Rheumatology Flashcards

1
Q

How would you examine the hands in PACES?

A

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

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2
Q

What the criteria for diagnosing RA?

A

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

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3
Q

What are the signs of RA in hands?

A
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
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4
Q

What are the extra-articular manifestations of RA?

A

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

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5
Q

What investigations would you do in somebody with possible RA?

A

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

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6
Q

What are the treatment options for RA?

A

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

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7
Q

How would you monitor a patient on a DMARD or anti-TNF?

A

Specialist review with history and examination
monitor FBC, U+E,. LFT
Infections

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8
Q

What are the side effects of methotrexate?

A

Hepatitis, alveolitis (pulmonary fibrosis), stomatitis

Pancytopenia/marrow suppression

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9
Q

WHat is the treatment of methotrexate toxicity?

A

Folinic acid

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10
Q

How do you manage a patient on anti-TNA who presents to A+E with minor infection?

A

Admit
Stop anti-TNF
IV abx
Seek specialist advice

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11
Q

List the possible causes of anaemia in RA

A
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
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12
Q

How do you distinguish RA from OA on XR?

A

RA: juxta-articular osteopenia, erosion, symmetry, deformity

OA: subchondral sclerosis, osteophytes

Both: joint space narrowing, bone cysts

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13
Q

What should you ask about in a person with possible psoriatic arthritis?

A
FH: if 1st degree relative has it the increases chances x50
Back pain
Iritis
IBD
Enthesitis
Plantar fasciitis
Dactylitis
DIP joint involvement
Nail signs
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14
Q

How would you manage somebody with psoriatic arthritis?

A

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

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15
Q

How do you differentiate between PsA and RA?

A
Asymmetry
Nail changes
Dactylitis
Negative anti-CCP/RhF
FH/PH of psoriasis
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16
Q

DO you know any HLA assoc of psoriatic arthritis?

A

HLA-B27 with sacroilitis
HLA-DR4 with RA distribution
HLA- B38/39- peripheral distal arthritis

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17
Q

What are the 11 criteria in SLE?

A

ACR criteria in diagnosis of SLE

  1. Malar rash: butterfly-shaped rash across cheeks and nose
  2. Discoid (skin) rash: raised red patches
  3. Photosensitivity: skin rash as result of unusual reaction to sunlight
  4. Mouth or nose ulcers: usually painless
  5. Arthritis (nonerosive) in two or more joints, along with tenderness, swelling, or effusion. With nonerosive arthritis, the bones around joints don’t get destroyed.
  6. Cardio-pulmonary involvement: inflammation of the lining around the heart (pericarditis) and/or lungs (pleuritis)
  7. Neurologic disorder: seizures and/or psychosis
  8. Renal (kidney) disorder: excessive protein in the urine, or cellular casts in the urine
  9. Hematologic (blood) disorder: hemolytic anemia, low white blood cell count, or low platelet count
  10. Immunologic disorder: antibodies to double stranded DNA, antibodies to Sm, or antibodies to cardiolipin
  11. Antinuclear antibodies (ANA): a positive test in the absence of drugs known to induce it.

Requires at least 4 criteria

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18
Q

What might you find on examination in patient with SLE?

A
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
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19
Q

What are the key features of Sjorgrens syndrome?

A

Dry eyes
Dry mouth
Bilateral parotid gland enlargement (differentials are lymphoma, sarcoidosis, parotid tumour, mumps)

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20
Q

What are the investigations for Sjorgrens syndrome?

A

Schirmers test- filter paper in lower eyelid and close eyes, normal >15mm
ENA antibodies: anti Ro/La
If antibodies negative then salivary gland biopsy

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21
Q

What is the management of Sjorgrens?

A

If primary require long term follow up as risk of lymphoma is 40x
Tear and salvia replacement

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22
Q

How would you investigate somebody with possible SLE?

A

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

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23
Q

How do you manage SLE?

A

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

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24
Q

What are the chances of passing SLE to offspring?

A

Genetic factors important but chances are low

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25
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 ```
26
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
27
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)
28
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? ```
29
What are the signs in systemic sclerosis?
Face: microstoma, facial telangiectasia Hands: sclerodactyly, ulcers, calcinosis, Raynaulds Cardioresp: pul.htn, pul.fibrosis
30
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
31
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 ```
32
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
33
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
34
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
35
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
36
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 ```
37
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 ```
38
Which drugs lower urate levels?
Losartan | Fenofibrate
39
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
40
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
41
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 ```
42
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
43
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
44
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
45
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
46
What are the absolute contraindications to aspiration of an acutely inflamed joint?
``` Prosthetic joint Overlying cellulitis Overlying psoriatic arthritis Excess anticoagulation INR >3 Coagulopathy ```
47
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
48
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
49
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 ```
50
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
51
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
52
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
53
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
54
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
55
What are the differential diagnosis of Ank spond?
Psoriatic spondyloarthropathy IBD related spondyloarthropathy Scheuermanns disease
56
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 ```
57
How do you assess severity of Ankylosing spondylitis?
``` BASDAI score (Bath anklosing spondylitis disease activity index) Subjective questionairre rating symptoms on a scale ```
58
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
59
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)
60
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
61
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
62
What are the associations with ankylosing spondylitis?
``` Aortitis Aortic regurgitation Anterior uveitus Achilles tendonitis Amyloidosis Apical lung fibrosis ```
63
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
64
How do you treat Pagets disease?
Bisphosphonates
65
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
66
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
67
What score is used to grade skin tightness in systemic sclerosis?
Rotnan score
68
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
69
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
70
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.
71
What is the antibody in mixed connective tissue disease?
Anti-RNP