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Clinical lectures: Yr1 > Systemic Lupus Erythematosus > Flashcards

Systemic Lupus Erythematosus Flashcards

1
Q

What is the definition of SLE?

A

Chronic multisystem autoimmune disease characterised by autoantibody production and immune complex deposition, which leads to organ inflammation and ultimately organ damage

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2
Q

Why is SLE defined as a multisystem disease?

A

Many organs are affected at same time

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3
Q

Is the incidence and prevalence of SLE equal in men and women?

A

No, incidence and prevalence is much higher in women than men

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4
Q

What percentage of individuals with SLE are female?

A

90%

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5
Q

What is the peak age onset range for SLE?

A

20-30

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6
Q

Is the incidence of SLE equal in non-white and white ethnic populations?

A

No, incidence is higher in non-white ethnic populations

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7
Q

Give 3 examples of ethnic populations in which SLE incidence is high?

A

Afro-American
Hispanic
Asian

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8
Q

What is the prevalence percentage of SLE in Caucasians?

A

0.03%

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9
Q

What is the percentage of prevalence of SLE in Afro-Caribbeans?

A

0.2%

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10
Q

How does SLE affect the risk of CVD mortality compared to age and gender controls?

A

Risk of CVD mortality increase 5x more by SLE than other age or genetic controls

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11
Q

What type of siblings have higher chance of developing SLE?

A

Monozygotic twins

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12
Q

What is a polymorphic gene?

A

Gene that has 2 or more forms when variation occurs in the population

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13
Q

Give one example of polymorphism that causes genetic predisposition to SLE?

A

Polymorphic variants at HLA gene locus

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14
Q

What 3 complement proteins can increase chance of SLE when they have inherited mutations?

A

C1q, C2,C4

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15
Q

What part of the immunoglobulin receptor can increase chance of SLE when affected by an inherited mutation?

A

FcγRIIIb

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16
Q

What enzyme can increase chance of SLE when affected by an inherited mutation?

A

TREX1 (DNA endonuclease)

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17
Q

What do most of the polymorphic genes of SLE express?

A

Regulation of immune cell function

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18
Q

How can poor apoptosis cause autoantibody production in SLE?

A

Poor apoptosis means that not all antigens are destroyed, which activates B-cells and T-cells, this stimulates autoantibody production

Autoantibodies seem to target antigens in the nucleus or in cell

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19
Q

In SLE, are the symptoms always the same in a patient at any given time?

A

No, SLE can cause flares of varying intensity, duration, frequency and can affect any organ

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20
Q

Give 5 examples of systemic features of SLE?

A

Weight loss
Arthralgia
Fever
Fatigue
Mild lymphadenopathy

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21
Q

What is lymphadenopathy?

A

Disease activity in lymph nodes

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22
Q

In what percentage of SLE cases does arthralgia occur?

A

90%

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23
Q

What are the 2 common symptoms in which arthralgia is presented, in SLE cases?

A

EMS

Tendosynovitis (inflammation of tendon sheath lining)

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24
Q

In SLE, how can tendosynovitis caused by arthralgia progress if untreated?

A

Causes tendon damage

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25
Q

Give 2 examples of rare symptoms in which arthralgia presents, that is caused by SLE?

A

Synovitis

Jaccoud’s arthropathy

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26
Q

What is Raynaud’s phenomenon, and explain what each colour change?

A

Improper blood circulation to extremities, where skin colour change indicates state of blood flow and occurs in response to cold, stress, anxiety

skin turns white when there is restricted blood flow, blue when vessels dilate, red when blood flows to extremities

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27
Q

In SLE cases, if examination of Raynaud’s phenomenon shows a colour change, what condition does this indicate?

A

Chronic ischaemia

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28
Q

In SLE cases, how does Raynaud’s phenomenon affect capillary nail loop patterns?

A

Loss of normal capillary nail loop patterns

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29
Q

In severe SLE cases, what structure can Raynaud’s phenomenon cause to develop on digits?

