Rheumatology Flashcards
What are features of polymyalgia rheumatica?
Weight loss Proximal muscle stiffness and tenderness Headaches Scalp tenderness Raised ESR
Why can false negatives occur with temporal biopsy in temporal arteritis?
The disease may patchily affect the artery so the sample may not contain inflammation
Why is it important to recognise and treat temporal arteritis early?
Reduce morbidity
Prevent blindness due to involvement of the optic arteries with retinal ischaemia
A 7 year old girl presents with ankle and knee pain and a skin rash. She has been well apart from a cold two weeks previously. She had a full term normal delivery with no neonatal problems. Her immunisations are up to date. There is no family or social history of note. On examination she looks well. The temperature is 37.8 with RR15, HR 80 and BP 100/70. She has a scanty non blanching rash over the shins. Her abdomen is soft and non tender. There is swelling, redness, tenderness and deceased movement of the right knee and left ankle. What is the likely diagnosis? What tests should be done now?
Henoch Schonlein purpura
Urine dipstick to look for renal involvement
A 76 year old lady presents with severe headache and pain on combing her hair. She has also noticed recent episodes of visual loss which last only a few seconds. During the past weeks she has been feeling more tired and has found it difficult to get up in the mornings due to stiff and aching joints. What is the immediate management?
Prednisolone 40-60mg daily
What are presenting complaints of Wegeners granulomatosis?
Severe haemorrhagic rhinorrhoea Paranasal sinusitis Nasal mucosal ulceration Serous or purulent otitis media Hearing loss Cough Haemoptysis Pleuritis Haematuria
What renal changes do you often see in Wegeners granulomatosis?
Glomerulonephritis with proteinuria, haematuria and red cell casts
What blood results might you see in Wegeners granulomatosis?
Raised ESR
Leukocytosis
Anaemia
ANCA
What are the Duckett Jones criteria for acute rheumatic fever?
Major: Migratory polyarthritis Pancarditis Chorea Erythema marginatum Nodules Minor: Fever Arthralgia Raised inflammatory indices Prolonged PR interval Diagnosis: recent strep infection, two major or one major and two minor criteria
What is splenomegaly associated with RA called?
Feltys syndrome
What are associated complications with RA?
Vasculitis
Pericarditis
Normochromic normocytic anaemia
Instability of Atlanto-axial joint
A 24 year old woman from western India presents with symptoms of lethargy and dizziness worse on turning her head. On examination her blood pressure is 176/128. Her pulses are impalpable at all peripheral sites. Auscultation of her chest reveals a systolic heart murmur. What is the diagnosis?
Takayasus arteritis
What conditions are associated as part of the autoimmune polyendocrine syndrome?
Pernicious anaemia Type 1 diabetes Addison's disease Vitiligo Sjogrens syndrome
What are features of SLE?
Fatigue Fevers Mouth ulcers Lymphadenopathy Malar rash Photosensitivity Raynauds Livedo reticularis Arthralgia Myocarditis Pleurisy Fibrosing alveolitis Glomerulonephritis Anxiety and depression Psychosis
What is the prevalence of ankylosing spondylitis?
1 in 2000
What are some complications of ankylosing spondylitis?
Chest pain/carditis Aortic regurgitation Cardiac conduction abnormalities Decrease in thoracic excursion Periositis of calcaneum and ischial tuberosities Amyloidosis Iritis Lung fibrosis
What immunology tests may be positive in SLE?
ANA 99% RF 20% Anti ds DNA Anti Smith Anti Ro Anti La
How can SLE be regularly monitored?
ESR
Complement levels low during active disease
Anti ds DNA titres
What is Feltys syndrome?
Splenomegaly and neutropenia in patient with RA
What are complications of RA?
Resp: pulmonary fibrosis, pleural effusion, nodules, methotrexate pneumonitis Ocular: keratoconjunctivitis sicca, episcleritis, scleritis, corneal ulceration, keratitis, steroid induced cataracts, chloroquine retinopathy Osteoporosis Ischaemic heart disease Increased risk of infections Depression Feltys syndrome Amyloidosis
A 48 year old male presents with an 8 week history of epistaxis and nasal stuffiness. On examination there is evidence of nasal crusting. A chest X-ray demonstrates multiple cavitary lesions, what is the diagnosis and what is the most appropriate antibody test?
