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BPT > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Catastrophic Antiphospholipid Syndrome

A

Rare, life threatening form of APS
Characterised by micro and macrovascular thrombosis of multiple organs that develop either simultaneously or in short succession

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2
Q

Mechanism of Obstetric Antiphospholipid Syndrome

A

Direct trophoblast inhibition

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3
Q

Overarching rule for Antiphospholipid Syndrome testing

A

Persistent positivity required

(positive result on two tests done at least 3 months apart)

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4
Q

When are the different antiphospholipid syndrome antibodies significant?

A

Cardiolipin >40
Anti-Beta2 Glycoprotein >40 or >99th percentile
Lupus Anticoagulant: positive

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5
Q

Risk stratification for Antiphospholipid Syndrome

A

High: Positive Lupus Anticoagulant, any Cardiolipin or Beta2-Glycoproteinresult

Moderate: Negative Lupus Anticoagulant, moderate to high Beta2 glycoprotein and/or Cardiolipin

Low: Negative Lupus Anticoagulant, low titre Cardiolipin and/or Beta2 glycoprotein

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6
Q

Who needs primary thromboprophylaxis in Antiphospholipid Syndrome?

A

High risk aPL profile, low risk aPL profile but have SLE, history of obstetric antiphospholipid syndrome

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7
Q

Management of Thrombosis in Antiphospholipid Syndrome

A

Warfarin indicated, DOAC inferior. Target INR 2.5-3.5, increasing to 3-4 if recurrent on therapy.

Lifelong if unprovoked, recurrent or high risk aPL profile, otherwise as per usual guidelines

Recurrence: lifelong, increase INR target, add Aspirin, consider change to LMWH

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8
Q

Management of Antiphospholipid Syndrome during pregnancy

A

High risk aPL but no history of thrombosis or pregnancy complications: Aspirin monotherapy

History of obstetric APS but no thrombosis (≥3 recurrent spontaneous miscarriages prior to 10 weeks, history of foetal loss)
- Aspirin + prophylactic dose heparin during pregnancy

History of thrombosis: therapeutic dose heparin, which needs to be continued for 6 months post partum

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9
Q

Diagnosis of Sjogren’s

A

Schirmer test <5mm wetting in 5 minutes = dryness
ANA, Ro/La (SSa/SSb)

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10
Q

Primary Lymphoma type associated with Sjogren Syndrome

A

Mucosal Associated Lymphoid Tissue Lymphoma

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11
Q

Clinical features of SJogren’s associated with higher lymphoma risk

A

Lymphadenopathy
Recurrent parotid swelling
Monoclonal gammopathy
Depressed C4
Decreased RF (if elevated at baseline)

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12
Q

What population needs additional testing prior to starting Allopurinol, and what testing is this?

A

Four South East Asian patients, need to check HLAB58*01

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13
Q

HLA associated with Behcet’s?

A

HLA B51

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14
Q

Etanercept

A

TNF Inhibitor

Shouldn’t be used for IBD

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15
Q

Infliximab

A

TNF Inhibitor

Risk of anti-drug antibodies

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16
Q

Adalimumab

A

TNF Inhibitor

Risk of anti drug antibodies

17
Q

Certolizumab

A

TNF Inhibitor

18
Q

Golimumab

A

TNF Inhibitor

19
Q

Anakinra

A

IL-1 Inhibitor

Less effective for inflammatory arthritis, so not used much. Can be used for gout, TRAPS, Familial Mediterranean Fever

20
Q

Tocilizumab

A

IL-6 Inhibitor

In GCA: Reduces flares by 40%, faster steroid wean.
Drops CRP drastically as IL-6 drives acute phase reactant production

21
Q

Secukinumab

A

IL-17 Inhibitor

Used for Psoriasis, PsA, AS, Axial Spondyloarthritis

22
Q

Ixekizumab

A

IL-17 Inhibitor

Only really used for PsA/Psoriasis

23
Q

Ustekinumab

A

IL-12/23 Inhibitor

Good for joints, less so skin

24
Q

Guselkumab

A

IL-23 Inhibitor

Good for joints, less so skin

25
Q

Abatacept

A

T cell costimulation blockade. Blocks CD28 from binding to CD80/86 → reduced Treg suppression and limitation of T effector cell activity

In a sense is a CTLA-4 analogue. SO downregulated T cell activity overall.

Similar efficacy to TNF Inhibitors in RA

26
Q

Rituxmab

A

Anti CD20 → depletes B cells, blocks formation of new antibodies

Can cause hypogammaglobulinaemia

27
Q

Belimumab

A

Inhibits B cell activation

Can be used in Lupus

28
Q

Tofacitinib

A

JAK Inhibitor: Reduced DNA transcription in lymphocytes

High risk of cardiovascular disease, stroke, VTE, cancer in older patients and smokers

29
Q

Upadacitinib

A

JAK Inhibitor: Reduced DNA transcription in lymphocytes

High risk of cardiovascular disease, stroke, VTE, cancer in older patients and smokers

30
Q

Baricitinib

A

JAK Inhibitor: Reduced DNA transcription in lymphocytes

High risk of cardiovascular disease, stroke, VTE, cancer in older patients and smokers

31
Q

Lofgren Syndrome

A

Pathognomic presentation of sarcoid

Fever plus triad of:
- Migratory polyarthritis
- Erythema nodosum
- Bilateral hilar lymphadenopathy

70-80% experience spontaneous remission, and biopsy is not required to confirm diagnosis

32
Q

Biopsy in Sarcoid

A

Gold standard for diagnosis. Skin lesions a good option as easily accessible

Noncaseating granulomas containing
- Giant cells
- Asteroid bodies
- Schaumann bodies

33
Q

Indications for Immunosuppression in Sarcoidosis

A
  • Symptomatic, progressive disease
  • Persistent pulmonary infiltrates
  • Progressive decline in lung function
34
Q

Sarcoidosis staging

A
  1. Bilateral Hilar Lymphadenopathy, no pulmonary infiltrates
  2. Pulmonary infiltrates, No Bilateral Hilar lymphadenopathy
  3. Both bilateral hilar lymphadenopathy and pulmonary infiltrates
  4. Substantial pulmonary fibrosis
35
Q

Sarcoidosis Pathology

A

Granulomas produced following macrophage activation → Th1 cell activation → IFN-gamma release → epithelioid cell and multinucleated giant cell formation

Epithelioid cells produce ACE and recruit fibroblasts leading to fibrosis

Alveolar macrophages increase calcitriol leading to hyperphosphataemia, hypercalcaemia, renal failure. Driven by 1-alpha hydroxylase

36
Q

Mitochondrial Myopathy

A

Maternal inheritance

External ophthalmoplegia, Ptosis, exertional muscle weakness. However also subtypes such as MELAS (encephalopathy, myopathy, lactic acidosis, stroke like episodes)

Lactic acidosis, CK normal

Muscle Biopsy shows: Ragged red fibres but no inflammatory cells or necrosis

37
Q
A

BPT (16 decks)

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