Cortext Week 2 Flashcards by Joanna Lowrie

What are these features suggestive of - joint pain with associated swelling, morning stiffness, improvement with excersise, synovitis on examination, raised CRP and PV and extra-articular symptoms?

Inflammatory arthritis

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In RA are women or men more commonly affected?

Women by 2-3 times more

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In the pathogenesis of RA - what is the immune response initiated against?

Synovium

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Name 3 triggers of RA?

Smoking, infection and trauma

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What joints are not affected in RA?

DIP

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Over time in RA the cervical spine can be affected and can cause cervical cord compression - what causes this?

Atlanto-axial subluxation

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Name 4 categories of extra-articular features in RA?

Rheumatoid nodules
Lung involvement - pleural effusions, interstitial fibrosis and pulmonary nodules
Cardiovascular morbidity
Ocular involvement - keratoconjunctivitis sicca, episcleritis, uveitis and nodular scleritis that may lead to scleromalacia

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What two autoantibodies can be measured in RA?

Rheumatoid factor

Anti-CCP - more specific!

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What is the timeframe for starting DMARD therapy in RA?

3 months

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What DMARD is usually first line in RA?

Methotrexate

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What are patients with seronegative diseases usually positive with?

HLA-B27

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In AS what gender is more commonly affected?

Males by 3:1 (age of onset 20-40)

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Over time with AS what shape does the spine form?

Question mark - loss of lumbar lordosis and increased thoracic kyphosis

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Name four associated conditions to AS? (A disease)

Anterior uveitis, aortitis, pulmonary fibrosis and amyloidosis

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What class of drugs is not used in AS?

DMARDS - instead NSAIDs, anti-TNF inhibitors

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Name two psoriatic nail changes?

Pitting and oncholysis

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How is psoriatic arthritis treated

With DMARDS

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What kind of infections most commonly cause reactive arthritis?

Genitourinary infections (Chlamydia and Neisseria) - joints become affectedc 1-3 weeks after the infection

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What four conditions make up Reiters syndrome?

Uveitis, urethritis or conjuctivitis and arthritis

Cant see, cant pee, cant bend the knee

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In what type of people is the prevelance of SLE high?

Black people

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What is the genetic predisposition of SLE supported by?

40% concordance in monoxygotic twins

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More than 90% of cases of SLE occur in which gender?

Women 11:1 during childbearing years. Onset is usually 20-30

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List the presenting features of SLE. NO MAPID RASH

Neurological symptoms
Oral ulcers
Malar rash
ANA positive (95% of patients but not specific)
Photo sensitivity
Immunodeficiency
Discoid rash
Renal problems
Arthritis
Serositis
Haemoglobin low

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Name the antibody specific to SLE and varies with disease activity?

Anti-dsDNA

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Name the antibody in SLE with low sensitivity but specific?

Anti-Sm

When SLE is active what are the complement C3/4 levels?

Low

What test must be done when diagnosing SLE?

Urinalysis for evidence of glomerulonephritis

For skin disease and arthralgia in SLE what drugs are commonly used?

Hydroxychloroquine, topical steroids and NSAIDs

What conditions affecting the lungs and heart can SLE cause?

Pericardial effusion and interstitial lung disease - azathioprine may be required

For severe disease of SLE involving lupus nephritis or CNS lupus what treatment is given?

IV steroids and Cyclophosphamide

What disease presents with dryness of eyes and mouth, arhtralgia, fatigue, vaginal dryness and parotid gland swelling?

Sjogrens

What is there an increased risk of with Sjogrens?

Lymphoma

How is the diagnosis of Sjogrens confirmed?

Ocular dryness (Schirmers test), anti-Ro, anti-La and typical features on lip gland biopsy

Pilocarpine is a drug used in Sjogrens - what does it do and name a side effect?

Stimulates saliva production but can cause flushing

Pulmonary hypertension, Raynauds and fibrosis occur in what connective tissue disease?

Systemic sclerosis

Name the components of CREST?

Calcinosis, raynauds, esophageal dysmotility, sclerodactyly and telangactasia + pulmonary hypertension CREST syndrome is seen in systemic sclerosis

What happens to the nose in systemic sclerosis?

Beaking

What antibody is associated with limited systemic sclerosis?

Anti-centromere

What antibody is associated with diffuse systemic sclerosis?

Anti-Scl-70

How is Raynauds treated?