Granulomatosis with polyangiitis (wegeners)
ANCA
What are features of granulomatosis with polyangiitis?
Upper respiratory tract: epistaxis, sinusitis, nasal crusting
Lower respiratory tract: dyspnoea, haemoptysis
Rapidly progressing glomerulonephritis
Saddle shaped nose deformity
Vasculitic rash
Proptosis
Cranial nerve lesions
What are features of antiphospholipid syndrome?
Recurrent miscarriages Strokes Thrombocytopenia DVT/PE MI Rash: livedo reticularis Chronic headaches, migraines
What investigations should be done for granulomatosis with polyangiitis?
cANCA positive in 90%
Chest X-ray
Renal biopsy: epithelial crescents in bowmans capsule
What is the management for granulomatosis with polyangiitis?
Steroids
Cyclophosphamide
Plasma exchange
What is the acute management of gout?
NSAIDs
Intra articular steroid injections
Colchicine
Oral steroids
What are features of temporal arteritis?
Rapid onset <1 month Headache Jaw claudication Visual disturbance secondary to anterior ischaemic optic neuropathy Tender palpable temporal artery Features of PMR: aching, morning stiffness Lethargy Depression Low grade fever Anorexia Night sweats
What are some side effects of methotrexate?
Myelosuppression
Liver cirrhosis
Pneumonitis
What are some side effects of sulfasalazine?
Rashes
Oligospermia
Heinz body anaemia
Interstitial lung disease
What are some side effects of leflunomide?
Liver impairment
Interstitial lung disease
HTN
What are some side effects of hydroxychloroquine?
Retinopathy
Corneal deposits
What are some side effects of prednisolone treatment?
Cushingoid features Osteoporosis Impaired glucose tolerance HTN Cataracts
What is a side effects of gold?
Proteinuria
What are some side effects of penicillamine?
Proteinuria
Exacerbation of myasthenia gravis
What are some side effects of etanercept?
Demyelination
Reactivation of TB
What are some side effects of azathioprine?
Black tarry stools Bleeding gums Blood in urine Fever Sores/ulcers in lips or mouth
What are X-ray findings of osteoarthritis?
Loss of joint space
Osteophytes
Subchondral sclerosis
Subchondral cysts
What are X-ray findings of rheumatoid arthritis?
Loss of joint space
Erosions
Soft tissue swelling
Soft bones - osteopenia
What is schobers test?
Line drawn 10 cm above and 5cm below back dimples. Distance between 2 lines should increase by more than 5cm when patients bends as far forwards as possible
Which respiratory problems may present in patients with rheumatoid arthritis?
Pulmonary fibrosis Pleural effusion Pulmonary nodules Bronchiolitis obliterans Methotrexate pneumonitis Pleurisy Caplans syndrome Infection secondary to immunosuppression
Which antibodies are associated with systemic sclerosis?
ANA
RF
Anti scl 70
Anti centromere
What tests can confirm antiphospholipid syndrome?
Lupus anticoagulant
Anti cardiolipin antibodies
What clotting abnormality might be detected in antiphospholipid syndrome?
Prolonged APTT that doesn’t correct when mixed with normal plasma
What are similarities and differences between granulomatosis with polyangiitis and churg Strauss syndrome?
Wegeners: renal failure, epistaxis, haemoptysis, cANCA
Both: vasculitis, sinusitis, dyspnoea
Churg-Strauss: asthma, pANCA, eosinophilia
Which systems are commonly involved in polyarteritis nodosa?
Skin Joints Peripheral nerves GI tract Kidney
What type of glomerulonephritis occurs in henoch schonlein purpura?
Membranoproliferative
What is lupus pernio?
Macular popular violaceous rash over nose cheeks and ears due to granulomatous infiltration of the skin usually due to sarcoidosis
What is lofgrens syndrome?
