Connective Tissue Disease Flashcards by Anna Skelton

What are connective tissue diseases

Autoimmune conditions assoc with spontaneous over activity of the immune system
Doesnt actually affect the CT

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What does all CT diseases have in common

Specific auto antibodies

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Name some CT disease

Systemic lupus erythematous 
Sjögren’s syndrome 
Systemic sclerosis 
Dermatomyositis / polymyositis 
Anti - phospholipid syndrome

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What are spondyloathropathies

Family inflammatory arthritis characterised by involvement of both spine and joints
HLA B27 assoc

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What are the 4 main spondyloarthropathies

Ankylosing spondylitis
Enteropathic arthritis
Reactive arthritis
Psoriatic arthritis

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How does mechanical back pain differ from inflammatory back pain

Mechanical - worse by activity, at end of day , better with rest
Inflammatory - worse with rest, better with activity, early morning stiffness >30 mins

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What are the other features present in most spondlyoarthropathies

Enthesitis 
Inflammatory arthritis 
Dactylitis 
Ocular inflammation 
Mucocutaneous lesions 
Rare aortic incompetence or heart block

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What is enthesitis

Inflammation at insertion of tendons into bones

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What is dactylitis

Sausage digits - inflammation of entire digits

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What is the treatment of ankylosing spondylitis

1st line : NSAIDs
DMARDS
Anti - TNF e.g infliximab

Obv physio

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What is inflammatory myopathies characterised

Weakness of the muscle

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How is polymyalgia rheumatica characterised

Pain and stiffness

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What is fibromyalgia characterised

Pain and fatigue

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What is myopathy

Disorders of muscles in which muscle fibres don’t function properly

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What is vasculitis

Inflammation of the blood vessels

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What are the classification of vasculitis

Large vessel
Medium vessel
Small vessel

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What are the large vessel vascualitis

Takayasu arteritus, giant cell arteriti

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What are the medium vessel vasculitis

Poly arteritis nodosa

Kawasaki disease

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What age does giant cell affect

> 50

Features of giant cell

Causes temporal arteritis 
Acute, unilateral temporal headache 
Jaw claudication 
Scalp tenderness
Visual disturbances

Ix of giant cell

ESR
Plasma viscosity
GOLD standard: temporal artery biopsy

Management of giant cell

40mg prednisolone if no visional effect

60 if there is

What are the features of takayasus arteritis

< 40

Females

What is the main symptoms of takaysus arteritis

Claudication
Blood pressure difference ie cant tell pulse on one arm
Bruit ( most commonly being carotid)

Ix of takayasus arteritis

Angiogram

What are the two categories of small vessel vasculitis

ANCA - assoc small vessel vasculitis | Non - ANCA assoc small vessel vasulitis

What are the ANCA associated small vessel vasculitis

Wegener’s granulomatosis Microscopic polyangitis Churg Strauss syndrome

What are the ANCA non assoc small vessel vasculitis

Henoch - schnlein purpura | Serum cryoglobulin

Is there granuloma present in microscopic polyangitis

Is there granuloma in churg Strauss syndrome and wegener’s granulomatosis

Yes

Difference in churg Strauss syndrome

Asthma and eosinophilia is present in churgs NOT wegener’s

What vasculitis is IgA Dominant immune deposit

Henoch schnlein purpura

No IgA dominant immune deposit, there is serum cryoglobulin

Cryoglobulinemia

Old name for wegener’s graulomatosis

Granulomatosis with polyangitis ( GPA )

Old na,e for Churg - Strauss syndrome

Eosinophilic granulomatosis with polyangitis ( EGPA)

Old name for microscopic polyangitiis

Microscopic polyangitis ( MPA)

Who gets wegener’s

Males | 33-55

What is henoch schonlein purpura mediated by

Acute immunoglobulin A mediated disorder | = ALWAYS A TRIGGER.. maybe a UTI

Management of HSP

Self limiting Symptoms will resolve within 8 weeks Release may occur Check urinalysis for renal involvement

What is the general ix for vasculitis

Urine dipstick ( as kidney involvement is asymptomatic bit life threatening) Gold standard: tissue biopsy ANCA, CT, complement level ( will be depleted)

Treatment for localised / early systemic ANCA associated vasculitis ( AVV)

Methotrexate + steroids

Treatment for generalised / systemic

1st line: Cyclophosphamide + steroids Rituximab + steroids ( alternative) Plasma exchange if creatinine > 500 Followed by azathioprine with alternatives being methotrexate

What is localised / early systemic

Upper / lower resp tract disease without any other systemic involvements Without organ involvement or life threatening

What is generalised / systemic

Renal ( creatinine < 500) or other vital organ failure

What is refractory

Progressive disease unresponsive to steroids + cyclo

Treatment of refractory

IV immunoglobulins | Rituximab