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
Heart defect with ehlers danlos syndrome?
Aortic Regurg
26
``` SE methotrexate? Pregnancy? Monitoring? What should be co-prescribed? What CANNOT be given with it? ```
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
When can anti-TNF drugs be considered in ank spond?
When they have failed on 2 different NSAIDs and meets the criteria for active disease on 2 occasions at least 12 weeks apart
28
What screening tests must be done for Rituximab?
Cardiac echo - can cause cardiac complications - esp of LVD
29
How many DMARDs must be tried before TNF inhibitors? Name 3 TNFI? What DAS28 values mean remission and high activity?
2, including MTX Etanercept, infliximab, adalimumab ``` <2.6 = remission >5.1 = high activity ```
30
OA XR signs?
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
Schober's test?
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
Hand features of OA?
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
Is obesity a CI for joint replacement in OA? Weight bearing after op? Thromboprophylaxis? 4 standard pieces of advice post-op for hip replacement?
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
'A' features of ank spond? (7)
``` Anterior uveitis Apical fibrosis Aortic regurg AV node block Amyloidisos Achilles tendonitis Arthritis (peripheral) ```
35
Ix for ank spond? | Rx?
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
What are the 4 seronegative arthropathies?
Ank spond Psoriatic Enteropathic Reactive All assoc w HLA-B27
37
Psoriatic arthritis - pattern? - other inflammations? - XR? - End stage? - Rx?
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
Enteropathic arthritis?
Usually large joint, assoc w UC/Crohn's Rx involves controlling bowel symptoms
39
Reactive arthritis?
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
Gout acute Rx? | After this?
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
Antibodies in dermatomyositis? Cutaneous features? Other features?
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
Sjogren's syndrome: - features? - Ix? - Rx? - increased risk of?
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
General management of SLE? Of acute flares? Skin symptoms? Nephritis?
Hydroxychloroquine Acute - haemolytic anaemia, nephritis, pericarditis, CNS disease - cyclophosphamide + pred Skin - topical steroids Nephritis - Cyclophosphamide or MMF
44
Anti-phospholipid syndrome: - What is it? - features? - antibodies? (3) - Rx?
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