Erythema nodosum
Polyarthritis
Bilateral hilar lymphadenopathy
Why can sarcoidosis be associated with diabetes insipidus?
Sarcoid tissue may infiltrate endocrine glands
If it infiltrates the hypothalamus and posterior pituitary it can lead to cranial DI
What are the most common causes of erythema nodosum in the U.K?
Sarcoidosis Tuberculosis OCP Strep infections EBV IBD Haematological malignancies
What are some complications of sarcoidosis?
Eyes: Anterior uveitis, conjunctival deposits, dry eyes, choroidoretinitis, retinal haemorrhages
Heart: Cardiomyopathy, conduction system disease, cardiac failure
Diabetes insipidus
Skin: lupus pernio, erythema nodosum
Which types of clots do people with antiphospholipid syndrome get?
MI Recurrent miscarriage DVT Stroke Pulmonary hypertension due to thromboembolic disease
What is Takayasus arteritis?
Large vessel granulomatous vasculitis with intimal fibrosis and vascular narrowing
Young or middle age women of Asian descent
Mainly affects aorta, branches of and the pulmonary arteries
Inflammatory phase: malaise, fever, night sweats, joint pain
Fainting due to subclavian steak syndrome or carotid sinus sensitivity
Pulseless phase: vascular insufficiency, arm or leg claudication, renal artery stenosis and HTN
What is Buergers disease?
Thromboangiitis obliterans
Inflammation and thrombosis of small and medium arteries and veins of hands and feet
Strongly associated with smoking
What are some facial features of systemic sclerosis?
Facial telangiectasia
Small mouth
Beaking of the nose
What is the management for raynauds?
Calcium channel blockers eg nifedipine
IV prostacyclin infusions
What is lofgrens syndrome?
Acute form of sarcoidosis Bilateral hilar lymphadenopathy Erythema nodosum Fever Polyarthralgia
What is Mikulicz syndrome?
Form of sarcoidosis with enlargement of parotid and lacrimal glands
Can also be due to TB or lymphoma
What is Heerfordts syndrome?
Uveoparotid fever Parotid enlargement Fever Uveitis Secondary to sarcoidosis
When should allopurinol be started after an attack of gout?
Start if 2 or more episodes in a 12 month period
What level of uric acid is diagnostic for gout?
> 450 micromol/l
What are skin features of dermatomyositis?
Photosensitive Macular rash over back and shoulders Heliotrope rash in periorbital region Gottrons papules Nail fold capillary dilatation
What are non skin features of dermatomyositis?
Proximal muscle weakeness/tenderness Raynauds Respiratory muscle weakness Interstitial lung disease - fibrosing alveolitis or organising pneumonia Dysphagia Dysphonia
What are characteristics of behchets syndrome?
Recurrent oral and genital ulcers Uveitis Seronegative arthritis Central nervous system symptoms Fever Thrombophlebitis Erythema nodosum Abdominal symptoms Vasculitis
What is adult onset stills disease?
Persistent high spiking fevers
Joint pain
Salmon coloured bumpy rash
What is stills disease?
Systemic onset juvenile idiopathic arthritis
Type of JIA with extra articular manifestations like fever, rash, splenomegaly, lymphadenopathy
What are the major criteria for diagnosis of adult onset stills disease?
High fever lasting one week or longer
Joint pain lasting two weeks or longer
Rash
Abnormal white cell count and other blood problems
What are minor criteria for the diagnosis of adult onset stills disease?
Sore throat
Swelling of lymph nodes or spleen
Liver problems
Absence of rheumatoid arthritis
Which scoring system is used to assess disease activity in RA?
DAS score
Which scoring system is used to assess disease activity in ankylosing spondylitis?
BAS DAI
What conditions can underly dermatomyositis?
Malignancy: lymphoma, breast, lung, ovarian, colon
RA
IBD
What are radiological features of rheumatoid arthritis?
Joint space narrowing Periarticular osteoporosis Erosions Periarticular soft tissue swelling Joint destruction Subluxation
What are possible causes for anaemia in a patient with RA?
