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Stage 2: Inflammation > Systemic Autoimmune Rheumatic Diseases > Flashcards

Systemic Autoimmune Rheumatic Diseases Flashcards

1
Q

What is antiphospholipid syndrome characterised by?

A

1) Thrombosis (clots) e.g. VTE

2) Pregnancy morbidity (miscarriage and neonatal death)

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2
Q

What are the three autoantibodies that are drivers of aPL syndrome?

A

1) Anti-cardiolipin (aCL)
2) Lupus anticoagulant (LA)
3) Anti-beta2 glycoprotein 1

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3
Q

What are the 3 Sydney classification criteria for aPL syndrome?

A

1) One or more arterial, venous or small vessel thrombosis
2) ≥1 fetal death ≥ 10 weeks
≥ 1 premature birth < 34 weeks
≥ 3 spontaneous abortions < 10 weeks
3) aCL, beta2GP1 or LA positive at least twice > 12 weeks apart

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4
Q

What increases the risk of having an aPL event?

A

If you are triple positive for all 3 antibodies

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5
Q

Why should all new lupus patients have a test for the aPL antibodies?

A

To figure out the risk of thrombosis in the future

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6
Q

What % of lupus patients have aPL?

A

30%

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7
Q

What are the risk factors for aPL and thrombosis?

A
  • Surgery
  • Immobilisation
  • Hyperlipidaemia
  • Hypertension
  • Obesity
  • Oestrogens
  • Pregnancy/puerperium
  • Cox-2 selective inhibitors
  • Long distance travels
  • 50% of APS + thrombosis ≥ 1 risk factor
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8
Q

What can occur in the brain in aPL?

A
  • Multiple strokes
  • Haemorrhage
  • Death
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9
Q

How is cerebral APS different from cerebral lupus (difficult to distinguish)?

A

Cerebral APS mainly consists of cerebral events whereas cerebral lupus is more hallucinations, seizures etc

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10
Q

What are the clinical features of cerebral APS?

A
  • Headache
  • Cognitive dysfunction
  • Ischaemic stroke
  • TIA
  • Acute ischaemic encephalopathy
  • Amaurosis fugax
  • Cerebral venous thrombosis
  • Chorea
  • Transverse myelitis
  • Seizures
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11
Q

How do you treat CNS lupus with aPL?

A

Anticoagulation for strokes/TIAs

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12
Q

What are consequences of aPL in pregnancy?

A
  • Uteroplacental insufficiency and IUGR - fetal death and still birth
  • Pre-eclampsia
  • Abruption
  • Premature delivery
  • Thrombosis
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13
Q

How would you treat someone who is aPL positive and pregnant but hasn’t had any thrombosis or pregnancy loss?

A
  • Careful monitoring

- Aspirin?

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14
Q

How would you treat someone who is aPL positive and pregnant who has had previous maternal thrombosis, recurrent embryonic loss < 10 weeks, late fetal death, IUGR or severe pre-eclampsia?

A

Heparin + aspirin

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15
Q

How do you treat someone with aPL postpartum?

A

Heparin for 6 weeks bc pregnancy is a high thrombotic state

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16
Q

How do you treat aPL patients who have had thrombosis and what is the target INR?

A
  • Long term warfarin
  • Target INR for venous thrombosis (DVT/PE) = 2-3
  • Target INR for arterial thrombosis (stroke, MI, recurrent events) = 3-4
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17
Q

What are the features of primary Raynaud’s?

A
  • Common
  • Young women affected
  • Symmetrical
  • Doesn’t involve thumbs
  • Doesn’t involve antibodies (can be discharged)
  • Due to spasm of arteries causing reduced blood flow
18
Q

What are features of secondary Raynaud’s?

A
  • Asymmetrical
  • Thumbs implicated
  • Macroscopic nailfold capillary loops
  • Digital infarcts
  • Autoantibodies
  • 95% predictive of CTD (SLE, systemic sclerosis)
  • Need more investigation and follow up
19
Q

What feature is pathognomonic of autoimmune Raynaud’s (disease)?

A

Nailfold capillaries

20
Q

Describe nailfold capillaries

A
  • Regular distribution of capillaries without pathological enlargements
  • Giant capillaries
  • Loss of capillaries
  • Haemorrhages
  • Neoangiogenesis
21
Q

How is (secondary) Raynaud’s treated?

A
  • Keep warm e.g. heated gloves
  • Calcium channel blockers e.g. nifedipine
  • Sildenafil
  • Prostacyclin infusions
  • Bosentan
  • (Sympathectomy)
22
Q

What antibodies are present in diffuse cutaneous systemic sclerosis?

A
  • Anti-Scl 70 - DNA topoisomerase I

- Anti-RNA polymerase I, III

23
Q

What antibodies are present in cutaneous systemic sclerosis?

A

Anti-centromere

24
Q

What are features of systemic sclerosis?

