GN Associated with SLE Flashcards
Most important cause of morbidity and mortality in SLE
Glomerulonephritis
Renal disease in childhood SLE is present in up to ___% of patients
80
T/F Renal disease in childhood SLE is more active in children than in adults
T
Pathogenesis of nephritis in SLE
Binding of autoantibodies to glomerular components rather than passive trapping of immune complexes
Deficiency of this complement component is the strongest single genetic risk for SLE
C1q
Gold standard for establishing the diagnosis of SLE nephritis
Kidney biopsy
WHO Class: No histologic abnormalities on LM; mesangial deposits on IF and EM
I, Minimal mesangial lupus nephritis
WHO Class: Both mesangial hypercellularity and increased matrix along with mesangial deposits containing Ig and complement
II, Mesangial proliferative
WHO Class: Mesangial ang endocapillary lesions involving less than 50% glomeruli
III
WHO Class: Mesangial ang endocapillary lesions involving more than or equal to 50% glomeruli
IV
WHO Class: Resembles idiopathic membranous nephropathy with subepithelial immune deposits
V, Membranous lupus nephritis
T/F Ethnicity and socioeconomic factors strongly predict development of lupus nephritis in children
F
Patients with class V nephritis commonly present with nephritic vs nephrotic syndrome
Nephrotic syndrome
T/F In patients with active disease (SLE), C3 and C4 levels are depressed
T
T/F Renal biopsy should be performed in all patients with SLE
T, since there is a lack of a clear correlation between the clinical manifestations and the severity of the renal involvement
Goals of immunosuppresive therapy in lupus nephritis
1) Clinical remission 2) Serologic remission
SLE: Clinical remission is defined as
Normalization of renal function and proteinuria
SLE: Serologic remission is defined as
Normalization of anti-DNA Ab, C3 and C4 levels
SLE: Prednisone is initiated at a dose of
1-2 mkday
SLE: Prednisone tapering
Over 4-6 mos begininning 4-6 weeks after receiving serologic remission
SLE: For patients with more severe forms of nephritis (Class III-IV) induction therapy begins with
6 consecutive monthly Cyclo at 500-1000 mg/m2; followed by infusions every 3 months for 18 months
SLE: Renal survival is defined as
CKD without the need for ESRD therapy
WHO class of SLE nephritis that exhibits the highest risk for progression to ESRD
Class IV
Risks of malignancy or infertility may be increased in those receiving a cumulative dose of ___ of Cyclo or other immunosuppresive therapies
> 20g
Characterized by mesangial and endocapillary lesions
Class III and IV
T/F WHO Class IV Lupus nephritis is associated with poorer outcomes but can be successfully treated with aggressive immunosuppresive therapy
T
Clinical findings in milder forms of lupus nephritis (Class I, II, and some III)
1) Hematuria 2) NORMAL RENAL FUNCTION 3) Proteinuria <1g/24h
Clinical findings in more severe forms of lupus nephritis (Class III and IV)
1) Hematuria 2) Proteinuria 3) Reduced renal function 4) Nephrotic syndrome 5) Acute renal failure
Therapy is initiated in all patients with what drug
Prednisone
May be used as a steroid-sparing agent in patients with WHO class I or II lupus nephritis
Azathrioprine
Renal survival without need for dialysis is seen in 80% of patients ___ years after diagnosis of SLE nephritis
10