Anaemia of chronic disease
GI blood loss secondary to nsaid use
Bone marrow suppression due to gold, phenylbutazone, indomethacin, penicillamine
Megaloblastic anaemia due to folic acid deficiency or pernicious anaemia
Feltys syndrome
What is the name of the joint deforming arthropathy found in SLE which can resemble RA?
Jaccouds arthropathy
What is the name given to the genital rash in reactive arthritis?
Circinate balanitis
What is the name of the skin rash in reactive arthritis?
Keratoderma blennorrhagica
What are the 5 types of psoriatic arthropathy?
Symmetric Asymmetric DIP predominant Spondylitis Arthritis mutilans
Which disease is associated with pencil in cup deformity on X-ray?
Psoriatic arthritis
What is the tetrad of symptoms in henoch schonlein purpura?
Purpuric rash
Abdominal pain
Arthritis/arthralgia
Glomerulonephritis
How is a diagnosis of Ankylosing spondylitis made?
Clinical criteria: low back pain for more than 3 months improved by exercise but not relieved by rest
Limitation of lumbar spine motion in sagittal and frontal planes
Limitation of chest expansion relative to normal values for age and sex
Radiological: sacroiliitis on X-ray
Definite if radiological criteria plus at least one clinical criteria
Probable if three clinical criteria alone or if radiological criteria but no clinical
What is shiny corner sign?
Enthesitis in spine - ankylosing spondylitis
What is the gold standard treatment for ANCA vasculitis?
Three pulses of IV steroids
Pulsed IV cyclophosphamide
In what proportion of primary biliary cirrhosis does sicca syndrome occur?
70%
Which antibody is associated with limited systemic sclerosis?
Anti centromere
Which antibody is associated with diffuse systemic sclerosis?
Anti Scl70
What is the management of a patient on long term steroids who’s dexa scan shows osteopenia?
Vitamin D
Calcium
Oral bisphosphonate
Repeat scan in 1-3 years
What are causes of pyoderma gangrenosum?
Idiopathic IBD RA SLE Myeloproliferative disorders Lymphoma Myeloid leukaemia Monoclonal gammopathy PBC
Which antibody is present in drug induced lupus?
Anti histone antibodies
Which drugs cause drug induced lupus?
Procainamide
Hydralazine
What are the 3 stages of churg strauss?
Stage 1: allergy, asthma, allergic rhinitis
Stage 2: eosinophilia
Stage 3: vasculitis
What are skin manifestations of SLE?
Photosensitive butterfly rash
Discoid lupus
Alopecia
Livedo reticularis
What are the American college of rheumatology criteria for diagnosis of SLE?
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis: non erosive, 2 or more joints
Serositis: pleuritis or pericarditis
Renal disorder: proteinuria or casts
Neurological disorder: fits or psychosis
Haematological disorder: anaemia, lymphopenia, leucopenia, thrombocytopenia
Immunological disorder: anti dsDNA or anti SM antibodies
ANA positive
What are oslers nodes? Which conditions is it associated with?
Tender purple/red raised lesions with a pale centre which occur as a result of immune complex deposition
Occur in association with endocarditis, SLE, gonorrhoea, typhoid, haemolytic anaemia
What X-ray findings are seen early and late in RA?
Early: loss of joint space, juxta articular osteoporosis
Late: periarticular erosions, subluxation
Which autoantibody is associated with PBC?
Antimitochondrial antibody
What is usually the earliest symptom of PBC?
Pruritus which can precede jaundice and hepatomgaly by several years
What are patients with PBC at risk of?
Hypercholesterolaemia
Oesophageal varices
Malabsorption (fat soluble vitamins ADEK)
Osteomalacia
What is primary antiphospholipid syndrome characterised by?
Thrombosis (arterial and venous)
Recurrent (more than 2) miscarriages
In absence of SLE
Other features: chorea, autoimmune thrombocytopenia, livedo reticularis, thrombotic stroke, heart valve abnormalities
Which autoantibodies are raised in CREST syndrome?
Anticentromere antibodies
What are featues of CREST syndrome?
