Rheumatology Flashcards

1
Q

What is the most commonly affected joint in pseudogout?

A

Knee

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2
Q

What are the common clinical signs of an inflammatory arthritis

A

Hot, swollen, tender joints with reduced ROM and loss of function

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3
Q

What antibodies are present in Sjogrens syndrome that are specific for the disease

A

Anti- Ro (anti SSA) and Anti-La (anti SSB)

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4
Q

What is the typical presentation that would warrant further investigation for Sjogren syndrome

A

Xerostomia (dry mouth) and Xeropthalmia (dry eyes) - combination is known as sicca. Patients often complain of having gritty eyes and difficulty swallowing from reduced saliva

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5
Q

What disease state are people with Sjogrens syndrome at higher rates of developing

A

Non-Hodgkins Lymphoma (MALT)

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6
Q

What antibody has high specificity for RA

A

Anti CCP

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7
Q

Variations in which gene are strongly associated with RA

A

HLA-DRB1 (has alleles that display the ‘shared epitope’)

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8
Q

What environmental triggers are strongly assosciated with RA

A

Lung - smoking/silica exposure; Mouth - P gingivalis bacterial disease; Gut - factors that alter the microbiome including sugary drinks and repeated exposure to ABx

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9
Q

What is the first line DMARD that should be used for RA

A

Methotrexate

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10
Q

In a gout flare, should you increase the dose of allopurinol

A

No - continue allopurinol at the current dose and introduce a short term anti-inflammatory drug (NSAID, colchicine, IL1 antagonist). Altering the dose of allopurinol has the risk of sudden changes in serum urate levels which can trigger/propagate gout flares

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11
Q

What is the most common site of venous and arterial thrombosis in antiphospholipid syndrome?

A

Venous - Deep veins of lower extremities
Arterial - Cerebral circulation

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12
Q

What are the obstetric complications of antiphospholipid syndrome?

A
  • Recurrent early (<10 weeks) miscarriages
  • Foetal demise beyond 10 weeks
  • Premature births ( <34 weeks) associated with eclampsia/preeclampsia or placental insufficiency
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13
Q

Which rheumatological disorder is most commonly associated with antiphohpholipid syndrome?

A

SLE

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14
Q

What is catastrophic APS (CAPS)?

A

Catastrophic APS is a life-threatening progressive macro and microvascular thromboembolic disease involving 3 or more organs simultaneously or over a short time course (occurs in 1% of people with APS)

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15
Q

What are the risk factors for catastrophic APS?

A

Risk factor for CAPS: Infection, surgery, cancer, estrogen use, pregnancy/postpartum, active SLE

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16
Q

What are the diagnostic criteria for antiphospholipid syndrome?

A

Diagnosis of APS requires presence of at least one clinical and one lab criteria.

Clinical criteria
- Unprovoked venous, arterial or small vessel thrombotic events
- Obstetric complications - early miscarriage (<10 weeks), foetal loss after 10 weeks gestation, premature birth (< 34 weeks) from pre-eclampsia or placental insufficiency

Lab criteria: Positive aPL abs on 2 separate occasions a least 12 weeks apart
- Lupus anticoagulant
- Anticardiolipin abs (IgG and M)
- Beta2glycoprotein I abs (IgG and M)

17
Q

What are the diagnostic criteria for catastrophic APS?

A

Diagnosis of CAPS
- Involvement of ≥3 organs, systems, or tissues
- Manifestations develop simultaneously or over <1 week
- Small vessel occlusion is confirmed histologically in at least one organ or tissue
- Presence of aPL (anticardiolipin antibodies, anti-beta2-glycoprotein I antibodies, and/or lupus anticoagulant) is documented twice, at least 12 weeks apart

18
Q

What are the thrombotic manifestations of antiphospholipid syndrome?

A

Venous thrombosis:
- DVT (most common 39%)
- PE
- Livedo reticularis - Blood clots in capillaries lead to swelling of venules, resulting in a purplish, net-like discoloration of the skin.
Arterial thrombosis:
- Stroke and TIA (most common 20%)
- Occlusion of organ arteries (e.g. MI, retina, renal)
- Occlusion of arteries supplying extremities (ischemia and gangrene)
Capillary thrombosis:
- Splinter haemorrhages

19
Q

What are the non thrombotic manifestations of antiphospholipid syndrome?

A

Valvular heart disease and cognitive decline

20
Q

What are the three antibodies that are included in the diagnostic criteria for APS? What do FBC and coagulation profile show in APS?

