Multisystem Autoimmune Diseases Flashcards by Sophie Pitcairn

What are types of connective tissue diseases?

SLE, systemic sclerosis, Sjogren’s syndrome, autoimmune myositis and mixed connective tissue disease

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What are types of systemic vasculitidies?

Giant cell arteritis, granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis

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What can myalgia/ arthralgia lead to?

Episcleritis - sinusitis - renal failure
Skin rash - pleurisy - CVA
Skin thickening - GORD - pulmonary hypertension
Sicca - skin rash - neuropathy

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What is the approach of multisystem inflammatory disease?

Cardinal clinical features
Bedside investigations
Immunology
Imaging
Tissue diagnosis
Exclusion of differential diagnoses

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What are mimics for multisystem autoimmune diseases?

Malignancy - lymphoma
Infections - endocarditis, hep B + C, TB and HIV
Drugs - cocaine, minocycline and propylthiouracil
Cardiac myxoma
Cholesterol emboli
Scurvy

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What is the incidence of SLE?

4 in 100000
9:1 female to males
Onset is 15-50 years
Significant ethnic diversity

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What is the aetiology for SLE?

Genetic factors - polygenic
Hormonal factors
Environmental factors - UV light, drugs and infections

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What is the pathogenesis of SLE?

Immune response against endogenous nuclear antigens
Immune complex formation
Complement activation
Tissue injury

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What is the clinical criteria for SLE?

Malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal, neurological, haematological, immunological and ANA
(any 4)

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What is the classes of lupus nephritis?

1 - minimal mesangial
2 - mesangial proliferation
3 - focal
4 - diffuse
5 - membranous
6 - advanced setting

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When should a diagnosis of SLE be considered?

Women of childbearing age, fever, weight loss, malaise, severe fatigue, skin rash, arthritis, pleuritic chest pain, renal disease and cytopenia

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What are the antibodies in SLE?

ANA
Seen in 95% of SLE but not specific to SLE
Seen in inflammatory, infectious and neoplastic diseases

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When is ANA seen in diseases?

SLE, systemic sclerosis, poly/ dermatomyositis, Sjogren’s syndrome, MCTD and drug induced lupus

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What are other autoantibodies are seen in SLE?

Anti-ds DNA - highly specific for SLE
Anti-Sm - highly specific for SLE
Anti-Ro - neonatal lupus
Antiphospholipid antibodies - anti-cardiolipin and lupus anticoagulant

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What is the classification for scleroderma?

Localised scleroderma
Systemic sclerosis - limited cutaneous systemic sclerosis (CREST) and diffuse cutaneous systemic sclerosis

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What is the incidence of systemic sclerosis?

10 in 1000000
Onset is 30-50 years
More females to males 3:1

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What is the aetiology of systemic sclerosis?

Environmental - silica, solvents and viral infections
Genetic predisposition

What is the pathogenesis of systemic sclerosis?

Vascular damage, immune system activation and fibrosis

What is included in limited SSc?

Anti-centromere antibodies
Pulmonary hypertension
Gastrointestinal

What is included in diffuse SSc?

Anti-Scl70 antibodies
Pulmonary fibrosis
Renal crisis
Small bowel bacterial overgrowth

What is the incidence of Sjogren’s syndrome?

4 in 100000
Onset is 40-50 years
More females to males 9:1

Describe Sjogren’s syndrome

Dry eyes and mouth
Anti Ro (SSA) and anti La (SSB) antibodies
Salivary gland biopsy
Parotid gland enlargement
1/3 have systemic upset

What is involved in systemic upset for Sjogren’s syndrome?

Fatigue, fever, myalgia, arthralgia and dry skin

What are the other systemic symptoms in Sjogren’s syndrome?

GI - dysphagia and abnormal oesophageal motility
Resp. - interstitial lung disease
PNS - sensory neuropathy and mononeuritis multiplex
CNS - fits, hemiplegia, ataxia and cranial nerve lesions
Skin - palpable purpura and Raynaud’s syndrome
Joints - arthralgia

What are the complications of Sjogren's syndrome?

Lymphoma, neuropathy, cutaneous vasculitis, interstitial lung disease and renal tubular acidosis

What is the incidence of polymyositis and dermatomyositis?

More common in females Peak incidence in 50-60 year olds Increased risk of maligancy

Describe myositis

Muscle weakness - symmetrical and proximal Raised CK level, EMG, MRI and muscle biopsy Interstitial lung disease - anti Jo1 antibodies

What are the cutaneous signs of myositis?

Gottron's papules Heliotrope rash

What syndromes does myositis overlap with?

Mixed connective tissue disease - Raynaud's, soft tissue swelling, myositis and arthralgia

What are the vasculitides?

Large vessel vasculitis Medium vessel vasculitis Small vessel vasculitis

Describe the giant cell arteritis classification criteria

3 of the following - age of onset above 50, new headache, temporal artery tenderness, ESR>50 and abnormal temporal biopsy

What imaging can be used for giant cell arteritis?

Temporal artery biopsy, US doppler, CT angiogram, MR angiogram, and FDR PET

What are the complications of giant cell arteritis?

Irreversible vision loss Aortic aneurysms Arterial stenosis and limb ischaemia Stroke

What is the treatment for giant cell arteritis?

Urgent high dose of Prednisolone PPI Bone protection Steroid sparing medication

What are the types of ANCA associated vasculitis?

Granulomatosis with polyangiitis Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis

Describe granulomatosis with polyangiitis (Wegener's)

Necrotising granulomatous inflammation Usually involved upper and lower resp. tract Hearing loss, sinusitis and haemoptysis Necrotising GN is common

What antibodies are found in granulomatosis with polyangiitis?

cANCA and anti PR3 antibodies

Describe microscopic polyangiitis (MPA)

Necrotising vasculitis, few or no immune deposits and mostly small vessels Granulomatosis inflammation is absent Renal and pulmonary involvement is common

What are the antibodies found in microscopic polyangiitis?

pANCA and anti MPO antibodies

Describe eosinophilic granulomatosis with polyangiitis (Chrug Strauss)

Eosinophil rich and necrotising granulomatous inflammation often involving resp. tract Late onset asthma, nasal polyps and eosinophilia Mainly small and medium vessels Neuro involvement Cardia and GI then poor prognosis

What antibodies are seen in eosinophilic granulomatosis with polyangiitis?

40-60% have anti MPO antibodies

What is the immunological profile for SLE?

DsDNA, Sm, Ro, low C3 and C4, cardiolipin and lupus anticoagulant