Multi-System Autoimmune Diseases Flashcards
What are examples of connective tissue diseases?
- Systemic lupus erythematous
- Scleroderma
- Sjogren’s syndrome
- Auto-immune myositis
- Mixed connective tissue disease
What are examples of systremic vasculitis?
- Giant cell arteritis
- Granulomatosis polyangiitis (Wegeners)
- Microscopic polyangiitis
- Eosinophilic granulomatosis polyangiitis (Churg-Stauss)
What is the differential diagnosis for multi-system autoimmune diseases?
- Drugs - cocaine, minocyline, PTU
- Infection - HIV, endocarditis, Hepatitis, TB
- Malignancy - lymphoma
- Cardiac myxoma
- Cholesterol emboli
- Scurvey
What does SLE stand for?
Systemic lupus erythematous
Describe the epidemiology of SLE in terms of sex, age and ethnicity?
- F:M 9:1
- Onset 15-50 years (known as disease of the young)
- Significant ethnic diversity
- Afro-Caribbean’s > Asian > Caucasian
What organs can be affected by SLE?
What is the classification critera for SLE?
Classification criteria (any 4):
- 1) Malar rash (butterfly rash)
- 2) Discoid rash (raised, scarring, permanent marks, alopecia)
- 3) Photosensitivity
- 4) Oral ulcers
- 5) Arthritis (2 joints at least)
- 6) Serositis (pleurisy or pericarditis)
- 7) Renal (significant proteinuria or cellular casts in urine)
- 8) Neurological (unexplained seizures or psychosis)
- 9) Haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
- 10) Immunological (anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement)
- 11) ANA
What is scleroderma?
Uncommon condition that results in hard, thickened areas of skin and sometime problems with internal organs and blood vessels
Describe the epidemiology of scleroderma in terms of sex and age?
- F:M 3:1
- Onset 30-50 years
What are the different kinds of scleroderma?
- Morphea
- Isolated patches of hardened skin with no internal organ involvement
- Limited
- Skin changes typically occur in lower arms and legs
- Diffuse
- More skin involved than limited, such as skin in the trunk is likely to be involved
What are complications of scleroderma?
- Limited
- Pulmonary hypertension
- Diffuse
- Pulmonary fibrosis
- Renal crises
- Small bowel bacterial overgrowth
What does Sjogen’s syndrome affect?
Glands
Describe the epidemiology of Sjogren’s syndrome in terms of sex and age?
- F:M 9:1
- Onset 40-50 years
What are the clinical features of Sjogren’s syndrome?
- Two most common symptoms are dry eyes and mouth
- Parotid gland enlargement
- Systemic symptoms
- Fatigue
- Fever
- Myalgia
- Arthalgia
What are potential complications of Sjogen’s syndrome?
- Lymphoma
- Neuropathy
- Purpura
- Interstitial lung disease
- Renal tubular acidosis
What is acute-immune myositis?
Disease involving chronic inflammation that leads to weakening of muscles over time
What are clinical features of auto-immune myositis?
- Muscle weakness
- Symmetrical, diffuse, proximal
- Polymyositis or dermatomyositis
- Gottron’s papules
- Heliotrope rash
What are potential complications of autoimmune myositis?
- Cancer
- Interstitial lung disease
What is overlap syndrome also known as?
Known as mixed connective tissue disease (MCTD)
What does MCTD stand for?
Mixed connective tissue disease
What is mixed connective tissue disease characterised by?
Characterised by features seen from:
- SLE
- Scleroderma
- Polymyositis
What are the clinical features of mixed connective tissue disease?
- Soft tissue swelling
- Raynauds
- Myositis
- Arthalgia
What are the different categories of vasculitides?
- Large vessel vasculitis
- Medium vessel vasculitis
- Small vessel vasculitis
What does GCA stand for?
Giant cell arteritis
What is giant cell arteritis?
Condition where temporal arteries become inflamed
What is the classification 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
What kind of vasculitis is gian cell arteritis?
Large vessel vasculitis
What does ANCA stand for?
Antineutrophil cytoplasmic antibodies
What are antineutrophil cytoplasmic antibodies?
Autoantibodies directed against antigens found in the cytoplasmic granules of neutrophils and monocytes
ANCA testing is helped to diagnose what conditions?
- Granulomatosis with polyangiitis (Wegener’s)
- Microscopic polyangiitis
- Eosiniphilic granulomatosis with polyangiitis
What is granulomatosis with polyangiitis also called?
Wegener’s disease
What is Wegener’s disease?
Necrotising granulomatous inflammation
What are clinical features of Wegener’s disease?
- Usually involving upper and lower respiratory tract
- Affecting predominantly small to medium vessels
- Necrotising glomerulonephritis is common
What does MPA stand for?
Microscopic polyangiitis
What is microscopic polyangiitis?
Disorder causing vasculitis (blood vessel inflammation) leading to organ damage
What are clinical features of microscopic polyangiitis?
- Necrotising vasculitis with few or no immune deposits, predominantly affecting small vessels
- Necrotising arteritis involving small and medium vessels may be present
- Necrotising glomerulonephritis very common
- Pulmonary capillaritis
- Granulomatous inflammation is absent
What is eosinophillic granulomatosis with polyangiitis also called?
Churg strauss
What are the clinical features of Eosinophillic Granulomatosis with Polyangiitis (Churg Strauss)
- 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 does ANA test detect?
ANA test detects antinuclear antibodies (ANA) in blood, immune system makes these to fight infection or in autoimmune disease
What are some conditions where ANA test is useful?
What is the treatment for multi-system autoimmune diseases?
Treatment depends on organ threat