A

Lupus anticoagulant
Anticardiolipin Ab (IgG or M)
Beta2glycoprotein1 Ab (IgG or M)

FBC: thrombocytopenia, haemolytic anaemia (autoimmune or microangiopathic)
Coagulation: prolonged aPTT

21
Q

Which infectious disease do patients with APS often test falsely positive for?

A

Syphilis (the test to detect it uses cardiolipin antigen)

22
Q

What is the treatment of antiphospholipid syndrome?

A

Primary prevention: for patients with aPL abs but no thrombotic event or obstetric complication - low dose aspirin

Secondary prevention: lifelong anticoagulation with warfarin. For venous thrombosis, warfarin (INR target 2-3), for arterial warfarin (INR 2-3) and low dose aspirin (75-100 mg). Recurrent thrombosis despite adequate INR – switch to LMWH, change INR target to higher, add aspirin or hydroxycholorquine

  • Obstetric APS – previous history APS obstetric complication – prophylactic dose LMWH and low dose aspirin recommended; thrombotic APS – therapeutic LMWH + low dose aspirin should be administered. For recurrent obstetric complications despite above treatment consider IVIG and hydroxychloroquine. Warfarin is teratogenic and contraindicated in pregnancy. Warfarin therapy recommended post partum in patients with risk of recurrent thrombosis.
23
Q

What is the treatment for catastrophic APS?

A

Combination therapy with glucocorticoids, heparin, and plasma exchange or IVIG together with appropriate management of triggering events such as infections. For refractory CAPS, B-cell depletion (e.g., with rituximab) or complement inhibition (e.g., with eculizumab) therapies are alternative options.

24
Q

What are the characteristic histopathological features of IgG4 disease?

A
  • Lymphoplasmacytic tissue infiltration of mainly IgG4-positive plasma cells and lymphocytes
  • Storiform fibrosis
  • Obliterative phlebitis
  • Modest tissue eosinophilia
25
Q

Which is the driving cell of the pathogenesis in IgG4 related disease?

A

CD4+ T cell

26
Q

What are the common clinical manifestations associated with IgG4 disease?

A
  1. Autoimmune pancreatitis and sclerosing cholangitis
  2. Mikulicz syndrome - abnormal enlargement of glands in head and neck - commonly lacrimal and parotid
  3. Retroperitoneal fibrosis
    ( see full list in A4 summary)
27
Q

What is the treatment of IgG4 related disease?

A
  • First line therapy – high dose glucocorticoids usually 0.5-0.6 mg/kg/day for 2-4 weeks with slow taper over 6 months
  • For relapsing or steroid resistant disease - rituximab is second line
  • Methotrexate may be used to maintain remission and azathioprine and mycophenolate mofetil have been used as steroid sparing agents
28
Q

RACP 2021 October Q4

  1. 20 year old female presents with poor dentition and bilateral parotid swelling. What is the most likely diagnosis?

a. sarcoidosis
b. Sjogrens
c. Lupus
d. Eating disorder

A
29
Q

RACP 2021 October Q15

  1. What antibodies are present in GPA?

A. c anca +ve /p anca -ve / Pr3 +ve /MPO -ve
B. c anca +ve / P anca -ve / Pr3 -ve/MPO +ve
C. c anca -ve/ p anca +ve / Pr3 +/ MPO -
D. c ance -ve/ P anca +ve / Pr3 - / MPO +

A
30
Q

RACP 2021 Oct Q41

  1. Dabigatran affects testing of which lupus antibody procoagulant
    a. lupus anticoagulant
    b. beta 2 microglobulin
    c. anti cardiolipin
    d. anti thrombin
A
31
Q

RACP 2021 Oct Q53

  1. Patient with right face pain, palpable temporal artery. No jaw claudication.
    ESR 50, normal crp. PET and TAB normal. The eye exam shows Anterior ischemic
    optic neuropathy. Diagnosis?
    a. GCA
    b. small vessel vasculitis
A
32
Q

RACP 2021 Oct Q54

  1. Which of the following is medium size vasculitis?
    a. PAN
    b. GCA
    c. Takayasu
    d. Henoch scholein purpura
A
33
Q

RACP 2021 Oct Q61

36 y/o Lady c/o stiffness, lower back pain. Mother has history of rheumatoid. She is tender on both hips. What is the investigation of choice?

A. Rheumatoid factor/anti-ccp
b. Ultrasound of both hips
c. Lumbar spine x-Ray d.
d. Reassure

A
34
Q

RACP 2021 Oct Q62

Lupus pernio (Image) Long standing. Presents with recent onset lethargy,
dyspnea, palpitations. ECG non-sustained VT. What is the likely cause of cardiac
symptoms?
A. Giant cell myocarditis
b. Lyme disease
c. Sarcoidosis.

A