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MSK/Derm > Systemic Lupus Erythematosus > Flashcards

Systemic Lupus Erythematosus Flashcards

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Dermatology Board Prep flashcards
1
Q

What is SLE?

A

an inflammatory, multisystem, autoimmune disease of unknown etiology with protean clinical and laboratory manifestations and a variable course and prognosis

lupus can be a mild disease, a severe and life-treatening illness, or anything inbetween

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2
Q

What is the epidemiology of lupus?

A

prevalence is 2-7/100,000 worldwide but as high as 207/100,000

incidence is 1-10/100,000 worldwide

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3
Q

What population is at highest risk for lupus?

A

women in their reproductive years

female:male ratio is approximately 9:1 postpuberty and premenopausal

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4
Q

What is the variation in ethniciy in lupus?

A

blacks (3x)

hispanics and native americans (2-3x)

asians (2x)

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5
Q

What are some classification criteria for lupus?

A

95% specificity, 85% sensitivity

external, systemic, and internal findings

hematologic disorder

antinuclear antibodies (ANA)

immunologic disorder

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6
Q

What are the external features of lupus?

A

synovitis

malar rash

oral ulcer

subacute cutaneous lupus erythematosus

discord rash

Jaccoud’s arthropathy (deforming arthritis)

vasculitis

lupus profundus

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7
Q

What are the internal findings of lupus?

A

serositis

pericardial effusion

cerebral infarct

brain atrophy

spherocytes

glomerulonephritis

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8
Q

What are the systemic findings of lupus?

A

pain

fatigue

memory loss

depression

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9
Q

What organ systems are affected by lupus?

A

eyes

skin

pleurisy

kidney

muscle

Raynaud’s vasculitis

joints

blood

heart

ears and nose

CNS

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10
Q

malar rash

A

fixed erythema, flat or raised, over the malar eminence, tending to spare the nasolabial folds

“butterfly rash”

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11
Q

discoid rash

A

erythematous raised patches with adherent keratotic scaling and follicular plugging

atrophic scarring may occur in older lesions

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12
Q

serositis from SLE

A

a) pleuritis; convincing history of pleuritic pain or rub heard by physician or evidence of pleural effusion
b) pericarditis; documented by electrocardiogram or rub or evidence of pericardial effusion

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13
Q

renal disorder from SLE

A

a) persistent proteinuria > 500 mg per day or > 3+ if quantitation no performed

or

b) cellular casts - may be red cell, hemoglobin, granular, tubular, or mixed

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14
Q

neurologic disorders of SLE

A

a) seizures or b) psychosis - in the absence of offending drugs or known metabolic derangement

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15
Q

hematologic disorders of SLE

A

a) hemolytic anemia - with reticulocytosis
b) leukopenia - < 4000/mm3 total
c) lymphopenia - < 1500/mm3 on two or more occasions
d) thrombocytopenia - < 100,000/mm3

**all in the absence of offending drugs

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16
Q

immunologic disorders of SLE

A

a) anti-DNA: antibody to native DNA in abnormal titer
b) anti-SM: presence of antibody to SM nuclear antigen
c) positive finding of antiphospholipid antibodies

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17
Q

anti-dsDNA Ab

A

not only of major diagnostic significance but in select patients, particularly those with renal involvement, a valuable means of predicting and assessing disease activity

18
Q

anti-Sm antibodies

A

recognize determinants on proteins associated with small ribonucleoproteins involved in processing of messenger RNA

of diagnostic importance but do not track disease

19
Q

SSA/Ro abd SSB/La antibodies

A

SSA - presence of antibodies can stain the cytoplasmic component of the cell and accounts for some ANA-negative lupus

SSB - involved in transcription termination, seen in patients with one or more of the following: photosensitivity, dry eyes and dry mouth (secondary Sjogrens), subacute cutaneous lesions, risk of a child with neonatal lupus

20
Q

What do all lupus patients have in common?

A

antinuclear antibodies (ANA)

this test is not diagnostic of lupus but almost all patietns have this

immunofluorescence is the most reliable - antibody staining

sensitive but not specific

21
Q

What are othe conditiosn with positive ANA?

