Rheumatology Flashcards

1
Q

Vasculitides:

  • 2 large vessel?
  • 2 medium vessel?
  • 3 ANCA small vessel?
  • 2 immune complex mediated small vessel?
  • 1 which only affects the smallest vessels (capillaries)?
A

Large: GCA, takayasu

Medium: polyarteritis nodosa, kawasaki

ANCA small: microscopic polyangitis, GPA, eGPA

Immune complex small: IgA (HSP), cryoglobulinaemic vasculitis

Capillaries: anti-GBM

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2
Q

Shared features in ANCA vasculitis?

A
Fever, weight loss
Purpuric rash
Arthralgia
GN
Mononeuritis multiplex
Lung opacities (CXR)
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3
Q

Specific features of GPA?

A

(Wegener’s)

  • cANCA (PR3)
  • ENT - epistaxis, crusting, oral ulcers, sinusitis, saddle deformity
  • LRT - dyspnoea, cough, haemoptysis
  • Uveitis
  • Necrotising GN
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4
Q

Specific features of eGPA?

A

(Churg-Strauss)
Similar to GPA + late-onset asthma + eosinophilia

Paranasal sinusitis
Mononeuritis multiplex/polyneuropathy

pANCA (anti-MPO)

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5
Q

Specific features of microscopic polyangitis?

A

Similar vasculitic picture
Necrotising GN v common

pANCA 60%
cANCA 40%

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6
Q

Rx ANCA vasculitides?

A

Cyclophosphamide + steroids (1st line)

Plasma exchange 2nd line

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7
Q

In GCA what is raised on blood test?

What is seen on CK, MR angio and PET CT?

A

ESR, CRP, PV raised

CK, MR angio and PET CT all normal

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8
Q

HSP where do they often get pain?

Rx?

A

Colicky abdo pain
(prupura on buttocks/legs)

Self-limiting

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9
Q

Kawasaki disease management? (3)

A

High dose aspirin
IVIG
Echocardiogram (coronary aneurysm)

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10
Q

Apart from vasculitis what else may pANCA be positive in? (anti-MPO)

A

UC (70%)
PSC (70%)
Anti-GBM (25%)
Crohn’s (20%)

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11
Q

General 1st line investigations for suspected vasculitis?

A

Urinalysis - blood and protein

Bloods:

  • FBC - normochromic/cytic anaemia & raised PLT
  • U&E - renal impairment
  • CRP - raised

CXR - nodular, fibrotic or infiltrative lesions

Kidney/lung biopsies may be taken by specialists to aid diagnosis

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12
Q

Rare life-threatening complication of RA?

Extra-articular manifestations?

A

Atlanto-axial subluxation

lung fibrosis
rheumatoid nodules
CVD
ocular stuff

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13
Q

XR of RA?
What other imaging may be of use?
What may be seen in hands?

A

Early - normal

  • Periarticular osteopenia/osteoporosis
  • Periarticular erosions
  • Subluxation
  • May be loss of joint space

USS - synovitis

Hands - ulnar deviation, Z-shaped thumb, swan neck/boutonniere

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14
Q

Initial Ix for RA?

What else is RF positive in?

A

RF and anti-CCP (ACPA)

Have similar sensitivity (70%) but anti-CCP much more specific and can be detected 10 years before development of RA

RF also pos in:

  • Felty’s syndrome (RA, splenomegaly, neutropaenia)
  • Sjrogen’s
  • infective endocarditis
  • SLE
  • systemic sclerosis
  • general population (5%)
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15
Q

Azathioprine:

  • What may be tested for in people before starting it?
  • SE?
  • Drug interaction?
  • Safe in pregnancy?
A

Thiopurine methyltransferase (TPMT) deficiency - look for individuals prone to azathioprine toxicity

SE:

  • myelosuppression
  • nausea/vomiting
  • pancreatitis
  • BCC/SCC skin cancer

Allopurinol - use lower doses of azathioprine

Yes

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16
Q

Antibodies in SLE?

A

ANA - 99%, not specific but useful to rule out

anti-dsDNA (70% sensitive, 99% specific)

anti-Smith/Sm (30% sensitive, 99% specific)

RF (20%)

Also anti-U1 RNP, anti-Ro, anti-La

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17
Q

Monitoring of SLE?

A

Raised ESR
CRP usually normal in active disease - raised CRP may indicate underlying infection

C3 and C4 complement levels LOW during active disease (used up in immune complex formation)

anti-dsDNA titres (but only present in 70%)

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18
Q

Systemic sclerosis:

  • what small bowel complication can occur?
  • Usual causes of death?
  • Monitoring
  • Management of Raynaud’s, Renal, GI and ILD problems with SSc?
A
  • bacterial overgrowth (Rx Rifampicin or Co-amox)
  • Lung or renal involvement (renal crisis from accelerated HTN)
  • Renal monitoring, pulmonary function tests, echocardiogram (fibrosis of heart muscle)

Raynaud’s: CCB or bosentan
Renal: ACEI (regardless of age)
GI: PPI (reflux from oesophageal dysmotility)
ILD: Cyclophosphamide

19
Q

5 drugs which can cause drug-induced lupus?

