Part 15 - Allergology, Immunology, Rheumatology Flashcards

1
Q

In SLE, what complement deficiencies are involved?

A

C1q,r,s
C2
C4

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2
Q

People with ______ karyotype have a significantly increased risk for SLE.

A

XXY (Klinefelter’s)

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3
Q

In SLE, at what skin layer (on skin biopsies) is deposition of immunoglobulin found?

A

Dermal-epidermal junction (DEJ)

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4
Q

At what class of disease is treatment for lupus nephritis NOT recommended?

A

Class I or II

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5
Q

What is the name of the criteria for classification of SLE?

A

Systemic Lupus International Collaborating Clinic (SLICC)

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6
Q

What is the most common pattern of vasculitis in SLE, that may also indicate active disease?

A

Leukocytoclastic vasculitis

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7
Q

In SLE, ANA is positive in how many percent of patients during the course of disease?

A

> 98%

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8
Q

What is the best screening test for SLE?

A

ANA

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9
Q

What autoantibodies are specific for SLE?

A

Anti-dsDNA

Anti-Sm

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10
Q

What autoantibody is SLE-specific and may correlate with disease activity?

A

Anti-dsDNA

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11
Q

Which autoantibody is more frequently seen in drug-induced lupus than in SLE?

A

Antihistone

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12
Q

Which autoantibody, when produces a positive test in serum correlates with depression or psychosis due to CNS lupus?

A

Antiribosomal P

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13
Q

In SLE, what is the gene defect found on the X chromosome?

A

Mutation in TREX1

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14
Q

What are the types/classes of lupus nephritis?

A
Class I: minimal mesangial
Class II: mesangial proliferative
Class III: focal
Class IV: diffuse
Class V: membranous
Class VI: advanced sclerotic
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15
Q

What is “Rhupus”?

A

Rheumatoid-like arthritis with erosions and fulfill criteria for both RA and SLE

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16
Q

In SLE, what diagnosis will you consider if arthralgia is persistent in a single joint?

A

Ischemic necrosis of bone

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17
Q

What is the most common chronic dermatitis in lupus?

A

Discoid lupus erythematosus

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18
Q

What is the treatment for discoid lupus erythematosus?

A

Topical or locally injected glucocorticoids and systemic antimalarials

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19
Q

What is the most serious manifestation of SLE?

A

Nephritis

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20
Q

What is the most common manifestation of diffuse CNS lupus?

A

Cognitive dysfunction

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21
Q

In SLE, at what dose of Prednisone does glucocorticoid-induced psychosis occur?

A

> or = to 40 mg

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22
Q

Endocarditis of SLE is called?

A

Libman-Sacks endocarditis

23
Q

What is the most common pulmonary manifestation of SLE?

A

Pleuritis with or without pleural effusion

24
Q

Life threatening pulmonary manifestations of SLE

A

Interstitial fibrosis
Shrinking lung syndrome
Intraalveolar hemorrhage

25
Q

What is the most frequent cardiac manifestation of SLE?

A

Pericarditis

26
Q

Endocardial involvement in SLE may lead to insufficiencies commonly on what valves?

A

Mitral or aortic

27
Q

What is the most frequent hematologic manifestation of SLE?

A

Anemia

usually normochromic normocytic

28
Q

What are serious ocular manifestations of SLE that may cause blindness?

A

Retinal vasculitis

Optic neuritis

29
Q

Three antiphospholipid antibodies

A

Anticardiolipin
anti-B2 glycoprotein
Lupus anticoagulant

30
Q

What titer level of IgG anticardiolipin is considered high?

A

> 40 IU

31
Q

What is the criteria for diagnosing APS?

A

Presence of 1 or more clotting episodes and/or repeated fetal losses
PLUS
at least 2 positive tests for antiphospholipid antibodies at least 12 weeks apart

32
Q

What autoantibody indicates increased risk for neonatal lupus, sicca syndrome, and subacute cutaneous lupus?

A

Anti-Ro (SS-A)

33
Q

What treatment has been shown to prevent SLE flares in patients with rising anti-DNA plus falling complement?

A

30 mg Prednisone daily for 2 weeks

34
Q

Antimalarials used for treatment of SLE

A

Hydroxychloroquine
Chloroquine
Quinacrine

35
Q

Mainstay of treatment for any inflammatory life-threatening manifestations of SLE

A

Systemic glucocorticoids
(0.5-1 mg/kg per day PO
OR
500-1000 mg of methylprednisolone succinate IV daily for 3 days followed by 0.5-1 mg/kg of daily prednisone or equivalent

36
Q

Cytotoxic/immunosuppressive agents added to glucocorticoids for treatment of severe SLE

A

Cyclophosphamide or mycophenolate mofetil

37
Q

Alkylating agent used to treat serious SLE

A

Cyclophosphamide

38
Q

Lymphocyte specific inhibitor of inosine monophosphatase / purine synthesis used to treat serious SLE

A

Mycophenolate mofetil

39
Q

Common manifestation of mycophenolate mofetil toxicity

A

Diarrhea

40
Q

Common manifestations of cyclophosphamide toxicity

A

Amenorrhea
Leukopenia
Nausea

41
Q

Common effect of high dose cyclophosphamide (not low dose)

A

Ovarian failure

42
Q

Given to reduce incidence of ovarian failure due to high-dose cyclophosphamide therapy for SLE

A

Gonadotropin-releasing hormone agonist (ex. Leuprolide 3.75 mg IM) prior to each cyclophosphamide dose

43
Q

Can be used if necessary to control active SLE in pregnant patients (if conventional therapy do not work)

A

Azathioprine

44
Q

A folinic acid antagonist that may be used for treatment of arthritis and dermatitis due to SLE (but not in nephritis or life-threatening disease)

A

Methotrexate

45
Q

Anti-BLys agent used for treatment of seropositive patients with SLE who have failed standard treatments

A

Belimumab

46
Q

Widely used scoring to measure SLE disease activity

A

Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)

47
Q

What score in SLEDAI reflects clinically active disease in SLE?

A

> 3

48
Q

Induction therapy of choice for crescentic lupus nepritis

A

High-dose cyclophosphamide

49
Q

Placental enzyme that deactivates glucocorticoids

A

11-B-dehydrogenase 2

50
Q

Pregnancy category of methotrexate

A

Category X

51
Q

Pregnancy category of azathioprine, hydroxychloroquine, mycophenolate mofetil, and cyclophosphamide

A

Category D

52
Q

Pregnancy category of cyclosporine, tacrolimus, rituximab

A

Category C

53
Q

Pregnancy category of glucocorticoids

A

Category A

54
Q

Active SLE in pregnant women should be controlled with?

A

Hydroxychloroquine > prednisone/prednisolone (lowest possible dose) > azathioprine