Rheumatology Flashcards
How would you examine the hands in PACES?
Look: at hands, elbows for nodules, ears for tophi, hairline for psoriasis
Feel: feel systematically
Move: assess power, grip my fingers tightly
FUNCTION: pick up a coin off table, undo button
What the criteria for diagnosing RA?
ACR/EULAR criteria
Joint distribution 1 large joint 0 2-10 large joints 1 1-3 small joints 2 4-10 small joints 3 >10 joints 5
Serology
Negative RF and negative anti-CCP 0
Low positive RF/anti-CCP 2
High positive RF/Anti-CCP 3
Symptom duration
< 6weeks 0
>6 weeks 1
Acute phase reactants
Normal CRP/ESR 0
Abnromal ESR/CRP 1
More than or equal to 6 is RA
What are the signs of RA in hands?
Symmetrical deforming polyarthritis affecting small joints of hands, wrists and elbows, sparing DIP Ulnar deviation of MCP joints Boutounnieres/ swan neck deformities Z thumb Piano key ulnar head Rheumatoid nodules Surgical scars
What are the extra-articular manifestations of RA?
Haematological: anaemia, splenomegaly (Feltys syndrome)
Skin: nodules, vasculitis, pyoderma gangrenosum
Eyes: scleritis, episcleritis, scleroderma perforans
CV: valvular disease, pericarditis, conduction defects, myocardial infarction, myocarditis, HF
Respiratory: pul. fibrosis, pleural effusions, bronchiolitis obliterans
Renal: amyloidosis
Neurological: peripheral neuropathy, mononeuritis multiplex, compression neuropathies, cervical myelopathy-atlanto-axial subluxation, carpal tunnel, ulnar neuropathy
What investigations would you do in somebody with possible RA?
FBC- anaemia, neutropenia
CRP/ESR
Rheumatoid factor: antibodies that recognise the Fc portion of IgG
Anti-CCP- increased specificity, TB can cause false positive
XR signs: periarticular osteopenia, erosions at margins of joints, joint space narrowing, osteopenia, subluxation
Doppler US/MRI for acute synovitis
CXR: pul.fibrosis or nodules
LFTs
HRCT
What are the treatment options for RA?
Exercise, physio, OT, home modification, mobility aids
DMARDS
Methotrexate + at least one other + short term glucocorticois
Others: sulphasalazine, leflunomide, hydroxychloroquine, gold, penicillamine
Biologics
Used if inadequate response to at least 2 DMARDs including methotrexate
- TNF-I: infliximab, adalimumab, golimumab, etanercept
Anti-CD20: rituximab
Anti-IL6: toculizumab
CTLA4 Ig: Abatercept
How would you monitor a patient on a DMARD or anti-TNF?
Specialist review with history and examination
monitor FBC, U+E,. LFT
Infections
What are the side effects of methotrexate?
Hepatitis, alveolitis (pulmonary fibrosis), stomatitis
Pancytopenia/marrow suppression
WHat is the treatment of methotrexate toxicity?
Folinic acid
How do you manage a patient on anti-TNA who presents to A+E with minor infection?
Admit
Stop anti-TNF
IV abx
Seek specialist advice
List the possible causes of anaemia in RA
Anaemia of chronic disease GI bleed due to NSAID or steroid use Feltys syndrome Renal amyloid Marrow suppression- methotrexate Autoimmune haemolytic anaemia Assoc pernicious anaemia
How do you distinguish RA from OA on XR?
RA: juxta-articular osteopenia, erosion, symmetry, deformity
OA: subchondral sclerosis, osteophytes
Both: joint space narrowing, bone cysts
What should you ask about in a person with possible psoriatic arthritis?
FH: if 1st degree relative has it the increases chances x50 Back pain Iritis IBD Enthesitis Plantar fasciitis Dactylitis DIP joint involvement Nail signs
How would you manage somebody with psoriatic arthritis?