Calcinosis Raynauds Oesophageal dysmotility Sclerodactyly Telangiectasia
What are rheumatoid factor antibodies?
IgM autoantibodies directed against IgG
What are the criteria for diagnosis of rheumatoid arthritis?
Joint distribution: 1 large joint - 0 points, 2-10 large joints - 1 point, 1-3 small joints - 2 points, 4-10 small joints- 3 points, >10 joints- 5 points
Serology: Negative RF and negative anti-CCP antibodies - 0 points, Low positive RF or anti-CCP antibodies (less than 3 x normal) - 2 points, High positive RF or anti-CCP antibodies (>3 x upper normal limit) - 3 points
Symptom duration: <6 weeks - 0 points, 6 weeks or more - 1 point
Acute-phase reactants: Normal CRP and ESR - 0 points, Abnormal CRP or ESR - 1 point
6 or more points is RA
With which ANCA is MPA associated?
pANCA with MPO specificity
With which ANCA is GPA (wegeners granulomatosis) associated?
cANCA with PR3 specificity
Which autoimmune conditions are associated with vitiligo?
Hashimoto’s thyroiditis Graves’ disease Addison’s disease Diabetes mellitus Alopecia areata Pernicious anaemia IBD Psoriasis Autoimmune polyglandular syndrome
Which ENA antibody can be used to test for dermatomyositis?
Anti Jo
Which ENA can be used to test for SLE?
Anti smooth muscle
What type of antibody is rheumatoid factor?
IgM against IgG
Who should be screened for antiphospholipid antibodies?
Young adults (50 years old or less) with ischaemic stroke Women with recurrent pregnancy loss (3 or more pregnancy losses) before 10 weeks of gestation
Which antiphospholipid antibodies can be checked?
Lupus anticoagulant
Anticardiolipin (aCL) antibody
Anti-b2-glycoprotein I antibody
What are some causes of a raised CK?
Neuromuscular disorders Myopathies Muscular dystrophies eg duchennes Rhabdomyolysis Myositis Acute myocardial infarction After strenuous exercise Intramuscular injection Renal disease Drugs: Statins, Neuroleptic malignant syndrome: Antipsychotic drugs and Dopaminergic drugs esp if abrupt dose reduction
What investigations should be done for PMR?
FBC CRP/ESR CK TFTs Igs and EP strip Early morning Urinary light chains Assess for malignancy Exclude rheumatoid with RF and anti-CCP antibodies
What is management for PMR?
Prednisolone 15mg – 20 mg daily for 2-4 weeks
Followed by tapering dose: Reduce by 2.5 mg every 2–4 weeks until the patient is at 10 mg daily, Once on 10mg daily, reduce by 1mg monthly
Warn patients to expect a temporary increase in symptoms for about a week after each dose reduction
Prophylaxis if prednisolone >7.5mg od, or high fracture risk. Don’t forget calcium and vitamin D
Gastroprotection should be considered, particularly in older patients
Advice on physical activity, weight bearing activity is important
What are the most common sites for gouty tophi?
Fingers Helix/ anti-helix of ears Toes Olecranon bursae Olecranon
What type of crystals are found in gout?
monosodium urate
What investigations are useful for a diagnosis of gout?
Raised serum urate > 420umol/l
Synovial fluid with negatively birefringent crytals when viewed under polarised light
Measure serum urate at 6 weeks post-attack
What medication is used for acute gout?
NSAIDs
Colchicine (inhibits microtubule polymerisation by binding to tubulin, blocks neutrophil mediated inflam responses)
Prednisolone
Canakinumab (recombinant monoclonal antibody, inhibitor of proinflammatory cytokine IL-1. Licensed for use in patients whose condition has not responded to treatment with NSAIDs or colchicine, or who are intolerant of them. Can be used for symptomatic treatment of frequent gouty arthritis attacks (at least three in previous 12m)
What is the chronic management of gout?