Ig?

A

Most common: hydralazine, procainimide

Less common: Isoniazid, minocycline, phenytoin

ANA and anti-histone +ve
Anti-dsDNA -ve

20
Q

4 XR findings in ank spond?

A
  • subchondral erosions
  • subchondral sclerosis
  • squaring of lumbar vertebrae
  • syndesmophytes (ossification of outer annulus fibrosus fibres)
21
Q

Systemic sclerosis:

  • difference in symptoms between limited and diffuse?
  • antibodies?
  • what is scleroderma?
A

Limited: CREST
Scleroderma affects distal limbs, tends not to affect above elbows

Diffuse:
Scleroderma affecting proximal limbs and trunk
Pulmonary fibrosis, pulmonary hypertension, kidney failure

Limited: anti-centromere
Diffuse: anti-Scl-70

Scleroderma - cutaneous involvement only, no internal organ involvement. Plaques are called morphoea

22
Q

Sulfasalazine:

  • people with allergies to what drugs may also have allergy to it?
  • SE? (5)
  • is it safe in pregnancy/breastfeeding?
A

Aspirin

Oligospermia
SJS
pneumonitis/lung fibrosis
heinz body anaemia
Myelosuppression

Yes

23
Q

Behcet’s classic triad?
Other symptoms?
Tests?

A

Oral ulcers, genital ulcers, anterior uveitis

Also: erythema nodosum, thrombophlebitis, colitis, arthritis, aseptic meningitis, acne-like lesions

No diagnostic tests
Pinpricks with needles may lead to pustule formation at puncture site

24
Q

SE hydroxychloroquine?

Is it safe in pregnancy?

A

bulls eye retinopathy - baseline ophthalmological exam necessary and annual screening

Yes

25
Q

Heart defect with ehlers danlos syndrome?

A

Aortic Regurg

26
Q
SE methotrexate?
Pregnancy?
Monitoring?
What should be co-prescribed?
What CANNOT be given with it?
A

Pneumonitis/pul fibrosis
Myelosuppression
Mucositis
Liver fibrosis

Both women and men must stop methotrexate for 6 months before trying to conceive

Weekly FBC, LFT and U&E until dose stabilised, then every 2-3 months (drug given weekly)

Folic acid 5mg

Trimethoprim - risk of bone marrow aplasia and fatal pancytopaenia
Caution with high dose aspirin as well - can increase effects of methotrexate by reducing excretion

27
Q

When can anti-TNF drugs be considered in ank spond?

A

When they have failed on 2 different NSAIDs and meets the criteria for active disease on 2 occasions at least 12 weeks apart

28
Q

What screening tests must be done for Rituximab?

A

Cardiac echo - can cause cardiac complications - esp of LVD

29
Q

How many DMARDs must be tried before TNF inhibitors?
Name 3 TNFI?
What DAS28 values mean remission and high activity?

A

2, including MTX

Etanercept, infliximab, adalimumab

<2.6 = remission
>5.1 = high activity
30
Q

OA XR signs?

A

LOSS

Loss of joint space
Osteophytes
Subchondral sclerosis
Subchondral cysts

1st - paracetamol + topical NSAID (if hand/knee)

2nd - NSAIDs/COX-2 inhibitors, opioids, capsaicin cream, intra-articular steroid injections
(PPI with NSIAD/COX-2, don’t give these drugs if on cardioprotective aspirin)

Non-pharm - support braces, TENS, in-soles, walking aids

If conservative fails then joint replacement

31
Q

Schober’s test?

A

Put dots in midline 10cm above PSIS and 5cm below - bend forward to touch toes, distance between dots should increase by 5+cm if normal

32
Q

Hand features of OA?

A

Painless Heberden’s (distal) and Bouchard’s (proximal) nodes - bony swellings (osteophytes)

Squaring of thumb base - deformity of CMC joint resulting in fixed adduction

CMC and PIP joints most commonly affected

Stiffness <30 mins. Intermittent ache provoked by use. Usually no functional impairment

33
Q

Is obesity a CI for joint replacement in OA?
Weight bearing after op?
Thromboprophylaxis?
4 standard pieces of advice post-op for hip replacement?