A

Digit ulcers

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30
Q

Describe the onset of secondary Raynaud’s phenomenon in SLE?

A

Common, early symptom of SLE, usual onset occurs in males over 25 with no family history of Raynaud’s phenomenon

31
Q

Give 2 common symptoms of neural involvement in SLE?

A

Headaches, poor concentration

32
Q

Give 5 examples of uncommon symptoms of neural involvement in SLE?

A

Visual hallucinations
Chorea
Lymphocytic meningitis
Transverse myelitis
Organic psychosis

33
Q

What is chorea, and what does it indicate when presented in SLE cases?

A

Involuntary, irregular muscle movements that indicate neural involvement in SLE

34
Q

Give 2 common symptoms of GI involvement in SLE cases?

A

Mouth ulcers

Acute pain caused by peritoneal serositis (inflammation of peritoneum/inner abdomen lining)

35
Q

What are 2 severe, rare symptoms of GI involvement in SLE cases?

A

Mesenteric vasculitis (abdomen pain, bowel infarction/perforation)

Hepatitis

36
Q

If a patient with SLE has symptoms of renal disease, what does this indicate about the state of SLE progression?

A

Renal disease is a hallmark of severe SLE

37
Q

In severe SLE cases, what 2 tests are regularly carried out to monitor renal function?

A

Urinalysis

Blood pressure

38
Q

Give 4 common symptoms of renal disease in severe SLE cases?

A

Severe haematuria

Severe proteinuria

Proliferative glomerulonephritis

Urinary casts on urine microscopy

39
Q

What are urinary casts, and what can they indicate in a patient with SLE?

A

Urinary cast is a tube-like structure that forms in urine as the result of conditions, infections, or problems with renal function

Therefore indicates that SLE patient has renal disease

40
Q

What are the 3 common symptoms of heart disease in SLE cases?

A

Pericarditis

Myocarditis

Libman-Sacks endocarditis

41
Q

What is Libman-Sacks endocarditis, and what does it indicate when present in SLE cases?

A

Non-bacterial endocarditis that presents in SLE when there is cardiovascular involvement, specifically heart disease

42
Q

How does arterial involvement in SLE cases cause an overall increased risk of MI and stroke?

A

Causes greater incidence of atherosclerosis, which causes inflammatory disease in endothelial walls, long-term steroid therapy and is associated with antiphospholipid antibodies

These increase the overall risk

43
Q

Give 2 common symptoms of lung involvement in SLE cases?

A

Pleuritic pain due to serositis

Pleural effusions

44
Q

Give 4 uncommon symptoms of lung involvement in SLE cases?

A

Breathlessness due to pulmonary fibrosis

atelectasis (partial collapse/incomplete inflation of lung)

pneumonitis (lung inflammation)

reduced lung volume

45
Q

Give 2 thrombosis conditions that can arise due to lung involvement in SLE cases, and why?

A

Lung involvement increases risk of thromboembolism, so can cause DVT and PE

46
Q

What molecules when present increase the risk of thromboembolism, due to lung involvement in SLE?

A

Antiphospholipid antibodies

47
Q

How do autoantibodies cause haematological abnormalities, and give 4 examples?

A

Autoantibodies destroy peripheral blood cells

neutropenia
lymphopenia (this level reflects disease activity), thrombocytopenia
haemolytic anaemia

48
Q

What is antiphospholipid syndrome (ALS)?

A

Autoimmune disease that increases risk of vascular thrombosis/blood clots, and is associated with pregnancy complications such as early/late foetal loss

49
Q

What is the procedure for lab diagnosis of antiphospholipid syndrome (ALS), and what are the 3 tests?

A

At least one type of APS test must be positive on 2 occasions with at least 12 week/3 month gap between them

Lupus anticoagulant test
Anticardiolipin test
Anti-beta-2-glycoprotein 1 test

50
Q

What percentage of SLE patients develop the classic facial/malar rash, and describe its appearance?