A

3-4% in normal people

95% in scleroderma

50% in Hashimoto’s thyroididis

50% idiopathic pulmonary fibrosis

incidence increases with age, chronic infections, and other chronic conditions

22
Q

What are other pathogenic antibodies and what do they cause?

A

anti-SSA and anti-SSB

causes:

  • subacute cutaneous lupus
  • neonatal lupus

complete heart block in utero

23
Q

What are all of the autoantibodies in SLE?

A

ANA

anti-dsDNA (nephritis)

anti-Sm

anti-RNP (arthritis, myositis, lung disease)

anti-SSA

anti-SSB

antiphospholipid

24
Q

What are the phases of lupus pathogenesis?

A

initiation

amplification and perpetuation

irreversible damage

25
Q

What are the characteristics of the initiation phase of lupus pathogenesis?

A

multiple proposed mechanisms that may vary from patient to patient

occurs years prior to onset of clnical symptoms

26
Q

What are the characteristiscs of the amplification and perpetuation phase of lupus pathogenesis?

A

amplification and perpetuation of dysregulated immune mechanisms and response of target organs to inflammatory insult

27
Q

What are the characteristics of the irreverisble stage of lupus pathogenesis?

A

arises from disease and secondary affects of treatment

28
Q

What are the behavior risks that can lead to lupus?

A

smoking

sun exposure

stress

toxins

29
Q

What are the environmental risk factors of lupus?

A

antigen

hormones (estrogen)

infections

toxins

medications

sun exposure

vitamin D deficiency

30
Q

What are the changes in B-cells in lupus?

A

defective selection/signaling

autoantibody production

31
Q

What happens to T-cells in lupus?

A

increased numbers of Th17 and Th2 cells and decreased numbers of Tregs

T-cells are less susceptible ot activation-induced cell death

32
Q

What are the changes in plasmacytoid dendritic cells in lupus?

A

produce large amounts of interferon

plasmacytoid dendritic cells - symulate activiation and proliferation of autoreactive T- and B-cells

33
Q

What are predctors of a flare in lupus?

A

new evidence of complement consumption

rising anti-dsDNA titers

increased ESR

new lymphopenia

34
Q

What are the characteristics of severe lupus?

A

abrupt onset of symptoms

increased renal, neurologic, hematologic, and serosal involvement

rapid accrual of damage (irreversible organ injury)

associated with race/ethnicity, younger age, male gender, low SE status

35
Q

What is the mortality of lupus?

A

current survival rate is >90%

leading causes of mortality are heart disease, malignancy, and infection

36
Q

What are the goals of therapy for lupus?

A

stop and reverse ongoing organ inflammation

prevent ro limit irreversible end-organ damage

37
Q

What are the drugs used in lupus treatment?

A

corticosteroids

cyclophosphamide

methotrexate

mycophenolate mofetil

azathioprine

hydroxychloroquine

belimumab

38
Q

What are the side effects for immunosuppressive drugs?

A

infection

cancer

infertility

39
Q

What are the common side effects of corticosteroids?

A

infection

Cushingoid appearance

osteoporosis

osteonecrosis

diabetes

mood disturbances

hypertension

lipid abnormalities

40
Q

What are new therapeutic strategies for treating lupus?

A

B-cell directed

cytokine inhibitors

costimulation blockade

peptide inhibitors

kinase inhibitors

T regulatory cells

stem cell transplants

41
Q

What are the guiding therapeutics strategies in treating lupus?

A

aimed at induction of remission, maintenacne therapy, and supportive therapy

titrate dose to treat effectively with focus on involved organs, and minimize toxicity

strategic use of preventive therapites - antibiotics and vaccinations

cardiovascular, cancer, and osteoporosis screening

42
Q

What is the prognosis of SLE?

A

over 90% of patients survive at least 2 yeasr after diagnosis, and 80-90% survive up to 10 years

patients who die within 5 years of diagnosis usually have active dsease requiring high doses of corticosteroids, immunosuppression, and have concomitant infections

late deaths are often the result of CVD

MSK/Derm (54 decks)

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