Multisystem Autoimmune Diseases Flashcards
What are types of connective tissue diseases?
SLE, systemic sclerosis, Sjogren’s syndrome, autoimmune myositis and mixed connective tissue disease
What are types of systemic vasculitidies?
Giant cell arteritis, granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis
What can myalgia/ arthralgia lead to?
Episcleritis - sinusitis - renal failure
Skin rash - pleurisy - CVA
Skin thickening - GORD - pulmonary hypertension
Sicca - skin rash - neuropathy
What is the approach of multisystem inflammatory disease?
Cardinal clinical features
Bedside investigations
Immunology
Imaging
Tissue diagnosis
Exclusion of differential diagnoses
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
What is the incidence of SLE?
4 in 100000
9:1 female to males
Onset is 15-50 years
Significant ethnic diversity
What is the aetiology for SLE?
Genetic factors - polygenic
Hormonal factors
Environmental factors - UV light, drugs and infections
What is the pathogenesis of SLE?
Immune response against endogenous nuclear antigens
Immune complex formation
Complement activation
Tissue injury
What is the clinical criteria for SLE?
Malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal, neurological, haematological, immunological and ANA
(any 4)
What is the classes of lupus nephritis?
1 - minimal mesangial
2 - mesangial proliferation
3 - focal
4 - diffuse
5 - membranous
6 - advanced setting
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
What are the antibodies in SLE?
ANA
Seen in 95% of SLE but not specific to SLE
Seen in inflammatory, infectious and neoplastic diseases
When is ANA seen in diseases?
SLE, systemic sclerosis, poly/ dermatomyositis, Sjogren’s syndrome, MCTD and drug induced lupus
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
What is the classification for scleroderma?
Localised scleroderma
Systemic sclerosis - limited cutaneous systemic sclerosis (CREST) and diffuse cutaneous systemic sclerosis
What is the incidence of systemic sclerosis?
10 in 1000000
Onset is 30-50 years
More females to males 3:1
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