Lifestyle measures: Weight loss, Diet, Exercise, Alcohol reduction, Increase water intake
Medication: Start allopurinol after two or more attacks of gout within a year or after first attack in people at higher risk- with one or more tophi, X-ray features of gouty arthritis, renal impairment, known uric acid stones, on long-term diuretic medication
Start allopurinol 1–2 weeks after inflammation has settled and titrate dose every few weeks until serum uric acid level below 300 micromol/L
When starting allopurinol, co-prescribe low dose NSAID or low-dose colchicine for at least 1 month to prevent acute attacks
Prescribe NSAIDs for up to 6 weeks and consider need for
gastroprotective medication. Prescribe colchicine for up to 6 months
If NSAIDs and colchicine are contraindicated, consider low-dose oral
prednisolone once a day for 4 to 12 weeks
What is second line for chronic gout if allopurinol is not tolerated or contraindicated?
Febuxostat
What is the mechanism of action of allopurinol?
Purine analogue
Inhibitor of xanthine oxidase so reduces production of uric acid
What can cause an increase in uric acid levels?
Dietary intake - meats, seafood, beans, yeast
Haematological causes - Chronic Haemolytic anaemia, CML
Alcohol
Genetic
Reduced renal excretion: Drugs - loop and thiazide diuretics, aspirin, Kidney disease, Diabetic ketoacidosis, Starvation, Genetic
What are rheumatological causes of secondary raynauds?
Systemic sclerosis (90% of patients have Raynaud’s phenomenon) Mixed connective tissue disease Systemic lupus erythematosus Dermatomyositis or polymyositis Rheumatoid arthritis Sjögren’s syndrome Vasculitis
What are haematological causes of secondary raynauds?
Polycythaemia ruba vera
Leukaemia
Thrombocytosis
Cold agglutinin disease (Mycoplasma infections)
Paraproteinaemias
Protein C deficiency, protein S deficiency, antithrombin III deficiency
Presence of the factor V Leiden mutation
Hepatitis B and C (associated with cryoglobulinaemia)
What features make primary raynauds more likely than secondary?
Younger age (usually under 30 yrs old) Female Genetic component (30% have an affected first degree relative) No symptoms/signs of underlying disease No tissue necrosis or gangrene Normal nail fold capillaries
What are key symptoms and signs of connective tissue disease?
Alopecia Arthralgia Arthritis Breathlessness Carpal tunnel syndrome Indigestion/GORD Mouth ulcers Photosensitivity Rashes Severe digital ischemia Weight loss
What investigations should be done for raynauds?
FBC
Inflammatory markers
ANA and ENA and dsDNA binding Antibody
If unilateral Raynauds: CXR – cervical rib, MRI if suspect thoracic outflow obstruction
What is the management of raynauds?
Conservative management: Keep warm peripheries, STOP SMOKING
Medical management: Vasodilators, Vasoconstrictor inhibitors, Calcium channel blockers - Nifedipine
What signs may be present in a patient with OA?
Tenderness to palpation of joint Bony thickening Small effusions Crepitus Deformity and restricted movement of joint Muscle wasting Generalised or localised arthritis Heberdens and Bouchards nodes in hands
What is the management of OA?
Weight reduction
Exercise to improve specific muscle strength
Exercise to improve aerobic fitness
Pain Control
Social support
Aids and appliances: insoles, walking sticks
Intra-articular corticosteroid injection if pain severe
Surgery: prosthetic joint replacement, consider referral for joint surgery for people with OA who experience joint symptoms (pain, stiffness and reduced function) that have a substantial impact on their quality of life and are refractory to non-surgical treatment
What pain control can be used in OA?
Paracetamol Topical NSAIDs Paracetamol/codeine NSAIDs: beware use in elderly, consider co-prescribing PPI, short-term use, lowest effective dose only TENs machine
What is joint hypermobility syndrome?
Musculoskeletal symptoms in presence of generalised joint
hypermobility not attributable to a systemic rheumatologic disease
What is the Beighton hypermobility score?
Passively extend the fifth MCP joint to > 90° (1+1)
Oppose thumb to the ant aspect of ipsilateral forearm (1+1)
Hyperextend elbow to > 10° (1+ 1)
Hyperextend knee to > 10° (1 + 1)
Place hands flat on floor without bending knees (1)
Score 5 or more for adults, 6 for children
What is the modified Brighton criteria for hypermobility?
