Multi-System Auto-Immune Disease Flashcards Preview

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Flashcards in Multi-System Auto-Immune Disease Deck (59)
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31

How can sjogren's syndrome affect the PNS?

-Sensory neuropathy
-Mononeuritis multiplex

32

How does sjogren's syndrome present?

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

33

What are the possible complications of sjogren's syndrome?

-Lymphoma
-Neuropathy
-Purpura
-Interstitial lung disease
-Renal tubular acidosis

34

What is the epidemiology of auto-immune myositis?

-Rare
-6/ million incidence

35

What is auto-immune myositis characterised by?

-Symmetrical, diffuse, proximal muscle weakness

36

What are the 2 forms of auto-immune myositis?

-Polymyositis
-Dermatomyositis

37

How do the populations affected by polymyositis and dermatomyositis differ?

-Polymyositis usually affects younger people
-Dermatomyositis usually affects older people

38

Name 2 skin signs associated with auto-immune myositis.

-Gottron's papules (80%)
-Heliotrope rash (30-60%)

39

What are the possible complications of auto-immune myositis?

-Cancer
-Interstitial lung disease

40

What syndromes does auto-immune myositis overlap with?

Mixed Connective Tissue Disorders
-Soft tissue swelling
-Raynauds
-Myositis
-Arthalgia

41

Give examples of large vessel vasculitis.

-Takayasu arteritis
-Giant cell arteritis

42

Give examples of medium vessel vasculitis.

-Polyarteritis nodosa
-Kawasaki disease

43

Give examples of ANCA-associated small vessel vasculitis?

-Microscopic polyangitis
-Granulomatosis with polyangitis
-Eosionophilic granulomatosis with polyangitis

44

Give examples of immune complex small vessel vasculitis

-Anti GBM disease
-Cryoglobulinemic vasculitis
-IgA vasculitis (Henoch-Schonlein)
-Hypocomplementemic urticarial vasculitis (Anti C1q vasculitis)

45

What is the criteria for giant cell arteritis?

3 of the following
-Age at onset ≥50 years
-New headache
-Temporal artery tenderness/reduced pulsation
-ESR≥50
-Abnormal temporal biopsy

46

Give examples of ANCA associated vasculitis.

-Granulomatosis with polyangiitis (Wegener’s)
-Microscopic polyangiitis
-Eosiniphilic granulomatosis with polyangiitis

47

What is the epidemiology of ANCA associated vasculitis?

-Overall incidence 15/million
-Overall Prevalence 150/million

48

What is the presentation of Wegner's granulomatosis?

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

49

What is the presentation of microscopic polyangiitis?

-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

What is the presentation of eosinophilic granulomatosis with polyangiitis

-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

What conditions is a positive ANA unhelpful?

-RA
-MS
-Infection

52

What is the specific ANA profile associated with SLE?

-dsDNA
-Ro
-Sm

53

What is the specific ANA profile associated with scleroderma?

-Scl-70
-Centromere

54

What is the specific ANA profile associated with polymyositis?

Jo-1

55

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

-Ro
-La

56

What are the different classes of lupus nephritis on biopsy?

-I: minimal mesangial
-II: mesangial proliferative
-III: focal
-IV: diffuse
-V: membranous
-VI: advanced sclerosing

57

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

Hydroxychloroquine

58

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

-Azathioprine
-Methotrexate
-Mycophenolate

59

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

-Cyclophosphamide
-Rituximab