A

Up to 20% patients

Erythematous, raised, itchy rash over cheeks and nose bridge, spares nasolabial regions

51
Q

What other type of rash tends to form on the face in SLE patients, other than the classic facial/malar rash?

A

Discoid rash: round, shiny, scaly (hyperkeratosis) sore that commonly develop on face and scalp (this leads to scarring alopecia)

52
Q

What 2 types of alopecia can develop in SLE patients?

A

Diffuse, non-scarring alopecia

Scarring alopecia that develops as result of discoid rash on scalp

53
Q

Give 3 common symptoms of skin involvement in SLE, other than facial rash, discoid rash and alopecia?

A

Urticaria/hives: raised, itchy welts

livedo reticularis

vasculitis

54
Q

What is the overall percentage of pregnancy loss that occurs in SLE, and what are the individual percentages of loss in first pregnancy and later pregnancies?

A

13.1% overall loss

6% first pregnancy

7.1% later pregnancies

55
Q

What percentage of pregnancies result in preterm birth, in SLE patients?

A

40.5%

56
Q

What is the pericardium?

A

Double-walled layer that surrounds heart

57
Q

Give 3 symptoms of SLE in infants (neonatal lupus)?

A

Pericardial effusion, conduction defects, skin rash

58
Q

Give 6 examples of areas that are affected by Raynaud’s phenomenon?

A

Fingers, toes, nipples, ears, nose, lips

59
Q

Give 3 symptoms of Raynaud’s phenomenon that a patient feels?

A

pain
numbness
pins and needles

60
Q

What are the 3 main target of SLE management?

A

Maintain normal function
Control symptoms
Prevent organ damage

61
Q

Give 2 examples of what SLE patients should avoid when managing disease?

A

Smoking

Sun exposure (use spf 50)

62
Q

What 2 factors are used to determine what drug therapies are used for SLE management?

A

Progression of SLE: mild, severe, end-organ disease

Type of organ involved

63
Q

Give 3 examples of medications that can be administered for mild active pain in skin and joints, when managing SLE?

A

Analgesics, NSAIDs and hydroxychloroquine

64
Q

Give 5 examples of medications that can be administered for severe active pain, when managing SLE?

A

prednisolone, MMF, MTX, azathioprine

belimumab (monoclonal antibody targets the β-cell growth factor BLyS)

65
Q

What medications are used for end-organ disease in SLE?

A

High-dose glucocorticoids and immunosuppressants

IV methylprednisolone (10 mg/kg IV) and IV cyclophosphamide for six cycles
SE: infection, hemorrhagic cystitis, infertility

Rituximab may be of benefit in some

66
Q

In maintenance therapy of SLE, how should prednisolone dosage be adjusted, and what is the duration of use?

A

Prednisolone tapered to low dose for long-term use

67
Q

Give 3 examples of immunosuppressants that are used for maintenance therapy of SLE?

A

Azathioprine, MMF, MTX

68
Q

If a patient with SLE has CVD involvement, what drug can be given and why?

A

Hydroxyquinone, as it is cardioprotective

69
Q

If a patient with SLE is at risk of thrombosis or ALS, what drug can be given and why?

A

Warfarin, as it is an anticoagulant

70
Q

What 2 factors should be checked when considering bone health of a patient with SLE?

A

Assess risk of osteoporosis and vitamin D deficiency

71
Q

Give 5 examples of how to manage Raynaud’s phenomenon?

A

keeping warm

preventing smoking

calcium channel blockers

iloprost and sildenafil (vasodilators)

72
Q

What type of blood test is highly sensitive but non-specific for lupus, and what percentage of lupus patients have a positive result?

A

Positive ANA test, which is found in 98% of lupus patients. Therefore it is highly sensitive but non-specific

73
Q

After positive ANA blood test, what other blood test is performed to aid lupus diagnosis as it is highly specific?

A

Anti-dsDNA blood test identifies anti-dsDNA antibodies in blood, which are highly specific markers of lupus

Clinical lectures: Yr1 (28 decks)

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