Calcium channel blockers, bosentan

How is renal involvement in systemic sclerosis treated?

With ACE inhbitiors

What are anti-RNP antibodies associated with?

Mixed connective tissue disease

What does anti-phospholipid syndrome manifest clinically as?

Recurrent venous or arterial thrombosis and/or foetal loss (late, spontaneous, second or third trimester, although can occur anytime)

What cardiovascular condition is seen in APS?

Libman-Sacks sterile endocarditis

What neurological symptom is seen in APS?

Migraine

What cutaneous finding is seen in APS?

Livedo reticularis

What condition can there be thrombocytopenia, lupus anticoagulant, anti-cardiolipin antibodies and anti0beta 2 glycoprotein positive?

Anti-phospholipid syndrome

How is APS managed?

Anti-coagulation (LMWH used in pregnancy)

What are gouty tophi?

Painless white accumulations of uric acid which occur in soft tissues and erupt throuh skin

What condition occurs in the elderly, involves proximal myalgia of the hip and shoulder girdle, morning stiffness lasting over 1 hour and symptoms improve as day goes on?

Polymyalgia rheumatica

What do 15% of patients with polymyalgia rheumatica develop?

Giant cell arteritis - 40/50% of patients with GCA have associated PMR

What do symptoms of PMR respond dramatically to?

Low dose steroids - prednisolone 15mg, reduced over 18 months until condition resolved

What condition involves visual disturbances, headache, jaw claudication and scalp tenderness?

GCA

What is raised in GCA?

PV, CRP and ESR

In GCA - what is jaw claudication a result of?

Ischaemia in maxillary artery

What is the definitive test for GCA?

Temporal artery biopsy

What is the treatment for GCA?

Prednisolone 40mg if no visual impairment but 60mg if visual symptoms. Give for 2 years until resolved.

What antibody is unique to dermatomyositis and polymyositis (symmetrical, proximal muscle weakness, more common in women)?

Anti-Jo-1 Also ANA, anti-SRP

Other than inflammatory markers, what else is raised in polymyositis and dermatomyositis?

When testing for polymyositis - what test findings are abnormal in 90% of patients?

Electromyographic EMG Muscle biopsy is also good to do

How is polymyositis managed?

Prednisolone 40mg with methotrexate or azathioprine

Give three clinical feature of dermatomyositis?

Vshaped rash over chest Gottrons papules Heliotropic rash

What three conditions are associated with fibromyalgia?

Depression IBS Migraine

Name two large vessel vasculitis?

Takayasu arteritis in under 50s usually young women | Giant cell arterirtis in over 50s

How is large vessel vasculitis managed?

Prednisolone 40-60mg and gradually reducing.

Name two medium vessel vasculitis

Polyarteritis nodosa | Kawasaki disease

Name three ANCA associated small vessel vasculitis

1. Wegeners granulomatosis (GPA) 2. Microscopic polyangitis 3. Churg-Strauss syndrome

If the vasculitis is ANCA associated with no granulomas present what is the diagnosis?

Microscopic polyangitis

If the vasculitis is ANCA associated with granulomas and asthma and eosinophils present - what is the diagnosis?

Churg-Strauss syndrome

If the vasculitis is nonANCA associated and IgA dominant immune deposition what is the diagnosis?

Henoch=-Schonlein Purpura

If the vasculitis is non-ANCA associated and not IgA dominatn immune deposition present, also serum cryoglobulin present - what is the diagnosis?

Cryoglobulinemic vasculitis

Name some features of small vessel vasculitis?

Fever, weight loss, a raised non-blanching purpuric rash, arthralgia, mononeuritis multiplex, glomerulonephritis and lung opacities

What symptoms are common in GPA?

ENT - nose bleeds, deafness, recurrent sinusitis and nasal crusting

What two associations does GPA have?

cANCA and PR3

What is the most important complication of microscopic polyangitis?

Glomerulonephritis

How are most cases of ANCA associated vasculiris treated?

IV steroids and cyclophosphamide

Who does Henoch Schonlein purpura affect?

Commonly children

In henoch schonlein purpura - what commonly precedes the symptoms?

URTI

What causes purpuric rash over the buttocks and lower limbs, abdo pain, vomiting and joint pain in choldren?

Henoch schonlein purpura

How is henoch schonlein purpura treated?

It is self limiting