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FRACP > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What are the 3 components of Lesch-Nyhan syndrome?

A

Lesch-Nyhan syndrome:

  1. Hyperuricaemia - gout
  2. Behavioural – self-mutilation/harm
  3. Neurological – cerebral palsy like motor deficits
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2
Q

Which 3 drugs can cause drug induced lupus?

A

Drug-induced lupus (HIP): hydralazine, isoniazid, procainamide

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3
Q

What is the treatment of PMR? Are relapses common?

A

20mg prednisolone for 1 month, slow taper to achieve lowest dose to keep symptoms at bay, may require 1-2 years of treatment, relapses are common.

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4
Q

True/False: PMR often presents with muscle weakness or tenderness.

A

False: PMR does not have objective muscle weakness or tenderness.

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5
Q

True/False: PMR is more likely in a patient < 50yrs.

A

False.

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6
Q

True/False: synovitis is less likely in PMR

A

True.

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7
Q

What are 3 important auto-antibodies in the diagnosis of anti-phospholipid syndrome? What is the regimen for detecting these Abs?

A
  1. Anti-cardiolipin Abs
  2. Lupus anticoagulant
  3. Anti-beta-2 glycoprotein 1 (AB2GP) Abs

For Dx; Abs need to be positive on 2 occasions > 12 weeks apart.

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8
Q

Patient has 2 week history of lower limb rash associated with arthralgia. On examination you note palpable purpura in both legs. Diagnosis?

A

Henoch-Schonlein purpura.

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9
Q

What does the biopsy reveal in Henoch-Schlonlein purpura?

A

Fibrinoid necrosis and neutrophils within the walls of dermal capillaries.

Vasculitis affects mostly postcapillary venules within the papillary dermis with inflammatory infiltrate (neutrophils and monoctyes)

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10
Q

What does immunofluorescence of a Henoch-Schlonlein purpura (HSP) biopsy of vessel walls reveal (3)? Which part of this differentiates it as a diagnosis from other forms of vasculitis?

A

FAC:

  1. Fibrin
  2. IgA - specific to HSP
  3. Complement C3
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11
Q

Patient has a rash, biopsy reveals neutrophils within the walls of medium sized arteries in the subcutis. Likely diagnosis?

A

Vasculitis of medium-sized vessels.

Most likely PAN (polyarteritis nodosa).

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12
Q

Whilst walking downstair a patient with OA will likely experience pain in which part of the body?

A

Patella-femoral compartment.

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13
Q

Aside from ESR and CRP which other sign of inflammation correlates well with disease activity in RA?

A

Platelet - thrombocytosis, acute inflammatory marker.

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14
Q

How do you differentiate between lower limb claudication due to peripheral vascular disease vs. spinal canal stenosis?

A

In spinal canal stenosis pain is improved with walking downstairs or leaning forward (relieves spinal pressure).

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15
Q

What major muscle groups are implicated in the Trendelenberg test?

A

Remember rhyme:
‘Tensor fascia latae, glut med and min
ABduct the thigh and rotate it in’

Main thigh ABductor is gluteus medius

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16
Q

Explain the Trendelenberg test/sign.

A

Test for weakness in the hip ABductors.

When walking - higher hip is the abnormal hip.

When standing on the abnormal hip it is higher, and the normal (contralateral) side sags.

17
Q

What are the clinical features of inclusion body myositis (IBM)?

Compare this to dermatomyositis (DM) and polymyositis (PM)

A

DISTAL myopathy with atrophy in quadriceps.

DM and PM cause PROXIMAL weakness.

18
Q

What is inclusion body myositis?

Which sex does it affect most?

A

Inflammation of the ‘endomysium’.

Affect mostly men.

Endomysium is the areolar connective tissue that ensheaths each muscle fibre/cell. Includes blood vessels, nerves and lymphatics.

19
Q

What are the biochemical and muscle biopsy findings of IBM?

