Multisystem Autoimmune Disease Flashcards
List the connective tissue autoimmune diseases
- Systemic lupus erythematosus
- Scleroderma
- Sjogren’s syndrome
- Auto-immune myositis
- Mixed connective tissue disease
List the systemic vasculitis diseases
- Giant cell arteritis
- Granulomatosis polyangiitis (Wegeners)
- Microscopic polyangiitis
- Eosinophilic granulomatosis polyangiitis
Name the possible mimics
- Drugs: cocaine, minocycline and PTU
- Infection: HIV, endocarditis, hepatitis and TB
- Cardiac myxoma
- Cholesterol emboli
- Scurvey
Which patient groups are most likely to have SLE?
- Female > male
- Aged 15-50yrs
- Afro-caribbean>asian>caucasian
List the classification criteria for SLE (need 4 for diagnosis)
- Malar rash (butterfly)
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis (at least 2 joints)
- Serositis (pleurisy or pericarditis)
- Renal: significant proteinuria or cellular casts in urine
- Neuro: unexplained seizures or psychosis
- Haematological: low WCC, platelets, lymphocytes and haemolytic anaemia
- Immunological: anti ds-DNA, SM, cardiolipin, lupus anticoagulant and low complement
- ANA (antinuclear antibody)
Which patient groups are most likely to be diagnosed with scleroderma?
- Females > males
- Age: 30-50yrs
What are the features of limited systemic scleroderma?
- Affects skin on the hands, lower arms, feet, lower legs and face
- Can affect the lungs and GI tract
- Often starts as Raynaud’s
- Symptoms: thickening of the skin over the hands, feet and face, red spots on the skin, hard lumps under the skin, heartburn and dysphagia
What are the features of diffuses systemic scleroderma?
- More likely to affect internal organs (can cause SOB, raised BP and pulmonary hypertension)
- Skin changes can affect the whole body
- Other symptoms: weight loss, fatigue, joint pain and stiffness
What are the possible complications of systemic scleroderma?
- Limited: pulmonary hypertension
- Diffuse: pulmonary fibrosis, renal crisis and small bowel bacterial overgrowth
Which population groups are more likely to get Sjogren’s syndrome?
- Females > males
- Age: 40-50yrs
What are the clinical features of Sjogren’s syndrome?
- Dry eyes and mouth
- Parotid gland enlargement
- 1/3 have systemic upset (fatigue, fever, myalgia and arthralgia)
What are the complications of Sjogren’s syndrome?
- Lymphoma
- Neuropathy
- Purpura
- Interstitial lung disease
- Renal tubular acidosis
What are the clinical features of auto-immune myositis?
- Muscle weakness: symmetrical, diffuse and proximal
- Polymyositis
- Dermatomyositis
- Gottron’s papsules
- Heliotrope rash
What are the complications of auto-immune myositis?
- Cancer
- Interstitial lung disease
Which syndromes are mixed connective tissue diseases?
- Soft tissue swelling
- Raynauds
- Myositis
- Arthalgia
List the classification criteria for giant cell arthritis (need 3)
- Age > 50 at onset
- New headache
- Temporal artery tenderness/reduced pulsation
- ESR > 50
- Abnormal temporal biopsy
What are the types of ANCA associated vasculitis?
- Granulomatosis with polyangiitis (Wegener’s)
- Microscopic polyangiitis
- Eosiniphilic granulomatosis with polyangiitis
What are the features of granulomatosis with polyangiitis?
- Necrotising granulomatous inflammation
- Usually involves upper and lowe respiratory tract
- Affects predominantly small to medium vessels
- Necrotising glomerulonephritis
What are the features of microscopic polyangiitis?
- Necrotising vasculitis (small vessels)
- Necrotising arteritis (small and medium arteries)
- Necrotising glomerulonephritis
- Pulmonary capillaritis
- Granulomatous inflammation is absent
What are the features of eosinophilic granulomatosis with polyangiitis?
- Eosinophil rich and necrotising granulomatous inflamm. often involving the respiratory tract
- Necrotising vasculitis (small to medium vessels)
- Associated with asthma and eosinophilia
- ANCA is more frequent with glomerulonephritis is present
In which conditions is a positive ANA not helpful?
- RA
- MS
- Infection
List the classification system for lupus nephritis
I: minimal mesangial II: mesangial proliferative III: focal IV: diffuse V: membranous VI: advanced sclerosing
What are the treatment options for multi-system autoimmune diseases?
- Mild: Hydroxychloroquine
- Moderate: azathioprine, methotrexate and mycophenolate
- Severe: cyclophosphamide and rituximab
