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MSK and Rheumatology > MSK and Rheum - SLE, Antiphospholipid Syndrome, Sjögrens Syndrome, Drug-Induced Lupus > Flashcards

MSK and Rheum - SLE, Antiphospholipid Syndrome, Sjögrens Syndrome, Drug-Induced Lupus Flashcards

1
Q

Systemic Lupus Erythematosus (SLE) - what is it?

A

Inflammatory multi-system autoimmune condition affecting connective tissue

Manifests differently in different patients

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2
Q

SLE - What does the erythematosus in Systemic lupus erythematosus refer to?

A

Refers to the typical red malar rash that occurs across the face

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3
Q

SLE - how does it present in terms of time period?

A

RELAPSING-REMITTING COURSE, with flares and periods where symptoms are improved

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4
Q

SLE - pathophysiology

A
  1. SLE is characterised by anti-nuclear antibodies
  2. These are antibodies to proteins within the persons own cell nucleus
  3. This causes the immune system to target theses proteins
  4. When the immune system is activated by these antibodies targeting proteins in the cell nucleus it generates an inflammatory response

TYPE 3 HYPERSENSITIVITY REACTION

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5
Q

SLE - what are flare ups/flares triggered by?

A

Oestrogen-containing contraception

Overexposure to sunlight

Infections

Stress

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6
Q

SLE - what is the classical presentation?

A

Some combination of:
1. Systemic upset (fever / myalgia / fatigue / weight loss)

  1. Joint and/or skin involvement
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7
Q

SLE - what are the dermatological presentations?

A

Photosensitivity - most common dermatological feature of SLE, occurs in form of a malar rash

Mouth / nose / genital ulcers

Cutaneous vasculitis - which manifests as splinter haemorrhages / purpura

Discoid rash

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8
Q

SLE - what are the CV presentations?

A

Pericarditis, which is the MOST COMMON cardiac feature of SLE

Myocarditis, which can present as arrhythmias and heart failure

Raynaud’s phenomenon

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9
Q

SLE - what are the respiratory issues that can occur?

A

Pleurisy

Pleural effusions

Pneumonitis - in the acute form can mimic pneumonia, chronic pneumonitis causes pulmonary fibrosis

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10
Q

SLE - what renal manifestations can occur?

A

LUPUS NEPHRITIS - most common cause of lupus-related death

Can be asymptomatic, before it presents as either nephritic or nephrotic syndrome

Patients should be monitored regularly for renal disease

Monitoring includes checking BP (sudden hypertension) and urinalysis (for proteinuria or haematuria)

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11
Q

SLE - what are the neurological manifestations?

A

Seizures

Migraines

Peripheral neuropathies

Psychiatric symptoms including psychosis or depression or anxiety

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12
Q

SLE - what are the haematological presentations?

A

Anaemia of chronic disease (common)

Lymphopenia (common)

Autoimmune haemolytic anaemia

Thrombocytopenia

Leucopoenia

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13
Q

SLE - what are the GI manifestations that can occur?

A

Peritonitis (aseptic)

Hepatosplenomegaly

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14
Q

SLE - what are conditions that can develop

A

Secondary Sjogren’s syndrome

Secondary anti-phospholipid syndrome

Mixed connective tissue disease

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15
Q

SLE - what antibodies are associated with SLE?

A

Antinuclear antibodies (ANA) - 99% are positive, high sensitivity makes it a useful rule out test, but has low specificity

20% are rheumatoid factor (Rf) positive

anti-dsDNA: highly specific (> 99%), but less sensitive (70%)

anti-Smith: highly specific (> 99%), sensitivity (30%)

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16
Q

SLE - how do you diagnose?

A

Use SLICC Criteria

Involves confirming the presence of antinuclear antibodies (ANA) and establishing ≥4 findings with at least one clinical feature and one laboratory test (like ANA or anti-dsDNA)

17
Q

SLE - what are the preventative steps a patient can take?

A

Avoid triggers for flares so:

Avoid oestrogen-containing contraception

Avoid overexposure to sunlight

Infections

Stress

18
Q

SLE - what is the pharmacological management?

A

No cure, immunosuppression is the mainstay of treatment, aim to reduce symptoms

1st line - HYDROXYCHLOROQUINE and NSAIDs, for MILD LUPUS

2nd line - Commonly used immunosuppressants in resistant or more severe lupus: Methotrexate, Mycophenolate mofetil, Azathioprine,Tacrolimus, Leflunomide, Ciclosporin

Biological therapies - severe disease or not responded to other treatments, Rituximab, Belimumab

19
Q

Antiphospholipid syndrome - what is it?