Main criteria: Four or more points of Beighton criteria, Joint pain for longer than 3 months in at least 4 joints
Minor criteria: One to 3-points of Beighton criteria, Joint pain in one, 2 or 3 joints or back pain or dorsal root pain for more than 3 months, Dislocation or subluxation of more than one joint, or one joint more than once, Three or more periarticular lesions (tennis elbow, tenosynovitis, bursitis), marfanoid habitus (tall, thin, arachnodactyly), skin abnormalities: striations, hyperextensibility, thin skin, Ocular signs myopia or Antimongoloid type, Varicosities or hernias or uterine or rectal prolapse
What are signs of connective tissue disease/joint hypermobility syndrome?
Skin: striae
Cardiovascular: thoracic aortic dilatation/rupture/dissection, aortic regurgitation, mitral valve prolapse, mitral regurgitation, abdominal aortic aneurysm, cardiac dysrhythmia
Lungs: pleural rupture causing pneumothorax
Eyes: lens dislocation, closed-angle glaucoma, high myopia
Skeleton: arachnodactyly, hypermobility, arthralgia, joint instability, finger contractures, pectus excavatum, kyphoscoliosis
Nervous system: dural ectasia hernias presenting with low back
pain and symptoms akin to cauda equina syndrome or chronic
postural headache due to CSF leakage
Facial characteristics: maxillary/mandibular retrognathia, long
face, high, arched palate, enophthalmos, downslanting palpebral fissures and malar hypoplasia
What is chronic fatigue syndrome?
Disabling fatigue of at least six months with one or more of the following symptoms:
Difficulty with sleeping, insomnia, hypersomnia, unrefreshing sleep, disturbed sleep-wake cycle
Muscle/ joint pain, multi-site and without evidence of inflammation
Headaches
Painful lymph nodes without pathological enlargement
Sore throat
Cognitive dysfunction, difficulty thinking, inability to concentrate, impairment of short-term memory, difficulties with word-finding, planning/organising thoughts and information processing
Physical or mental exertion makes symptoms worse
General malaise or flu-like symptoms
Dizziness and/or nausea
Palpitations in the absence of identified cardiac pathology
What are causes of back pain?
Mechanical back pain: muscle strain, ligament, disc herniation, compression fracture, lumbar spinal stenosis, osteoarthritis, spondylolisthesis
Infections: discitis, epidural abscess, osteomyelitis
Primary Tumours (spinal cord or vertebrae)
Metastatic Vertebral Tumours
Ankylosing spondylitis and other inflammatory disorders
What are causes of vasculitis?
Idiopathic (45-55%)
Infection (15-20%): HSP, septic vasculitis, upper respiratory tract flares of Wegener’s granulomatosis, polyarteritis nodosa
Inflammatory disease (15-20%): SLE, RA, Crohn’s disease, UC
Drug-induced (10-15%): sulfonamides, beta-lactams, quinolones, NSAIDs,oral contraceptives, thiazides, anti-influenza vaccine
Chemicals such as insecticides and petroleum products
Neoplastic (<5%): paraproteinaemia or lymphoproliferative disorder
What is the chapel hill consensus conference classification for vasculitis?
Infection
Non-infective: Large vessel, Medium vessel, Small vessel, Variable vessel, Single organ, Systemic disease-associated, Probably associated
What are examples of infective vasculitis?
Rickettsial vasculitis
Syphilitic aortitis
Aspergillus arteritis
What are examples of different non infective vasculitis?
Large vessel: GCA Medium vessel: Kawasaki disease Small vessel: immune complex Variable vessel: Behçet's disease Single organ: isolated aortitis Systemic disease-associated: rheumatoid Probably associated: hepatitis B, hepatitis C
What are signs of small vessel vasculitis?
Palpable purpura 1-3 mm (may join to form plaques/ulcer) Tiny papules Splinter haemorrhages Urticaria Vesicles Livedo reticularis (rare)
What are features of medium vessel vasculitis?