NSAIDs mild non-erosive disease
Steroid injection esp enthesitis
DMARD- sulphasalazine or leflonamide
Methotrexate is sig skin disease
AVOID hydroxychloroquine as may worsen skin
Steroid in flares
Consider anti-TNF
Rarely goes into remission so lifelong treatment
How do you differentiate between PsA and RA?
Asymmetry Nail changes Dactylitis Negative anti-CCP/RhF FH/PH of psoriasis
DO you know any HLA assoc of psoriatic arthritis?
HLA-B27 with sacroilitis
HLA-DR4 with RA distribution
HLA- B38/39- peripheral distal arthritis
What are the 11 criteria in SLE?
ACR criteria in diagnosis of SLE
- Malar rash: butterfly-shaped rash across cheeks and nose
- Discoid (skin) rash: raised red patches
- Photosensitivity: skin rash as result of unusual reaction to sunlight
- Mouth or nose ulcers: usually painless
- Arthritis (nonerosive) in two or more joints, along with tenderness, swelling, or effusion. With nonerosive arthritis, the bones around joints don’t get destroyed.
- Cardio-pulmonary involvement: inflammation of the lining around the heart (pericarditis) and/or lungs (pleuritis)
- Neurologic disorder: seizures and/or psychosis
- Renal (kidney) disorder: excessive protein in the urine, or cellular casts in the urine
- Hematologic (blood) disorder: hemolytic anemia, low white blood cell count, or low platelet count
- Immunologic disorder: antibodies to double stranded DNA, antibodies to Sm, or antibodies to cardiolipin
- Antinuclear antibodies (ANA): a positive test in the absence of drugs known to induce it.
Requires at least 4 criteria
What might you find on examination in patient with SLE?
Lymphadenopathy or fever Arthritis: Jaccoud's arthropathy (deformity at rest) which resolves on making fist Malar rash Discoid lupus Vasculitis SCarring alopecia Mouth ulcers Livedo reticularis Libman-Sacks endocarditis- AR/MR Pulmonary fibrosis/effusions Nephrotic syndrome or signs of RRT CN lesions, mononeuritis Cushingoid from steroids
What are the key features of Sjorgrens syndrome?
Dry eyes
Dry mouth
Bilateral parotid gland enlargement (differentials are lymphoma, sarcoidosis, parotid tumour, mumps)
What are the investigations for Sjorgrens syndrome?
Schirmers test- filter paper in lower eyelid and close eyes, normal >15mm
ENA antibodies: anti Ro/La
If antibodies negative then salivary gland biopsy
What is the management of Sjorgrens?
If primary require long term follow up as risk of lymphoma is 40x
Tear and salvia replacement
How would you investigate somebody with possible SLE?
Immunological tests:
- ANA
- DsDNA
- ENA: Ro, La, Smith, RNP
- C3, C4
- Antiphospholipid antibodies
FBC: anaemia, thrombocytopenia, lymphopenia, coombs test
Urine: dip and casts
CXR
ECHO
Skin biopsy
How do you manage SLE?
Simple analgesics , NSAIDs (caution)
Steroids
Hydroxychloroquine
Cyclophosphamide- reserved for life threatening disease- lupus nephritis, vasculitis, cerebral disease
Mycophenolate mofetil- as effect as cyclophosphamide with lower risk ovarian failure
Azathioprine
Biologics have been used
IVIg and plasma exchange in life threatening disease
Renal disease: ACE-I/ARB
Anti-phospholipid syndrome: warfarin/LMWH
What are the chances of passing SLE to offspring?
Genetic factors important but chances are low
What investigations help to determine if a patients lupus is active?
Urinalysis for blood and protein Urine cytology for red cell casts U+E ESR FBC- cytopenia Anti-dsDNA C3, C4
What are the major risks of cyclophosphamide therapy and what can be done to prevent them?
Infection
- reactivation of latent TB, HIV, hep B/C
- bone marrow suppression
- septrin prophylaxis
Bladder toxicity- haemorrhagic cystitis, use mesna
Malignancy
Infertility- freeze sperm, ovarian protection
Nausea, vomiting, alopecia, teratogenicity
What is scleroderma?
