Flashcards in Multi-System Auto-Immune Disease Deck (59)
How can sjogren's syndrome affect the PNS?
How does sjogren's syndrome present?
-Dry eyes and mouth
-Parotid gland enlargement
-1/3 have systemic upset including fatigue, fever, myalgia and arthralgia
What are the possible complications of sjogren's syndrome?
-Interstitial lung disease
-Renal tubular acidosis
What is the epidemiology of auto-immune myositis?
-6/ million incidence
What is auto-immune myositis characterised by?
-Symmetrical, diffuse, proximal muscle weakness
What are the 2 forms of auto-immune myositis?
How do the populations affected by polymyositis and dermatomyositis differ?
-Polymyositis usually affects younger people
-Dermatomyositis usually affects older people
Name 2 skin signs associated with auto-immune myositis.
-Gottron's papules (80%)
-Heliotrope rash (30-60%)
What are the possible complications of auto-immune myositis?
-Interstitial lung disease
What syndromes does auto-immune myositis overlap with?
Mixed Connective Tissue Disorders
-Soft tissue swelling
Give examples of large vessel vasculitis.
-Giant cell arteritis
Give examples of medium vessel vasculitis.
Give examples of ANCA-associated small vessel vasculitis?
-Granulomatosis with polyangitis
-Eosionophilic granulomatosis with polyangitis
Give examples of immune complex small vessel vasculitis
-Anti GBM disease
-IgA vasculitis (Henoch-Schonlein)
-Hypocomplementemic urticarial vasculitis (Anti C1q vasculitis)
What is the criteria for giant cell arteritis?
3 of the following
-Age at onset ≥50 years
-Temporal artery tenderness/reduced pulsation
-Abnormal temporal biopsy
Give examples of ANCA associated vasculitis.
-Granulomatosis with polyangiitis (Wegener’s)
-Eosiniphilic granulomatosis with polyangiitis
What is the epidemiology of ANCA associated vasculitis?
-Overall incidence 15/million
-Overall Prevalence 150/million
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
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
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
What conditions is a positive ANA unhelpful?
What is the specific ANA profile associated with SLE?
What is the specific ANA profile associated with scleroderma?
What is the specific ANA profile associated with polymyositis?
What is the specific ANA profile associated with Sjorgren's disease?
What are the different classes of lupus nephritis on biopsy?
-I: minimal mesangial
-II: mesangial proliferative
-VI: advanced sclerosing
What is the treatment for mild multi-system autoimmune disease?
What is the treatment for moderate multi-system autoimmune disease?