A

Autoimmune disorder characterised by blood becoming more prone to clotting and raised antiphospholipid antibodies

Patient is in a hyper-coagulable state

20
Q

Antiphospholipid syndrome - what are the main features? CLOT

A

C - Clots: usually venous (DVT and PE), can also be arterial (stroke and MI)

L - Livedo Reticularis: mottled, lace-like appearance of the skin on the lower limbs

O - Obstetric loss: recurrent miscarriages, pre-eclampsia, stillbirth

T - Thrombocytopenia

21
Q

Antiphospholipid syndrome - what are the associated antibodies and what investigation do you have to do?

A

One or more of the following positive blood tests are needed on 2 occasions, 12 WEEKS APART to diagnose APS:

  1. Anti-cardiolipin antibodies (aCL)
  2. Anti-beta-2 glycoprotein I antibodies
  3. Positive lupus anticoagulant assay (LA)
22
Q

Antiphospholipid syndrome - management

A

Reduce RF’s for thromboembolism
Venous - Avoid oestrogen-containing contraception/HRT and long periods of immobility

Arterial - Control CVS risk factors, BP, BM, smoking

Treatment of VTE:

  1. Not recommended prophylactically in patients that have not experienced a thromboembolic event
  2. If VTE event, lifelong warfarin / NOAC
  3. Warfarin is teratogenic, so use LMWH in pregnancy
23
Q

Antiphospholipid syndrome - passmed’s ‘key point for the exam’ about APTT, and what is APTT?

A

APTT measures how long it takes your blood to form a clot

‘A key point for the exam is to appreciate that antiphospholipid syndrome causes a paradoxical rise in the APTT. This is due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade’

24
Q

Sjögrens syndrome - what is it?

A

Autoimmune disorder affecting exocrine glands, causing dry mucosal membranes

25
Q

Sjögrens syndrome - what are the two different types of Sjögrens syndrome you can get?

A

Primary

Secondary

26
Q

Sjögrens syndrome - what is Primary Sjögrens syndrome?

A

Condition occurs in isolation

27
Q

Sjögrens syndrome - what is Secondary Sjögrens syndrome?

A

Occurs related other autoimmune conditions like SLE or rheumatoid arthritis

28
Q

Sjögrens syndrome - what are the clinical features?

A

Dry eyes: keratoconjunctivitis sicca (dryness of the conjunctiva)

Dry mouth

Vaginal dryness

Arthralgia

Raynaud’s

Myalgia

29
Q

Sjögrens syndrome - what antibodies are associated and are tested for?

A
  1. anti-Ro (SSA) antibodies in 70% of patients
  2. anti-La (SSB) antibodies in 30% of patients
  3. Rheumatoid Factor (RF) +ve in 50% of patients
  4. ANA positive in 70%
30
Q

Sjögrens syndrome - what other tests can you do?

A

Schirmer’s test - folded piece of filter paper under the lower eyelid with a strip hanging out over the eyelid is left in for 5 minutes

Distance along the strip hanging out that becomes moist is measured

Tears should travel 15mm in healthy adult
Result of LESS THAN 10mm is significant

31
Q

Sjögrens syndrome - management

A

Eyes: Artificial tears

Mouth: artificial saliva, pilocarpine may stimulate saliva production

Vagina: Vaginal lubricants

Hydroxychloroquine is used to halt the progression of the disease

32
Q

Sjögrens syndrome - what malignancy is there an increased risk of in Sjögrens syndrome?

A

There is a marked increased risk of lymphoid malignancy (40-60 fold)

33
Q

What are the types of hypersensitivity reactions?

A

Types of Hypersensitivity reactions - ACIDS

Type I - Anaphylactic

Type II - Cytotoxic

Type III- Immune Mediated

Type IV = Delayed

Type V = Stimulatory Antibodies (e.g. Grave’s, Myasthaenia Gravis)

34
Q

Drug-Induced Lupus - what is it?

A

Milder form of SLE, not all typical features present like in SLE, and it is triggered by chronic use of certain drugs for over 1 month

Symptoms should not be present before starting the drug, and should stop after discontinuing drug

35
Q

Drug-Induced Lupus - what are the clinical features?

A
  1. Systemic upset (fever / fatigue / myalgia).
  2. Arthritis (non-erosive) or arthralgia.
  3. Serositis in the form of PLEURISY or pericarditis
  4. Skin - malar rash

Nervous and renal system (SLE renal disease most common cause of death in SLE) involvement - RARE

36
Q

Drug-Induced Lupus - antibody test?

A

Anti-histone antibodies - found in 80-90%, specific for drug-induced lupus

ANA - positive in 100%

anti-dsDNA - NEGATIVE

MSK and Rheumatology (8 decks)

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