A spectrum of disease encompassing:
- Raynauds phenomonen: 2nday to systemic sclerosis, SLE, Sjorgrens, MCTD, dermatomyositis
- Localised scleroderma- Morphoea, Linear scleroderma, En Coup de Sabre
- Systemic sclerosis- limited or diffuse (CREST)
What are your screening questions for connective tissue diseases?
Do you have any rashes? Worse in sun? Are you losing hair? Do you suffer with mouth ulcers? Do you have dry eyes and mouth? Are you short of breath? (pul.htn) Do you have difficulty swallowing/heartburn? Do you have diarrhoea/weight loss? Are your muscles painful/ weak? Do you have difficulty rising from a chair?
What are the signs in systemic sclerosis?
Face: microstoma, facial telangiectasia
Hands: sclerodactyly, ulcers, calcinosis, Raynaulds
Cardioresp: pul.htn, pul.fibrosis
What are the complications of systemic sclerosis?
GI: oedophageal dysmotility, small bowel and large bowel involvement- chronic diarrhoea, malabsorption, bacterial overgrowth
Pulmonary involvement: pul.htn and fibrosis
Renal crisis: sudden onset htn, headache, visual disturbance, seizures, thrombocytopenia
What investigations would you do in systemic sclerosis?
ANA Anti-Scl70 (topoisomerase) - diffuse Anti-centromere- limited Nailfold capillaroscopy: enlarged cappileries, haemorrhages, dropouts Lung function tests HRCT: ILD ECHO: pul pressures XR hands: calcinosis
What is the treatment of systemic sclerosis?
No proven effective treatment
Raynaulds: CCB, iloprost infusions, sildenafil, endothelin receptor antagonists
Consider immunosuppression: MTX, cyclophosphamide
Treat pul.htn: sildenafil, endothelin receptor antagonists: bosentan, iloprost
Renal: ACE-i
Can you cure systemic sclerosis and what are the chances of passing on to children?
No but most slowly progress over years
All autoimmune diseases have an increased incidence in close relatives but not hereditary
If a young patient presents with Raynaulds how would you determine if it was primary or secondary?
Any symptoms of CTD
Autoantibodies
Examination of nail folds or capillaroscopy
late onset or acute onset
What is the presentation and management of scleroderma renal crisis?
Prognosis is poor and is more common in diffuse SSc.
Can be precipitated by corticosteroids
Acute hypertension, rapidly rising creatinine and sometimes evidence of microangiopathic haemolytic anaemia
Treatment involves ACE-I, ITU support and iloprost infusions
What can trigger a flare of gout?
Alcohol Excess purines - shell fish, oily fish, legumes Haemorrhage Infection Trauma Surgery Radiotherapy Dehydration Drugs- thiazide diuretics, low dose aspirin, theophylline, ciclosporin, levodopa, ethambutol
What conditions predispose you to gout?
Renal impairment Hypothyroidism Myeloproliferative/lymphoproliferative disorders Polycythaemia rubra vera Hyperparathyroidism Diabetes mellitus Diabetes insipidus Barters syndrome Sarcoidosis Psoriasis Hyperlipidaemia Obesity
Which drugs lower urate levels?
Losartan
Fenofibrate
How would you investigate somebody with possibly gout?
Serum urate, lipids, renal and thyroid function
XR joints: punched out lateral erosions with sclerotic margins and overhanging edges. Joint space preserved until late disease and no juxta-articular osteopenia
What is your management of gout?
Acute: NSAIDs, Colchicine, Short course of steroids
Prophylaxis:
Lifestyle modification- diet, alcohol, weight loss
Allopurinol- if >3 attacks/yr, erosions, tophi, renal impairment
Feboxstat- new powerful xanthine oxidase inhibitor
Uricase occasionally
What medical conditions cause the undersecretion of urate?
90% of patients are undersecretors of urate (10% are over producers) Renal disease Ketoacidosis/lactic acidosis Respiratory acidosis Hypothyroidism Hyperparathyroidism
What is the mechanism of action of allopurinol and why is it not helpful in an acute attack of gout?