A

1) Early diffuse disease < 2 years
2) Rapid cutaneous progression
3) Renal crisis
4) GI involvement (diverticular disease, peritonitis due to perforation, fibrosis)
5) Early interstitial lung disease (pulmonary fibrosis)
6) Pulmonary hypertension
7) Microscopic nail folds (nailfold capillaroscopy)
8) Systemic (sclero)dactyly (shiny, fibrosed, tight skin)
9) Raynaud’s
10) Telangiectasia
11) Digital ulceration bc blood vessels are not supplying the digits with sufficient blood leading to areas of ischaemia

25
Q

Describe renal disease in systemic sclerosis?

A
  • Present with severe hypertension
  • Vasculopathy
  • Close down renal vasculature
  • Onion skinning
  • Can go from no kidney problem to dialysis in 10 days
26
Q

Which group of systemic sclerosis patients have must more common pulmonary hypertension?

A
  • CREST syndrome

- Limited cutaneous systemic sclerosis

27
Q

What is systemic sclerosis?

A

Autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, (fibrosis) in the skin and internal organs and by injuries to small arteries

28
Q

How do you treat systemic sclerosis?

A
  • Early = low dose steroids, immunosuppressives

- Late = symptomatic treatments, BP control

29
Q

What are the 4 inflammatory myopathies?

A

1) Dermatomyositis
2) Polymyositis
3) Cancer associated myositis
4) Inclusion body myositis

30
Q

What are the symptoms/features of inflammatory myopathies (similar to lupus)?

A

1) Muscle weakness e.g. shoulder/neck flexor weakness - intense inflammation and necrosis of muscle fibres, CKA low
2) Raynaud’s
3) Joint pains
4) Fevers
5) Rashes
(Skin biopsy looks like lupus)

31
Q

Describe the myositis autoantibodies

A
  • MDA5 - life threatening lung disease, skin necrosis
  • HMGCR - statin induced myopathy
  • In a patient in 70s with TIF1 gamma antibody, search hard for malignancy (can be skin disease as well)
32
Q

Describe ANCA vasculitis (granulomatous polyangiitis)

A
  • Anti-neutrophil cytoplasmic antibodies
  • Pulmonary, skin and nerve involvement
  • Lung or kidney disease tends to evolve over a month
  • May present with sensory hearing loss
  • May get walking foot drip due to nerve inflammation
  • Cough w pneumonia CXR but abx don’t work
  • Haemoptysis, cavity in lungs, infiltrates spread to both lungs and acute renal failure
  • Treatment = cyclophosphamide (aggressive immunosuppression)
33
Q

What are other vasculitides?

A
  • Behcet’s
  • Takayasu’s
  • Polymyalgia/giant cell arteritis
34
Q

Describe relapsing polychondritis

A
  • Immune response against cartilage leading to inflammation of cartilaginous structures
  • Present with painful and episodic red eats
  • Problems with coughing, weak voice, stridor and trouble breathing
  • Ears and airways become floppy
  • May need lung transplant if airways can no longer sustain the pressures
35
Q

Describe sarcoid

A

Can manifest in almost any organ system e.g. skin, eye, brain, lungs, heart, kidneys and can mimic many other diseases
- Granulomas develop in organs

36
Q

What is a systemic illness involving fevers?

A

Periodic fever syndromes

37
Q

What are the key symptoms of Sjogren’s syndrome?

A
  • Sicca - dryness of exocrine glands leading to dry eyes, mouth, skin and vagina
  • Swelling of salivary glands (between jaw and ears)
  • Symptoms of lupus - fatigue, muscle/joint pain, photosensitive rash
38
Q

What are pulmonary symptoms of CT disease?

A

Chest pain and dyspnoea

39
Q

What are symptoms of vasculitides?

A
  • Mouth/genital ulcers
  • Splinter haemorrhages (ANCA)
  • Skin rash
  • Eyes
  • Sinusitis
  • Abdominal pain
  • Respiratory symptoms incl. haemoptysis
  • Systemic symptoms
  • Neurological symptoms
40
Q

What two autoimmune diseases occur in older people > 50 and not in younger people (unlike all other autoimmune diseases? (Linked to IL-6 and CRP which increase with ageing)

A

1) Polymyalgia rheumatica
2) Giant cell (temporal) arteritis (large vessel vasculitis)
(Common to have both of these)

41
Q

What are symptoms of giant cell arteritis?

A
  • Frequent, severe headaches
  • Pain and tenderness over the temples
  • Jaw pain while eating or talking
  • Vision problems (risk of vision loss)
  • Treat with steroids
42
Q

What are symptoms of polymyalgia rheumatica?

A
  • Morning stiffness lasting > 45 mins
  • Pain, stiffness and inflammation in the muscles around the shoulders, neck and hips (thighs)
  • High CRP and ESR (inflammatory markers)
  • Treat with prednisolone

Stage 2: Inflammation (39 decks)

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