GN Associated with SLE

Question 1

Most important cause of morbidity and mortality in SLE

Answer 1

Glomerulonephritis

Question 2

Renal disease in childhood SLE is present in up to ___% of patients

Answer 2

80

Question 3

T/F Renal disease in childhood SLE is more active in children than in adults

Answer 3

T

Question 4

Pathogenesis of nephritis in SLE

Answer 4

Binding of autoantibodies to glomerular components rather than passive trapping of immune complexes

Question 5

Deficiency of this complement component is the strongest single genetic risk for SLE

Answer 5

C1q

Question 6

Gold standard for establishing the diagnosis of SLE nephritis

Answer 6

Kidney biopsy

Question 7

WHO Class: No histologic abnormalities on LM; mesangial deposits on IF and EM

Answer 7

I, Minimal mesangial lupus nephritis

Question 8

WHO Class: Both mesangial hypercellularity and increased matrix along with mesangial deposits containing Ig and complement

Answer 8

II, Mesangial proliferative

Question 9

WHO Class: Mesangial ang endocapillary lesions involving less than 50% glomeruli

Answer 9

III

Question 10

WHO Class: Mesangial ang endocapillary lesions involving more than or equal to 50% glomeruli

Answer 10

IV

Question 11

WHO Class: Resembles idiopathic membranous nephropathy with subepithelial immune deposits

Answer 11

V, Membranous lupus nephritis

Question 12

T/F Ethnicity and socioeconomic factors strongly predict development of lupus nephritis in children

Answer 12

F

Question 13

Patients with class V nephritis commonly present with nephritic vs nephrotic syndrome

Answer 13

Nephrotic syndrome

Question 14

T/F In patients with active disease (SLE), C3 and C4 levels are depressed

Answer 14

T

Question 15

T/F Renal biopsy should be performed in all patients with SLE

Answer 15

T, since there is a lack of a clear correlation between the clinical manifestations and the severity of the renal involvement

Question 16

Goals of immunosuppresive therapy in lupus nephritis

Answer 16

1) Clinical remission 2) Serologic remission

Question 17

SLE: Clinical remission is defined as

Answer 17

Normalization of renal function and proteinuria

Question 18

SLE: Serologic remission is defined as

Answer 18

Normalization of anti-DNA Ab, C3 and C4 levels

Question 19

SLE: Prednisone is initiated at a dose of

Answer 19

1-2 mkday

Question 20

SLE: Prednisone tapering

Answer 20

Over 4-6 mos begininning 4-6 weeks after receiving serologic remission

Question 21

SLE: For patients with more severe forms of nephritis (Class III-IV) induction therapy begins with

Answer 21

6 consecutive monthly Cyclo at 500-1000 mg/m2; followed by infusions every 3 months for 18 months

Question 22

SLE: Renal survival is defined as

Answer 22

CKD without the need for ESRD therapy

Question 23

WHO class of SLE nephritis that exhibits the highest risk for progression to ESRD

Answer 23

Class IV

Question 24

Risks of malignancy or infertility may be increased in those receiving a cumulative dose of ___ of Cyclo or other immunosuppresive therapies

Answer 24

> 20g

Question 25

Characterized by mesangial and endocapillary lesions

Answer 25

Class III and IV

Question 26

T/F WHO Class IV Lupus nephritis is associated with poorer outcomes but can be successfully treated with aggressive immunosuppresive therapy

Answer 26

T

Question 27

Clinical findings in milder forms of lupus nephritis (Class I, II, and some III)

Answer 27

1) Hematuria 2) NORMAL RENAL FUNCTION 3) Proteinuria <1g/24h

Question 28

Clinical findings in more severe forms of lupus nephritis (Class III and IV)

Answer 28

1) Hematuria 2) Proteinuria 3) Reduced renal function 4) Nephrotic syndrome 5) Acute renal failure

Question 29

Therapy is initiated in all patients with what drug

Answer 29

Prednisone

Question 30

May be used as a steroid-sparing agent in patients with WHO class I or II lupus nephritis

Answer 30

Azathrioprine

Question 31

Renal survival without need for dialysis is seen in 80% of patients ___ years after diagnosis of SLE nephritis

Answer 31

10