Cortext Week 2 Flashcards
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
In RA are women or men more commonly affected?
Women by 2-3 times more
In the pathogenesis of RA - what is the immune response initiated against?
Synovium
Name 3 triggers of RA?
Smoking, infection and trauma
What joints are not affected in RA?
DIP
Over time in RA the cervical spine can be affected and can cause cervical cord compression - what causes this?
Atlanto-axial subluxation
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
What two autoantibodies can be measured in RA?
Rheumatoid factor
Anti-CCP - more specific!
What is the timeframe for starting DMARD therapy in RA?
3 months
What DMARD is usually first line in RA?
Methotrexate
What are patients with seronegative diseases usually positive with?
HLA-B27
In AS what gender is more commonly affected?
Males by 3:1 (age of onset 20-40)
Over time with AS what shape does the spine form?
Question mark - loss of lumbar lordosis and increased thoracic kyphosis
Name four associated conditions to AS? (A disease)
Anterior uveitis, aortitis, pulmonary fibrosis and amyloidosis
What class of drugs is not used in AS?
DMARDS - instead NSAIDs, anti-TNF inhibitors
Name two psoriatic nail changes?
Pitting and oncholysis
How is psoriatic arthritis treated
With DMARDS
What kind of infections most commonly cause reactive arthritis?
Genitourinary infections (Chlamydia and Neisseria) - joints become affectedc 1-3 weeks after the infection
What four conditions make up Reiters syndrome?
Uveitis, urethritis or conjuctivitis and arthritis
Cant see, cant pee, cant bend the knee
In what type of people is the prevelance of SLE high?
Black people
What is the genetic predisposition of SLE supported by?
40% concordance in monoxygotic twins
More than 90% of cases of SLE occur in which gender?
Women 11:1 during childbearing years. Onset is usually 20-30
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
Name the antibody specific to SLE and varies with disease activity?
Anti-dsDNA
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?
CK
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
- Wegeners granulomatosis (GPA)
- Microscopic polyangitis
- 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