Allopurinol inhibits xanthine oxidase
Xanthine oxidase converts hypoxanthine to xanthine which is converted to uric acid
Allopurinol inhibits xanthine oxidase so lowers concentration of insoluble urates in tissues, plasma and urine and increases amount of more soluble xanthenes in tissues.
Initiation of allopurinol therapy may lower serum urate levels rapidly with potential urate reabsorption from tissue deposits provoking an attack
What important drug interactions do you need to know about in the management of gout?
Allopurinol and azathioprine
- mercaptoprine is metabolised by xanthine oxidase pathway so inhibiting this may cause toxic bone marrow suppression
Allopurinol and warfarin
- Effect of warfarin increased due to inhibition of this metabolism
Colchicine and statins
- Increase risk of myopathy
Colchicine and ciclosporin
- nephrotoxicity and /or myopathy
What are the differential diagnosis of acute hot gout?
Septic arthritis
Gout
Pseudogout
Monoarticular presentation related to polyarticular gout (psoriatic, reactive arthritis, acute sarcoidosis)
Rare conditions: Avascular necrosis, coagulopathy (haemophillia), Whipples disease, Palindromic rheumatism
What investigations would you perform on somebody with an acute hot gout?
XR
Joint aspirate- septic/crystal
If you suspect mycobacterial infection- synovial biopsy
Bloods: FBC, CRP, ESR, RhF, ANA, HLA-B27, serum ace, urate level, blood culture
PA CXR: TB, sarcoid, sacroiliac joints
MRI: avascular necoriss, osteomyelitis, PVNS, tauma
What are the absolute contraindications to aspiration of an acutely inflamed joint?
Prosthetic joint Overlying cellulitis Overlying psoriatic arthritis Excess anticoagulation INR >3 Coagulopathy
Having obtained joint fluid aspirate, which investigations should be ordered?
Urgent gram stain and cell count
MC&S
Cytology for crystals
Zeil-Neilson stain and culture of AFP
What are the signs of temporal arteritis?
Thickened inflamed, tender temporal arteries
Check visual acuity and fields
Fundoscopy: blurred, swollen disc of acute optic neuritis, central retinal artery occlusions, optic atrophy
Peripheral pulse in head and neck tender, thrombosed
What are the differential diagnosis of PMR?
Inflammatory arthritis eg RA Muscle disease eg PM, DM Paraneoplastic syndrome Hypo/hyperthyrodism Infective endocarditis OA Bilateral frozen shoulder Depression
what are the investigations would you perform on somebody with possible PMR?
ESR (>100 normally)
Other bloods: FBC, U+E, LFT, ferritin, CK, ANA, RhF, anti-CCP
Temporal artery biopsy
If diagnostic uncertainty remains then PET scan may be indicated
What is the management of PMR and temporal artery biopsy?
IV methylprednisolone (if visual symptoms) or high dose oral prednisolone 60mg OD. Reducing over a couple of years, intially by 5mg each week then 2.5mg, every week and every 4weeks
For PMR usually only 15-20mg
Monitor ESR
Bisphosphonate
What symptoms differentiate giant cell arteritis from other forms of headache?
Cranial symptoms- headache precipitated by brushing hair, scalp tenderness, jaw claudication, abnormal tongue sensation, visual disturbance
Constitutional symptoms- fever, weight loss, malaise
Response to steroid therapy
Who would you offer bone protection therapy and how would you investigate them?
Average age of patient puts them at high risk of OP
Commence a bisphosphonate with steroids (NICE recommends consider anybody taking >7.5mg for longer then 3 weeks)
Perform DEXA at end of treatment to assess risk
Talk me through the examination of somebody with possible ankylosing spondylitis?
