseases

A
  • SLE
  • systemic sclerosis
  • Sjogren’s syndrome
  • Auto-immune myositis
  • mixed connective tissue disease
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2
Q

list examples of systemic vasculitidies

A
  • giant cell arteritis
  • granulomatosis with polyangiitis (Wegeners)
  • miscroscopic polyangiitis
  • eosinophilic granulomatosis with polyangiitis (Churg-strauss syndrome)
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3
Q

who is usually affected by SLE? (epidemiology)

A
  • 9x more common in women
  • most common in afro-caribbean and Asian origin
  • disease onset most commonly occurs in 20s-30s
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4
Q

SLE pathogenesis

A
  • immune response against endogenous nuclear antigens
  • immune complex formation
  • complement activation
  • tissue injury
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5
Q

SLE aetiology

A

genetic
hormonal
environmental: UV light, drugs, infections

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6
Q

SLE presentation

A

SLE is a heterogeneous disease and some symptoms can be nonspecific, leading to a delay in diagnosis.

The classical presentation of SLE usually has some combination of:
- Systemic upset (fever, myalgia, fatigue and weight loss)
- Joint and/or skin involvement

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7
Q

what antibodies are associated with SLE?

A
  • Usually positive for ANA, not specific for SLE
  • Anti-ds DNA, seen in 60% of patients, highly specific
  • Anti-Sm (10-30%), highly specific
  • Anti-Ro
  • antiphospholipid antibodies
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8
Q

when should a diagnosis of SLE be considered?

A
  • usually seen in women of childbearing age
  • constitutional symptoms of fever, weight loss, malaise and severe fatigue
  • skin rash and/or stomatitis
  • arthritis
  • pleuritic chest pain
  • renal disease
  • cytopenia
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9
Q

systemic slerosis aetiology

A
  • environmental: silica, solvents, viral infections
  • genetic predisposition
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10
Q

systemic sclerosis pathogenesis

A
  • vascular damage (microcirculation)
  • immune system activation/inflammation
  • fibrosis
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11
Q

what antibodies are implicated in Limited cutaneous systemic sclerosis (CREST)

A

anti-centromere antibodies

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12
Q

which antibodies are indicated in Diffuse cutaneous systemic sclerosis?

A

anti Scl70 antibodies

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13
Q

what is Sjogren syndrome?

A

an inflammatory autoimmune disorder characterised by decreased lacrimal and salivary gland secretion, leading to manifestations of dry eyes and mouth.

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14
Q

which antibodies are implicated in Sjogren’s syndrome?

A
  • anti Ro (SSA)
  • anti La (SSB) antibodies
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15
Q

sjogren’s syndrome presentation

A
  • dry eyes
  • dry mouth
  • parotid gland enlargement
  • 1/3 have systemic upset e.g. fatigue, fever, myalgia, arthralgia, dry skin
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16
Q

Sjorgen’s syndrome complications

A
  • lymphoma
  • neuropathy
  • cutaneous vasculitis
  • interstitial lung disease
  • renal tubular necrosis
17
Q

describe polymyositis and dermatomyositis

A

Polymyositis and dermatomyositis are inflammatory muscle diseases characterized by bilateral, proximal muscle weakness, and in the case of dermatomyositis, skin rashes.

18
Q

what antibodies are indicated in myositis?

A

anti Jo1 antibodies

19
Q

giant cell arteritis classification criteria

A

3 of the following:
- age at onset > than 50 years
- new headache
- temporal artery tenderness/reduced pulsation
- ESR > 50
- abnormal temporal biopsy

20
Q

list some investigations for giant cell arteritis

A
  • temporal artery biopsy
  • US doppler
  • CT angiogram, MR angiogram
  • FDG PET
21
Q

GCA complications

A
  • irreversible visual loss
  • aortic aneurysms
  • arterial stenosis and limb ischaemia
  • stroke
22
Q

GCA treatment

A
  • urgent initiation of high dose prednisolone
  • PPI
  • bone protection
  • steroid sparing medication
23
Q

which antibodies are implicated in Granulomatosis with Polyangiitis (Wegener’s) (GPA)?

A

cANCA
anti PR3 antibodies

24
Q

Granulomatosis with Polyangiitis (Wegener’s) (GPA) pathogenesis

A

Necrotising granulomatous inflammation
Usually involving the upper and lower respiratory tract
Hearing loss, sinusitis, hemoptysis
Necrotising glomerulonephritis is common

25
Q

Microscopic polyangiitis (MPA) pathogenesis

A
  • Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels
  • Granulomatous inflammation is absent
  • Renal and pulmonary involvement common
26
Q

which antibodies are implicated in microscopic polyangitis?

A

pANCA
anti MPO antibodies

27
Q

Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA) pathogenesis

A
  • Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
  • Late onset asthma, nasal polyps and eosinophilia
  • Necrotising vasculitis predominantly affecting small to medium vessels
  • Neurological involvement
  • Cardiac, gastrointestinal – poor prognosis
28
Q

what antibodies are implicated in Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA)?

A

40-60% anti MPO antibodies positive

29
Q

what is the likely diagnosis?

A

SLE

MSK Systems (38 decks)

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