23 Systemic Lupus Erythematosus

Question 1

Autoantibodies MC associated with SLE

Answer 1

ANA and anti-dsDNA

Question 2

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

Answer 2

T

Question 3

3 SLE classification criteria

Answer 3

ACR 1997, SLICC 2012, EULAR/ACR 2019

Question 4

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

Answer 4

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

Question 5

EULAR/ACR entry criterion

Answer 5

ANA at a titer of 1:80 or greater

Question 6

EULAR/ACR minimum score required to classify as SLE

Answer 6

10 + entry criterion

Question 7

Average age of onset of cSLE

Answer 7

12

Question 8

Characteristic of the malar rash of SLE compared to JDM

Answer 8

Spares the nasolabial folds

Question 9

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

Answer 9

> 3+

Question 10

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

Answer 10

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

Question 11

Hallmark of lupus

Answer 11

Production of autoantibodies and hypergammaglobulinemia

Question 12

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

Answer 12

F, at least 2 joints

Question 13

Typical pleurisy of SLE based on SLICC

Answer 13

Lasting for more than 1 day

Question 14

Typical pericardial effusion of SLE based on SLICC

Answer 14

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

Question 15

Manifestations of SLE mediated by direct cellular injury

Answer 15

Cytopenias

Question 16

Manifestations of SLE mediated by ICs

Answer 16

LN and vasculitis

Question 17

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

Answer 17

EBV and CMV

Question 18

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

Answer 18

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

Question 19

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

Answer 19

pDCs

Question 20

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

Answer 20

UVB

Question 21

Drugs MC associated with DIL

Answer 21

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

Question 22

MC drug class implicated in DIL among children

Answer 22

Antiepileptics

Question 23

Antibodies implicated in DIL

Answer 23

Anti-histone antibodies

Question 24

T/F Anti-histone Abs are diagnostic of SLE

Answer 24

F, suggestive but not diagnostic

Question 25

When does DIL resolve after discontinuation of implicated drug

Answer 25

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

Question 26

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

Answer 26

T

Question 27

Histological hallmark of LN

Answer 27

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

Question 28

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

Answer 28

Class VI or end-stage LN

Question 29

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

Answer 29

Class II

Question 30

Acute nephritic syndrome is generally seen in what class of LN

Answer 30

Class III and IV

Question 31

Nephrotic syndrome is the MC presentation of what class of LN

Answer 31

Class V

Question 32

Spot UPCr consistent with proteinuria

Answer 32

>0.2mg/mg

Question 33

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

Answer 33

10

Question 34

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

Answer 34

20

Question 35

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

Answer 35

1 in 10

Question 36

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

Answer 36

T

Question 37

T/F DIL usually occurs after several months of treatment

Answer 37

T

Question 38

T/F Major organ involvement is common in DIL

Answer 38

F

Question 39

LN Class: <50% of glomeruli

Answer 39

Class III

Question 40

LN Class: ≥50%of glomeruli

Answer 40

Class IV

Question 41

LN Class: Membranous LN

Answer 41

Class V

Question 42

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

Answer 42

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

Question 43

Proteinuric or renal flare

Answer 43

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

Question 44

Nonproteinuric/nephritic flare

Answer 44

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

Question 45

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

Answer 45

Amenorrhea and infections

Question 46

Compared to AZA, MMF have lower rates of what complications

Answer 46

Anemia and leukopenia

Question 47

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

Answer 47

40

Question 48

Predictors of poor renal outcome in LN

Answer 48

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

Question 49

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

Answer 49

2-30

Question 50

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

Answer 50

T

Question 51

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

Answer 51

T

Question 52

Leading cause of death in LN

Answer 52

Infection and cardiovascular complications