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Inflammatory Connective Tissue Disease > 23 Systemic Lupus Erythematosus > Flashcards

23 Systemic Lupus Erythematosus Flashcards

1
Q

Autoantibodies MC associated with SLE

A

ANA and anti-dsDNA

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2
Q

T/F: Childhood-onset SLE is typically more severe than adult disease

A

T

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3
Q

3 SLE classification criteria

A

ACR 1997, SLICC 2012, EULAR/ACR 2019

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4
Q

SLICC criteria for SLE requires 4 items with at least 1 clinical and 1 immunological item OR

A

Biopsy-proven nephritis compatible with lupus in the presence of ANA or anti-dsDNA

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5
Q

EULAR/ACR entry criterion

A

ANA at a titer of 1:80 or greater

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6
Q

EULAR/ACR minimum score required to classify as SLE

A

10 + entry criterion

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7
Q

Average age of onset of cSLE

A

12

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8
Q

Characteristic of the malar rash of SLE compared to JDM

A

Spares the nasolabial folds

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9
Q

This value of urine protein via dipstick is equivalent to proteinuria >0.5g/day if quantified

A

> 3+

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10
Q

In the EULAR/ACR criteria for SLE, the highest points are given to parameters that point to what organ system

A

Renal, renal biopsy class III or IV = 10 pts, class II or V=8 pts

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11
Q

Hallmark of lupus

A

Production of autoantibodies and hypergammaglobulinemia

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12
Q

T/F Synovitis in SLE may be unilateral according to criteria

A

F, at least 2 joints

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13
Q

Typical pleurisy of SLE based on SLICC

A

Lasting for more than 1 day

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14
Q

Typical pericardial effusion of SLE based on SLICC

A

Pain with recumbency, improved by sitting forward for >1 day

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15
Q

Manifestations of SLE mediated by direct cellular injury

A

Cytopenias

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16
Q

Manifestations of SLE mediated by ICs

A

LN and vasculitis

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17
Q

A higher prevalence of these viruses virus has been described in SLE

A

EBV and CMV

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18
Q

T/F Patients with Klinefelter syndrome (XXY) have more severe disease compared to XY men

A

False, higher risk of nephritis and renal failure in XY males

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19
Q

Cells that produce IFN-a implicated in the pathophysiology of SLE

A

pDCs

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20
Q

UV that is implicated as a trigger for cutaneous and systemic lupus

A

UVB

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21
Q

Drugs MC associated with DIL

A

HPI MM QC: hydralazine, procainamide, isoniazid, methyldopa, minocycline, quinidine, chlorpromazine

22
Q

MC drug class implicated in DIL among children

A

Antiepileptics

23
Q

Antibodies implicated in DIL

A

Anti-histone antibodies

24
Q

T/F Anti-histone Abs are diagnostic of SLE

A

F, suggestive but not diagnostic

25
Q

When does DIL resolve after discontinuation of implicated drug

A

Within weeks to months; lack of reversibility suggests that an underlying SLE may have been unmasked

26
Q

T/F In DIL, it has been observed that there is worsening of manifestations with reexposure to the drug

A

T

27
Q

Histological hallmark of LN

A

Full house pattern: IgG, IgM, IgA, C3, C1q, kappa and lambda light chain deposits

28
Q

Fibrocellular and fibrous crescents with glomerulosclerosis are characteristics of what class of LN

A

Class VI or end-stage LN

29
Q

Isolated hematuria and/or nonnephrotic proteinuria is generally seen in what class of LN

A

Class II

30
Q

Acute nephritic syndrome is generally seen in what class of LN

A

Class III and IV

31
Q

Nephrotic syndrome is the MC presentation of what class of LN

A

Class V

32
Q

Spot UPCr consistent with proteinuria

A

> 0.2mg/mg

33
Q

1st-degree relatives of patients with SLE have a ___% chance of having the disease

A

10

34
Q

There is a ___-fold increased risk of siblings having SLE compared to the general population

A

20

35
Q

___ family members of patients with SLE also have a connective tissue disease

A

1 in 10

36
Q

T/F Patients with DIL tend to have milder disease compared to those with idiopathic SLE

A

T

37
Q

T/F DIL usually occurs after several months of treatment

A

T

38
Q

T/F Major organ involvement is common in DIL

A

F

39
Q

LN Class: <50% of glomeruli

A

Class III

40
Q

LN Class: ≥50%of glomeruli

A

Class IV

41
Q

LN Class: Membranous LN

A

Class V

42
Q

T/F Gross (macroscopic) hematuria is not common in SLE

A

T, if present, it should raise the suspicion for other causes such as renal vein thrombosis (as a complication of nephrotic syndrome or APAS)

43
Q

Proteinuric or renal flare

A

Persistent increase in the UPCr of 0.5 after complete remission or proteinuria OR doubling of UPCr to a value >1 after achieving a partial response

44
Q

Nonproteinuric/nephritic flare

A

Increase or recurrence of active urine sediment with or without increase in proteinuria

45
Q

Compared to CYC, AZA and MMF have been shown to have lower rates of what complications

A

Amenorrhea and infections

46
Q

Compared to AZA, MMF have lower rates of what complications

A

Anemia and leukopenia

47
Q

About ___% of patients with LN have a relapse with a median of 32-79 months

A

40

48
Q

Predictors of poor renal outcome in LN

A

1) African-American race 2) low GFR <60mL/min/BSA 3) Nephrotic range proteinuria at presentation 4) Treatment resistance 5) Lack of response in the 1st 6 months of induction 6) Htn 7) Delay in diagnosis of LN 8) Higher chronicity score on biopsy 9) Greater degree of tubulointerstitial disease

49
Q

Recurrence of SLE after renal transplantation occurs in ~___% of patients

A

2-30

50
Q

T/F Recurrence of SLE after renal transplantation is generally mild and is not associated with decreased patient survival

A

T

51
Q

T/F Recurrence of SLE after renal transplantation is associated with greater rate of graft loss

A

T

52
Q

Leading cause of death in LN

A

Infection and cardiovascular complications

Inflammatory Connective Tissue Disease (8 decks)

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