Multi-System Auto-Immune Disease Flashcards
What is the diagnosis for someone presenting with:
- Mylagia/arthralgia
- Episcleritis
- Sinusitis
- Renal failure
Wegner’s granulomatosis
What is the diagnosis for someone presenting with:
- Mylagia/arthralgia
- Skin rash
- Pleurisy
- CVA
Lupus
What is the diagnosis for someone presenting with:
- Mylagia/arthralgia
- Skin thickening
- GORD
- Pulmonary hypertension
Scleroderma
What is the diagnosis for someone presenting with:
- Mylagia/arthralgia
- Sicca
- Skin rash
- Neuropathy
Sjorgen’s syndrome
Give examples of autoimmune connective tissue diseases.
- Systemic Lupus Erythematosus
- Scleroderma
- Sjogren’s syndrome
- Auto-immune myositis
- Mixed connective tissue disease
Give examples of autoimmune systemic vasculitis?
- Giant cell arteritis
- Granulomatosis polyangiitis (Wegeners)
- Microscopic polyangiitis
- Eosinophilic granulomatosis polyangiitis (Churg-Strauss)
How are multisystem autoimmune diseases diagnosed?
- Cardinal clinical features: History & Exam
- Immunology
- Imaging
- Tissue
- Exclusion of differential diagnosis
What can mimic multisystem autoimmune diseases?
- Drugs such as cocaine, minocycline, PTU
- Infections including HIV, endocarditis, Hepatitis and TB
- Malignancy, in particular lymphoma
- Cardiac myxoma
- Cholesterol emboli
- Scurvey
What is the epidemiology of SLE?
- UK Prevalence: 28/100,000
- UK incidence: 4/100,000
- F:M 9:1
- Onset: 15-50 years
- Significant ethnic diversity: Afro-Caribbean> Asian> Caucasian
How can SLE affect the skin and hair?
- Butterfly rash
- Photosensitivity
- Alopecia
- Vasculitis
- Purpura
- Urticaria
How can SLE affect the nervous system?
- Fits
- Hemiplegia
- Ataxia
- Peripheral neuropathy
- Cranial nerve lesions
How can SLE affect the heart and chest?
- Pericarditis
- Endocarditis
- Aortic valve lesions
- Pleurisy
- Pleural effusion
- Lung fibrosis
How can SLE affect the MSK system?
- Myopathy
- Aseptic necrosis of the hip
- Arthritis in small joint
How can SLE affect the blood?
- Anaemia (nomochromic normocytic Coombs test positive)
- Leukopenia
- Thrombocytopenia
How can SLE affect a person in general?
- Fever
- Depression
- Raynaud’s phenomenon
- Abdominal pain
What is the classification criteria for SLE?
Any 4
- Malar rash (butterfly rash)
- Discoid rash (raised, scarring, permanent marks, alopecia)
- Photosensitivity
- Oral ulcers
- Arthritis (2 joints at least)
- Serositis (pleurisy or pericarditis)
- Renal (significant proteinuria or cellular casts in urine)
- Neurological (unexplained seizures or psychosis)
- Haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
- Immunological (anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement)
- ANA
What is the earliest sign of kidney involvement in SLE?
Blood and protein on urine dipstick
What is scleroderma characterised by?
Thickening of the skin
What is the epidemiology of scleroderma?
- UK Prevalence: 24/100,000
- UK Incidence: 10/1,000,000
- Onset: 30-50 years
- F:M 3:1
What is the name for scleroderma limited?
Crest syndrome
What are the features of Crest syndrome?
- Calcinosis
- Raynaud’s
- Oesophageal dysmotility
- Sclerodactyly
- Telangiectasia
What is a possible complication of limited scleroderma?
Pulmonary hypertension
What are the possible outcomes of diffuse scleroderma?
- Pulmonary fibrosis
- Renal crisis
- Small bowel bacterial overgrowth
What is the epidemiology of Sjogren’s syndrome?
- Prevalence: 1 in 100
- Incidence: 4 in 100,000
- Onset: 40-50yrs
- F:M 9:1
How can sjogren’s syndrome affect the GI system?
- Dysphagia
- Abnormal oesophageal motility
How can sjogren’s syndrome affect the respiratory system?
Interstitial lung disease
How can sjogren’s syndrome affect the CNS?
- Fits
- Hemiplegia
- Ataxia
- Cranial nerve lesions
How can sjogren’s syndrome affect the renal system?
Renal tubular acidosis
How can sjogren’s syndrome affect the skin?
- Palpable purpura
- Raynaud’s syndrome
How can sjogren’s syndrome affect the joints?
Arthralgia
How can sjogren’s syndrome affect the PNS?
- Sensory neuropathy
- Mononeuritis multiplex
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?
- Lymphoma
- Neuropathy
- Purpura
- Interstitial lung disease
- Renal tubular acidosis
What is the epidemiology of auto-immune myositis?
- Rare
- 6/ million incidence
What is auto-immune myositis characterised by?
-Symmetrical, diffuse, proximal muscle weakness
What are the 2 forms of auto-immune myositis?
- Polymyositis
- Dermatomyositis
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?
- Cancer
- Interstitial lung disease
What syndromes does auto-immune myositis overlap with?
Mixed Connective Tissue Disorders
- Soft tissue swelling
- Raynauds
- Myositis
- Arthalgia
Give examples of large vessel vasculitis.
- Takayasu arteritis
- Giant cell arteritis
Give examples of medium vessel vasculitis.
- Polyarteritis nodosa
- Kawasaki disease
Give examples of ANCA-associated small vessel vasculitis?
- Microscopic polyangitis
- Granulomatosis with polyangitis
- Eosionophilic granulomatosis with polyangitis
Give examples of immune complex small vessel vasculitis
- Anti GBM disease
- Cryoglobulinemic vasculitis
- 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
- New headache
- Temporal artery tenderness/reduced pulsation
- ESR≥50
- Abnormal temporal biopsy
Give examples of ANCA associated vasculitis.
- Granulomatosis with polyangiitis (Wegener’s)
- Microscopic polyangiitis
- 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?
- RA
- MS
- Infection
What is the specific ANA profile associated with SLE?
- dsDNA
- Ro
- Sm
What is the specific ANA profile associated with scleroderma?
- Scl-70
- Centromere
What is the specific ANA profile associated with polymyositis?
Jo-1
What is the specific ANA profile associated with Sjorgren’s disease?
- Ro
- La
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
What is the treatment for mild multi-system autoimmune disease?
Hydroxychloroquine
What is the treatment for moderate multi-system autoimmune disease?
- Azathioprine
- Methotrexate
- Mycophenolate
What is the treatment for severe multi-system autoimmune disease?
- Cyclophosphamide
- Rituximab