A

CK normal (or mildly raised)

Muscle Bx: inclusion bodies (obviously)

20
Q

What is the treatment of IBM?

A

Prednisone + immunosuppressant (MMF / cytotoxics / AZA / Rituximab)

21
Q

What is fibromyalgia?

What are the examination findings?

What is the CRP and ESR?

How is it diagnosed?

A

Non-inflammatory chronic pain syndrome with generalised pain and no diurnal variation.

Reproducible muscle and joint pain.

Normal CRP/ESR.

Clinical diagnosis - no specific investigations.

22
Q

What is Sjogren’s syndrome?

What is the presentation?

A

Chronic inflammatory autoimmune disorder that is characterised by Sicca complex (decreased lacrimal and salivary gland secretion)

Presents with fatigue, dry eyes (keratoconjunctivitis Sicca) and dry mouth (xerostomia)

23
Q

Which 4 blood tests aid the diagnosis of Sjogrens syndrome?

A

S-SEA!

‘Sjogren went to Sea, Sea, Sea’

Positive:
SSA/Ro
SSA/La
ENA
ANA
24
Q

What is the treatment of Sjogren’s syndrome?

A

Mainly symptomatic.

25
Q

Patient has gout, which is the BEST antihypertensive to use?

A

‘GO LO! = GOut LOsartan’

Losartan has hypouricaemic properties.

26
Q

What is Prof. Barnsley’s approach to interpreting rheumatological X-rays?

A

JABS:

Joint space and margins: chondrocalniosis, erosions, osteopenia, sclerosis

Alignment

Bones: normal, thick, thin, trabecular pattern

Soft tissue: swollen, calcification, tophus

27
Q

Which joints in the hands are spared in RA?

A

DIP

28
Q

What is the most specific test for RA?

What is the sensitivity for this test?

Is it a useful prognostic marker

A

anti-CCP (anti-cyclic citrullinated peptide_ has 98% specificity.

Poor sensitivity - 50%

Predicts rapid disease and risk of bony erosions.

29
Q

What are the ‘juxta-articular’ changes on X-ray in RA?

A

Narrowed joint space
Osteopenia
Erosion

30
Q

Which of the following are contraindications to starting TNFi treatment:

  1. Treated Hep C
  2. NYHA Class IV
  3. Multiple Sclerosis
  4. Latent TB treatment 1 yr ago
  5. Breast cancer treated 10 yrs ago
A

Contraindications:

Multiple sclerosis: TNFi may cause demyelination and irreversible neurological damage.

CCF:
infliximab contraindicated (or must be used at low doses)
etanercept may be used

The rest are NOT contraindications.

31
Q

Which rheumatological disorder is Reynaud’s phenomena is most closely associated with?

A

Scleroderma

32
Q

Give 4 abnormal X-ray findings for Gout.

What 2 joint features are preserved?

A

‘STOP JaB’:

  1. Subchondral cysts
  2. Tophi
  3. Overhanging edges
  4. Punched-out biopsies

Preserved:

  1. Joint space
  2. Bone (no periarticular osteopenia)
33
Q

What is the prototypical form of IgG4-related disease?

A

Autoimmune pancreatitis.

34
Q

True/False: IgG4 related disease often implicates joints.

A

False.

Ironic that rheumatologists look after this disorder.

35
Q

What are typical biochemical and biopsy findings of a patient with IgG4 related disease?

A

Biochemistry: elevated serum levels of IgG4

Biopsy: infiltration of IgG4 bearing plasma cells in organ.

36
Q

Give 7 diseased that fall under IgG4 related diseases.

A

SPLARTS:

Sclerosing cholangitis (not PSC)
Pancreatitis (autoimmune)
Lung / Lacrimal (Mikulicz)
Aortitis
Retroperitoneal fibrosis
Thyroiditis (Reidels)
Salivary (Kuttners/Mikulicz)

FRACP (20 decks)

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