fever/ fatigue - skin: malar rash, cutaneous lupus/ discoid lupus, ulcers - rheumatological: arthritis, synovitis >2jts - neuro: delirium, psychosis, seizure - serositis: effusions (pleural/ pericardial) - haematological: leukopenia, thrombocytopenia renal: nephritis

R: Autoimmune Disease Flashcards by Costanza Cascio

AI disease of joints can be divided into…

NMJ transmission disease, connective tissue disease and muscle disease

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the main disease in NMJ transmission disease is…

myasthenia gravis

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what is MG?

AI disorder of NMJ transmission

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epi of MG?

4/100,000, W, 30s

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which type of hypersensitivity reaction is MG?

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pathophysiology of MG?

auto IgG bind to Ach receptors on muscle cells which blocks ligand binding leading to defects in nerve impulse transmission at skeletal muscle NMJs

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aetiology of MG?

thyme hyperplasia (70% of MG pt have this), thymic tumour, some due to HLA association/ other AI disease

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s/s of MG?

severe muscular weakness, progressive fatigue

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what is a common early and late sign of MG?

early: ocular myasthenia
late: difficulty with mastication

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which 2 antibodies would be seen in a patient with MG?

anti AchR IgGs

anti MuSK abs

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which study may be useful in MG?

nerve conduction studies (EGM)

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mx of MG?

acute: O2, plasmapheresis, IV IgGs, high-dose corticosteroids
chronic: inc neurotransmission (anti-cholinesterase agents), surgery (thyroidectomy), immunosuppression

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what are the 5 connective tissue diseases?

systemic lupus erythematosus (SLE), sjogren’s syndrome, systemic sclerosis, antiphospholipid syndrome, mixed connective tissue disease

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what is SLE?

chronic autoimmune condition that attacks cells and tissues resulting in inflammation and tissue damage

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epi of SLE?

F (9:1), women of childbearing age, afro-carribean/ hispanic/ asian
20/100,000

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types of SLE?

Mild: rash, no organ involvement
Moderate: arthritis, rash
severe: major organ involvement- nephritis, pneumonitis etc

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aetiology of SLE?

genetic + environmental (smoking, silica exposure)

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antibodies for SLE?

anti-nuclear antibody, dsDNA, anti-sm, anti-Ro, anti-RNP

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pathogenesis of SLE?

inc apoptosis > cells release necrotic material that act as auto-antigens> B& T cells stimulated so produce antibodies > complexes form and deposited in basement membrane + activation of inflammatory cells = necrosis/ scarring

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s/s of SLE?

fever/ fatigue

skin: malar rash, cutaneous lupus/ discoid lupus, ulcers
rheumatological: arthritis, synovitis >2jts
neuro: delirium, psychosis, seizure
serositis: effusions (pleural/ pericardial)
haematological: leukopenia, thrombocytopenia
renal: nephritis

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ix for SLE?

serology: ANA, dsDNA (highly specific), anti-sm (highly specific), anti-Ro
bloods: complement proteins (C3/C4)
urinalysis
imaging for organ involvement

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SLE score for monitoring/prescribing is called…

SLEDAI score

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SLE mx?

sun protection, minimise steroid use
mild= HCQ
moderate: MTX
severe: biologics/ immunosuppression e.g. rituximab

SLE prognosis?

10 year survival is 90%

what is Sjogren's syndrome?

characterised by lymphocytic infiltrates in exocrine organs causing excessive dryness

s/s of sjogren's?

dry everything ! - dry eyes, mouth, vagina bilateral parotid gland enlargement, joint pain, fatigue

types of sjogren's

1y- absence of other pathology | 2y- along with RA or other AI disease

antibodies associated with sjogren's?

ANA, anti-Ro, anti La

ix for sjogren's?

serology- ANA, anti-Ro, anti La, inc IgG, inc plasma viscosity tests lip gland biopsy

what are the 2 tests done to investigate sjogren's?

schrimmer tear test, rose Bengal staining

mx for Sjogren's?

saliva replacement/tear replacement, HCQ for fatigue

what is systemic sclerosis?

multisystem AI disease characterised by vasculopathy, fibrosis, atrophy of skin

types of systemic sclerosis/ scleroderma?

limited cutaneous scleroderma*, diffuse cutaneous

antibodies associated with scleroderma?

ANA, anti-centromere, anti-Scl-70

s/s of scleroderma?

raynaud's, skin thickening/ calcinosis - facial: small mouth with puckering, beaked nose, tight skin, telangiectasia - GI: dysphagia, GORD, GAVE, malabsorption - cardio/resp: ILDs, pulmonary HT - renal: scleroderma renal crisis - vasculopathy: digital ischaemia, sclerodactyl

what is the process of Raynaud's...

1. blanching 2. acrocyanosis 3. reactive hyperaemia

mx for Raynauds

Ca channel blockers (nifedipine), ARBs/nitrates PDE-5 inhibitors

ix of scleroderma?

serology: ANA, anti-centromere, anti-poisimerase | organ screening= important!

mx of scleroderma?

treat each issue, stem cell transplant

what is antiphospholipid syndrome?

disorder of antiphospholipid ABs so blood prone to clotting which manifests clinically as recurrent thrombosis

antibodies associated with antiphospholipid syndrome?

anti-cardiolipin, lupus anti-coagulant, anti-beta 2 glycoprotein

s/s antiphospholipid syndrome?

miscarriage, headaches (migraines), levido articulares | MI/ stroke may be 1st time presentation

which type of endocarditis is seen with people with APS?

Libman-Stacks endocarditis

mx of APS?

warfarin, LMWH in pregnant women

what is CAPS?

a complication of APS- catastrophic APS is multi organ infarction that occurs over days to weeks

what is the main antibody associated to mixed connective tissue disease?

anti-RNP

Polymyositis: _____ muscle ache and ______ polymyalgia rheumatica: Proximal _____ _______ and pain myopathy: weakness but no ____

polymyositis: proximal muscle ache and weakness polymyalgia rheumatica: proximal morning stiffness and pain myopathy: weakness but no pain

what is polymyositis/ dermatomyositis?

inflammation of striated muscles causing symmetrical proximal muscle weakness

epi of polymyositis/ dermatomyositis?

females (2:1), rare, 45-60yo, inc risk of malignancy

pathophysiology of polymyositis/ dermatomyositis?

t-cell mediated cytotoxic process directed against muscle antigens

antibodies associated with polymyositis/ dermatomyositis?

anti-Jo-1, anti-SRP, ANA

difference between polymyositis/ dermatomyositis?

polymyositis: just proximal muscle weakness dermatomyositis: muscle weakness and skin rash

what is dermatomyositis like?

V-shaped rash on chest, heliotrope rash - eyes: may have peri-orbital oedema - fingers: Gottron's papules (purple patches on knuckles) - shawl sign

ix for polymyositis/ dermatomyositis

exam: test power, isotonic testing bloods: muscle enzymes- CK, inflammatory markers, antibodies EMG MRI Muscle biopsy

what are 2 signs that would be seen on muscle biopsy of polymyositis?

vascular inflammation and muscle necrosis

tx for polymyositis/dermatomyositis

corticosteroids- prednisolone, immunosuppression- MTX

T/F: malignancy is more common with dermatomyositis

true: breast, ovarian, stomach, lung, bladder, colon