32. Systemic lupus erythematosus Flashcards Preview

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Flashcards in 32. Systemic lupus erythematosus Deck (53)
1

classic linical presentation of SLE

rash , joint, pain and fever

2

SLE is MC ( sex, age origin)

female of reprodructive age and African descent

3

Lupus endocarditis - name

Lidman - Sacks endocarditits

4

Lidman - Sacks endocarditits of lupus

nonbacterial, wart - lile ( verrucous ) vegetations on both sides of valve ( mitral ) or aortic valve

5

Lupus nephritis is a result of

type III hypersensitivity

6

Lupus nephritis - MC

nephritic- diffuse proliferative glomerulonephritis nephrotic - membranous glomerulonephritis
MC and severe type is diffuse proliferative

7

Common causes of death in SLE

1. cardiovasuclar disease
2. Infenction
3. Renal disease

8

SLE symptoms / findings / treatmnent - MNEMONIC RASH OR PAIN

RASH OR PAIN
Rash, Arhtitis, Soft tissues/ Serosistis, Hematologic disorders, Oral and nasopharyngeal ulcers , Renal disease/ Raynaud phenomenon, Photosensitivity, Antinuclear antibodies
Immunologic disorder ( anti -ds DNA, anti - SM, antiphopholipids) , Neurologic disorders

9

SLE - hematologic disorders - example

pancytopenia

10

SLE- heart

it can affect any layer of the heart ( endo, myi, pericarditis

11

SLE - rash

malar or discoid

12

SLE treatment

1. NSAID
2. Steroids
3. Immunosuppressants
4. Hydroxychloroquine

13

SLE - lab findings

1. antinuclear antibodies ( ANA)
2. anti- dsDNA antibodies
3. anti - Smith antibodies
4. decreased C3 C4 CH50
5. Anti- histone antibodies

14

SLE - decreased C 3 C4 CH50 due to

immune complex formation

15

SLE - sensitive, not specidic test

ANA

16

SLE - specific, poor prognosis test

anti- dsDNA ( renal disease)

17

SLE - specific , not prognostic test

anti- Smith

18

SLE - sensitive for drug induced lupus

antihistone antibodies

19

Anti- Smith antibodies - against ....

sn RNPs

20

antiphospholipid syndrome is a ... or .... disorder

1ry or 2ry autoimmune disorder

21

antiphospholipid syndrome as a 2ry autoimmune disorder is MC in

SLE

22

antiphospholipid syndrome - autoantibodies against

phospholipids

23

antiphospholipid syndrome - autoantibodies against

phospholipids

24

diagnosis of antiphospholipid syndrome is based on

clinical criteria and lab findings

25

clinical criteria of antiphospholipid syndrome

1. history of thrombosis ( arterial or venous )
2. recurrent spontaneous abortion

26

antiphospholipid syndrome arterial or venous thrombosis includes

1. DVT
2. hepatic vein thrombosis
3. placental thrombodis

27

antiphosphoolipid syndrome - lab findings

1. lupus anticoagulants
2. anticardiolipin
3. anti - β2 - glycoprotein antibodies

28

lupus anticoagulant is an

immunoglobulin that binds to phospholipids and proteins associated with cell membrane

29

antiphospholipid syndrome - lupus anticoagulants and anticardiolipin antibodies can cause .... ( lab)

false positive VDRL/RPR
falsely elevated PTT

30

antiphospholipid syndrome - treatment

systemic anticoagulation

31

Drug induced lupus - drugs

1. Hydralazine
2. Procainamide
3. Isoniazid

32

Lupus endocarditis - valves

1. mitral
2. aortic

33

Mixed onnective tissues disease - clinical presentation

Feature of SLE, systemic sclerosis, and /or polyomyositis

34

Mixed connective tissues disease - Labs

anti - U1 RNP antibodies ( speckled ANA)

35

SLE - lab findings ( and their characteristics )

1. antinuclear antibodies ( ANA) - sensitive, not specific
2. anti-ds DNA antibodies - specific , poor prognosis ( renal disease)
3. anti- Smith antibodies - specific , not prognostic ( directed against snRNPs)
4. decreased C3, C4, CH50- due to immune complex formation
5. Anti- histone antibodies - sensitive dor drug -induced lupus )

36

• What demographic is most commonly affected by lupus?

Females of reproductive age and African descent

37

• A patient with lupus dies of complications of renal disease. Autopsy of her heart valves may show what pathology?

Nonbacterial verrucous endocarditis (i.e., Libman-Sacks endocarditis) with vegetations on both sides of the valves

38

• A man with glomerulonephritis, Raynaud phenomenon, and photosensitivity is VDRL/RPR positive. What antibodies are specific for this disease?

Anti-dsDNA and anti-Smith antibodies (the patient likely has systemic lupus erythematosus)

39

• A 19-year-old woman with a malar rash notes that her hands turn white and then blue when she is cold. What condition does she likely have?

Raynaud phenomenon, which is associated with systemic lupus erythematosus

40

• A 27-year-old woman with a history of lupus develops hematuria. What histologic finding(s) do you expect on renal biopsy?

Evidence of diffuse proliferative glomerulonephritis (a common cause of nephritic syndrome in lupus patients)

41

• A lupus patient suffers from nephrotic syndrome. What type of glomerulonephropathy do you expect her to have?

Membranous glomerulonephritis

42

• Patients with systemic lupus erythematosus may be diagnosed falsely with which disease based on serologic testing? Why does this happen?

Syphilis (due to a positive VDRL/RPR test); antiphospholipid antibodies cross-react with the cardiolipin antigen used in the test

43

• A patient presents with a malar rash, mouth ulcers, and arthritis. She is about to go on vacation to Hawaii. What do you tell her?

She has systemic lupus erythematosus and therefore could be photosensitive—she should protect against sun exposure

44

• Which serologic findings (antibodies) can be positive in patients with lupus?

Antinuclear, anti-double-stranded DNA, and anti-Smith antibodies (antihistone antibodies in cases of drug-induced lupus)

45

• A man on procainamide develops arthritis, fatigue, and pericarditis. His serum might reveal the presence of which antibody?

Antihistone antibodies, specific for drug-induced lupus

46

• What are possible mucocutaneous manifestations of systemic lupus erythematosus?

Malar or discoid rashes, mucositis (oropharyngeal ulcers), photosensitivity

47

• Which antibody is specific for lupus but is not prognostic?

Anti-Smith antibodies

48

Which antibody is specific for lupus and predicts a poor prognosis?

Anti-double-stranded DNA antibodies

49

• Which antibody is sensitive but not specific for lupus and is used as a primary screening test?

Antinuclear antibodies

50

• A young woman has leukopenia and oropharyngeal ulcers. She also has diffuse joint pains and a facial rash. What treatments can be offered?

Treat with NSAIDs, steroids, immunosuppressants, and/or hydroxychloroquine (this is likely systemic lupus erythematosus)

51

• Renal biopsy of a lupus patient shows immune complex deposition. Which lab findings would support the formation of these complexes?

Decreased C3, C4, and CH50 levels

52

• A young woman presents with a history of seizures and psychotic episodes. Is lupus on your differential diagnosis?

Yes, as systemic lupus erythematosus may present with neurologic abnormalities such as seizures and psychosis

53

• Use the mnemonic RASH OR PAIN to list the classic findings seen in patients with systemic lupus erythematosus.

Rash, Arthritis, Serositis, Hematologic, Oral ulcers, Renal, Raynaud, Photosensitive, Positive VDRL/RPR, ANA, Immunosuppression, Neurologic

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