Observe for hyperextension of cervical spine and loss of lumbar lordosis (back against wall- normal distance =0cm)
Active cervical spine movements: flexion, extension, lateral flex right and left and lateral rotation L&R
Assess low back movements: forward flexion, extension, lateral flexion L& R, lateral rotation, L&;R- slide arm down side of leg, cross arms over chest- fix pelvis and twist
Schobers test: Mark 10cm above dimples of venous and 5cm below, ask patient to flex forward and measure distance between the 2 line (increase <5cm positive)
Finger to floor distance on forward flexion (normal is <19cm)
FABERS test: sacroiliac tenderness
Examine CV system: pulse rate, heart block, AR, cardiomyopathy
Listen apex lungs for pul. fibrosis and assess chest expansion
Check BP and urine dip (renal impairment due to NSAIDs, amyloidosis, IgA nephropahty
What are the differential diagnosis of Ank spond?
Psoriatic spondyloarthropathy
IBD related spondyloarthropathy
Scheuermanns disease
What investigations would you do for somebody with AS?
Radiographs of lumbosacral spine and views of sacroiliac joints CXR- apical fibrosis MRI FBC, U+E. CRP/ESR HLA-B27
How do you assess severity of Ankylosing spondylitis?
BASDAI score (Bath anklosing spondylitis disease activity index) Subjective questionairre rating symptoms on a scale
What is the management of anklosing spondylitis?
Physio, NSAIDs
TNF-alpha blockers (intially used 2nd line but new evidence suggests better to use them earlier)
Cortisone injections for enthesitis
What is the value of HLA-B27 test in a patient with back pain?
90% of caucasians with AS are HLA-B27 positive (drops with other ethnic groups)
Presence of HLA-B27 is latitude dependent (more in scandinavia)
Can you reassure him that his child will not develop the condition?
2% of HLA-B27 positive individuals develop AS but this rises to 15-20% if 1st degree relative has the disease
What advice should be given to a patient with AS who recieves anti-TNF?
Always inform doctor or dentist they are on treatment
Stop 2 doses before surgery
Stop if any infection
Seek urgent medical attention if infection
Be up to date with vaccinations
What are the associations with ankylosing spondylitis?
Aortitis Aortic regurgitation Anterior uveitus Achilles tendonitis Amyloidosis Apical lung fibrosis
What are the complications of Pagets disease?
Skeletal: bone pain, deformity and fracture
Neurological: deafness, CN palsies, spinal stenosis, headaches, optic atrophy, cerebral steal syndrome (pagetic bone has a 3x increased blood flow)
Cardiac: angina, HTN, rarely high output cardiac failure
Malignancy: osteogenic sarcoma, fibrosardcoma, benign giant cell tumour
Metabolic: hypercalcaemia, nephrocalcinosis
How do you treat Pagets disease?
Bisphosphonates
Which tests are helpful in Pagets disease?
ALP- frequently elevated
Serum calcium and phosphate: normal
PTH normal
XR: osteolytic or osteoblastic lesions or mixture, trabecular thickening of inner aspect of pelvis
Technetium bone scan- shows uptake, mistaken malignancy
If available urinary markers of bone turnover such N–telopeptide may be useful
What are the important side effects of bisphosphonates?
Bone: bone pain, hypocalcaemia, micro-fractures
GI tract: oesophagitis, ulcerative
Systemic: fever, flu examination, arthrlagia, myalgia
Dental: osteonecorsis
What score is used to grade skin tightness in systemic sclerosis?
Rotnan score
How do you grade severity of RA?
DAS28 score (disease activity score- looks at 28 joints)
Count number of tender or swollen joints
Measure ESR/CRP
> 5.2 3 months apart to qualify for biologic therapy
What questions do you ask for arthritis in feet and what do you need to remember to examine?
Ask if they have trouble walking on cobbled streets?Remember the talus joint: hand on sole of foot
Remember to look for callous on bottom of feet, may suggest subluxation of toes
How might an adult appear who has had JIA since childhood?
Small stature
Small chin due to TMJ involvement
Cushingoid
Can get radial deviation of fingers instead of ulnar as growing.
What is the antibody in mixed connective tissue disease?
Anti-RNP