A

Relative - no extra adverse long-term outcomes in terms of joint survival

Weight bearing as tolerated ASAP

LMWH for 4 weeks post-op

  • Avoid hip flexion >90 degrees
  • avoid low chairs
  • do not cross legs
  • sleep on back for first 6 weeks
34
Q

‘A’ features of ank spond? (7)

A
Anterior uveitis
Apical fibrosis
Aortic regurg
AV node block
Amyloidisos
Achilles tendonitis
Arthritis (peripheral)
35
Q

Ix for ank spond?

Rx?

A

HLA-B27 (90% +ve)
Raised ESR CRP

1st - spinal XR

  • sacroiliitis - subchondral sclerosis/erosions
  • squaring of lumbar vertebrae
  • syndesmophytes - ossification of annulus fibrosus
  • bamboo spine (late and uncommon)

If XR normal but high clinical suspicion - MRI
- marrow oedema

CXR - apical fibrosis

RX: NSAID’s
Must try at least 2 before TNF inhibitors
DMARD’s only useful if peripheral arthritis
Regular exercise
Monitor spirometry for restrictive defect

36
Q

What are the 4 seronegative arthropathies?

A

Ank spond
Psoriatic
Enteropathic
Reactive

All assoc w HLA-B27

37
Q

Psoriatic arthritis

  • pattern?
  • other inflammations?
  • XR?
  • End stage?
  • Rx?
A

Asymmetric oligoarthritis, usually affective small joints first - can affect DIP
Look for nail/skin changes

Dactylitis, enthesitis and tenosynovitis

  • Erosions
  • Tuft resorption
  • Eventually pencil-in-cuo

Arthritis mutilans - telescoping

Same as RA - DMARDs, try MTX first
Joint replacement for large joints

38
Q

Enteropathic arthritis?

A

Usually large joint, assoc w UC/Crohn’s

Rx involves controlling bowel symptoms

39
Q

Reactive arthritis?

A

AKA Reiter’s Syndrome (nazi)

Large joint arthritis
Urethritis/recent diarrhoea
Uveitis

Also yellow/brown papules on palms/soles called keratoderma blenorrhagica, and circinate balanitis

Rx: NSAID’s, intra-articular steroids
Sulfasalazine if persistent
Rarely lasts past 12 months

40
Q

Gout acute Rx?

After this?

A

NSAIDs or Colchicine 1st line
(Colchicine main Rx diarrhoea - and has slower onset)

Oral steroids 2nd line

Urate lowering therapy after 1st attack:

  • allopurinol 1st line (lower dose if reduced GFR) - wait 2 weeks after acute to start
  • febuxostat 2nd line
41
Q

Antibodies in dermatomyositis?
Cutaneous features?
Other features?

A

Anti-Jo-1
Anti-Mi-2
Anti-SRP

Heliotrope rash (eyes)
Shawl sign (over shoulders)
Gottron's papules (dorsal MCP and along extensor tendons)

Proximal muscle weakness, ILD, dysphagoa, raynaud’s

Prednisolone +/- MTX/azathioprine

42
Q

Sjogren’s syndrome:

  • features?
  • Ix?
  • Rx?
  • increased risk of?
A

Exocrine dysfunction - may be primary or secondary to RA.

  • dry mouth/eyes/vagina
  • Arthralgia/myalgia
  • Raynaud’s
  • Parotid swelling (recurrent)
  • Sensory polyneuropathy
Ix:
- RF, ANA, anti-Ro, anti-La
(Ro and ANA most sensitive - 70%)
- Schirmer's test - <5mm in 5 mins
- histology - focal lymphocytic infiltration
  • artificial tears
  • pilocarpine (muscarinic agonist) may be used to stimulate saliva

60-fold increased risk lymphoid malignancy

43
Q

General management of SLE?
Of acute flares?
Skin symptoms?
Nephritis?

A

Hydroxychloroquine

Acute - haemolytic anaemia, nephritis, pericarditis, CNS disease
- cyclophosphamide + pred

Skin - topical steroids

Nephritis - Cyclophosphamide or MMF

44
Q

Anti-phospholipid syndrome:

  • What is it?
  • features?
  • antibodies? (3)
  • Rx?
A

Predisposition to both arterial and venous thrombosis with recurrent foetal loss and thrombocytopenia. Can be primary or secondary to others, usually SLE

Arterial/venous thrombosis
Recurrent foetal loss
Livedo reticularis
Thrombocytopaenia
Pulmonary hypertension (due to multiple/recurrent emboli)
Prolonged aPTT
- due to reaction of lupus anticoagulant with phospholipids involved in coag cascade

Anti-cardiolipin
Lupus anticoagulant
anti-beta 2 glycoprotein

Primary prophylaxis: low-dose aspirin

Secondary: Lifelong warfarin
Target INR 2-3 if initial
Recurrent - 3-4