Ulcers Digital infarcts Nodules Livedo reticularis Papulo-necrotic lesions Hypertension: damage to renal vessels
What are features of large vessel vasculitis?
End-organ ischaemia (TIA/CVE)
Hypertension
Aneurysms
Dissection/ haemorrhage or rupture
What is alopecia areata?
Autoimmune condition
Localised well demarcated patches of hair loss
At edge of hair loss might be small broken exclamation mark hairs
What is the prognosis in alopecia areata? What is the management?
Hair will regrow in 50% patients by 1 year and 80-90% eventually Topical or intralesional steroids Topical minoxidil Phototherapy Dithranol Contact immunotherapy Wigs
What is antiphospholipid syndrome?
Acquired disorder characterised by predisposition to venous and arterial thromboses, recurrent foetal loss and thrombocytopenia
Can be primary or secondary to other conditions eg SLE, lymphoproliferative disorders
Why does antiphospholipid syndrome cause rise in APTT despite being a prothrombotic condition)?
Ex vivo reaction of lupus anticoagulant autoantibodies with phospholipids involved in coagulation cascade
What is the management of anti phospholipid syndrome?
Initial VTE: warfarin, target INR 2-3 for 6 months
Recurrent VTE: lifelong warfarin. If VTE whilst already on warfarin then target INR 3-4
Arterial thrombosis: lifelong warfarin, target INR 2-3
Which drugs are used as an induction agent to rapidly gain control of disease in ANCA vasculitis? What is the mechanism?
Cyclophosphamide: causes DNA crosslinking and so apoptosis of rapidly dividing cells including lymphocytes
Rituximab: anti CD20 monoclonal antibody, causes B cell depletion
Which drugs are used as maintenance agents for ANCA vasculitis?
Azathioprine
Mycophenolate
What is the classic triad of symptoms in behcets syndrome?
Oral ulcers
Genital ulcers
Anterior uveitis
What are features of behcets syndrome?
Oral ulcers Genital ulcers Anterior uveitis Thrombophlebitis Arthritis Aseptic meningitis Abdo pain, diarrhoea, colitis Erythema nodosum DVT
What is feltys syndrome?
Rheumatoid arthritis
Splenomegaly
Granulocytopenia
What is polyarteritis nodosa?
Medium vessel vasculitis
Necrotising inflammation leading to aneurysm formation
More common in middle aged men
Associated with hep B
What are features of polyarteritis nodosa?
Fever Malaise Arthralgia Weight loss HTN Mononeuritis multiplex Testicular pain Livedo reticularis Haematuria ANCA Hep B
What are extra articular manifestations of RA?
Pulmonary: effusion, fibrosis, bronchiectasis, caplans nodules
Eyes: secondary sjogrens, scleritis
Haem: anaemia, feltys syndrome
Neuro: carpal tunnel, Atlanto axial subluxation
Other: amyloidosis, nephrotic syndrome, pericarditis
What is Auspitz sign?
If you pick off a psoriatic plaque, capillary bleed under it
What are the different types of psoriatic arthritis?
Mono/oligo (less than 4 joints) Spondylitis Asymmetrical polyarthritis (including DIP) Arthritis mutilans Rheumatoid like
Which antibodies are associated with dermatomyositis?
Anti Jo1
Anti Mi2
RhF
ANA
What are causes of raynauds?
SLE RA Dermatomyositis Ehlers Danlos Beta blockers Polycythemia rubra Vera MGUS Hypothyroidism
What are examples of different types of vasculitis?
Small vessel ANCA positive: microscopic polyangiitis, churg Strauss, wegeners
Small vessel ANCA negative: HSP, cryoglobulinaemia, goodpastures
Medium vessel: polyarteritis nodosa, Kawasaki disease
Large: takayasus arteritis, temporal arteritis
What are causes of mononeuritis multiplex?
Vasculitis HIV/AIDS RA Diabetes Sarcoidosis Polyarteritis nodosa Leprosy Carcinomatosis
