Noon conference template ce-9-27

1. Noon Conference
Cheyenne Enevold
9/27/18

2. © 2016 Virginia Mason Medical Center
Systemic Lupus Erythematous
Renal Disease
• Differential
• Diagnostic tests/criteria
• Classification
• Prognostic factors
• Treatment
• Illness script
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Objectives

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Match the The Differential
1. Hemoptysis & elev. Cr
2. Purpuric rash & elev. Cr
3. Recent illness & elev Cr.
4. Levido reticularis & elev. Cr
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o Antiphospholipid syndrome
micro emboli
o Goodpasture’s
o Cholesterol emboli
o IgA nephropathy
o Poststrep glomerulonephritis
o Henoch schonlein purpura
o Cryoglobulinemia
o Wegner Granulomatosis
o Microscopic polyangiitis

4. © 2016 Virginia Mason Medical Center
Match the The Differential
1. Hemoptysis & elev. Cr
o Wegener Granulomatosis
o Microscopic polyangiitis
o Goodpasture’s
1. Purpuric rash & elev. Cr
o Henoch Schönlein purpura
o Cryoglobulinemia
1. Recent illness & elev Cr.
o IgA nephropathy
o Poststrep glomerulonephritis
1. Levido reticularis & elev. Cr
o Antiphospholipid syndrome micro emboli
o Cholesterol emboli
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The work-up
1. Hemoptysis & elev. Cr
o Wegener Granulomatosis – c-ANCA
o Microscopic polyangiitis — p-ANCA
o Good pasture’s – anti-GBM ab
1. Purpuric rash & elev. Cr
o Henoch Schönlein purpura — hx & IgA nephropathy
o Cryoglobulinemia —cryoglobulins
1. Recent illness & elev Cr.
o IgA nephropathy — IgA complex on mesangium
o Poststrep glomerulonephritis – ASO titer
1. Levido reticularis & elev. Cr
o Antiphospholipid syndrome micro emboli – antiphospholipid, anticardiolipin,
B2-GP
o Cholesterol emboli –cholesterol on biopsy, clinical history
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Diagnostic tests for SLE
Nephritis
• UA w/ micro
• Random urine protein and creatinine
• Anti-dsDNA, C3, c4
• ANCA
• In this case: anti-GBM, cryoglobulins, ANCA,
ASO titer, antiphospholipid Ab (& b2 and
cardiolipin)
• Renal biopsy
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Diagnostic criteria
-Urinalysis:
-hematuria and cellular casts
-proteinuria – protein/creatine ratio >500 but
usually less than 3.5g per day
- Elevated anti-dsDNA and
Hypocomplementemia, anti-C1q Ab
- (because of Immune complex deposition and
complement activation)
-Definitive Diagnosis = Kidney Biopsy
-Clinical features: HTN, LE edema, oliguria,
hyperlipidemia
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What percentage of people with SLE have renal disease at
presentation?
A. 16%
B. 20%
C. 24%
D. 28%

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What percentage of people with SLE have renal disease at
presentation?
A. 16%
B. 20%
C. 24%
D. 28%— % at 10 years

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right lung nodules and infiltrates
Six types of SLE Nephritis, Based on Histopathology
Renal Pathology/International Society of Nephrology 2004
1. Minimal Mesangial lupus nephritis: rare, nml UA, minimal
proteinuria, nml Cr—> often not biopsied
• Mesangial immune deposits-IM and EM, no Light
Micro findings
2. Mesangial Proliferative Lupus Nephritis: microhematuria
and/or proteinuria
• LM: mesangial hypercellularity
3. Focal Lupus Nephritis: Hematuria/proteinuria, some HTN,
dec. GFR, +/- nephrotic syndrome
• subendothelial Immune deposits on IF
• <50% of glomeruli involved on LM
Classification

11. © 2016 Virginia Mason Medical Center
4. Diffuse Lupus Nephritis: MC, Most severe. Hematuria, proteinuria, HTN,
nephrotic syndrome, dec. GFR
• Hypocomplementemia, elevated ds-DNA (esp active disease)
• >50% of glom. On LM, subendothelial Immune complex
deposits
5. Lupus membranous Nephropathy: nephrotic syndrome presentation,
minimal HTN, hematuria, mild cr change
• Diffuse thickening of glom. Cap.
• Subepithelial Immune deposits on IF
• Can be present w/o other signs of SLE (nml complement and anti-
dsDNA)
6. Advanced Sclerosing Lupus Nephritis: slow prog. Cr increase, bland
UA.
• Global sclerosed, >90% glomeruli
• Healing of prior injury, advanced stage of class 3,4 or 5
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13. © 2016 Virginia Mason Medical Center
Prognostic factors
Worse outcomes if present at time of presentation:
• Elevated Cr
• HTN
• Nephrotic range proteinuria
• Anemia
• Black and hispanic ethnicity
• Tubulointerstitial disease
• Cellular crescents
• Poor response to initial treatment
• Repeat Flares
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Treatment
Class 1: no treatment
Class 2: no treatment
Class 3 & 4: Glucocorticoids w/ taper
• Methylpred 500mg QD 2-3 days, taper w/ oral
prednislone 60 mg
• Mycophenolate mofetil vs. Cyclophosphamide
Class 5: ACEI, BP control, lipid control, Immunosuppressive
therapy if cont. Nephrotic range proteinuria >3.5 g/day or
+prolif features (MM +GC)
Class 6: Usually less responsive to immunosuppressive therapy
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Cyclophosphamide is associated with
what distinct complication?
A. Teratogenicity
B. Bone marrow suppression
C. Myelodysplasia
D.Hemorrhagic cystitis
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Cyclophosphamide is associated with
what distinct complication?
A. Teratogenicity
B. Bone marrow suppression
C. Myelodysplasia
D.Hemorrhagic cystitis
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What medication is used to prevent
this complication?
A. Pyridoxime
B. MESNA
C. Ifosamide
D.Leucovorin
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18. © 2016 Virginia Mason Medical Center
What medication is used to prevent
this complication?
A. Pyridoxime
B.MESNA
C. Ifosamide
D.Leucovorin
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19. © 2016 Virginia Mason Medical Center 19
Mycophenolate Mofetil vs. Cyclophosphamide
• Both are preferred agents for proliferative LN.
• No superiority -- choice based on side effects
• Mycophenolate mofetil
• preferred in black and Hispanic patients
• Preferred in women of childbearing age
• Cyclophosphamide:
• Increased risk of ovarian failure
• Bladder cancer/hemorrhagic cystitis
• Hematologic malignancies
• Bone marrow suppression
• Used IV once per month due to toxicity
• Can use lower oral doses daily, more applicable to other
rheum conditions

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Illness Scripts
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Lupus nephritis
(difuse proliferative class IV)
Goodpasture’s syndrome
(type 1 crescentic glomerulonephritis)
Pathophysiology
Anti-dsDNA, complement activation and
Immune complex deposition, neutrophil
influx
Anti-GBM
(alpha-3 subunit of type IV collagen)
Epidemiology
20-45 yo
F>M
African American, hispanic and asian> whites
20-30 yo > 60-70 yo
M > F
HLA DR15 gene
Time course subacute/chronic subacute/chronic
Clinical
presentation
hematuria, proteinuria, rising Cr, HTN, LE
edema
(SLE symptoms joint pain, vasculitis, fatigue)
hematuria, hemoptysis, sinusitis, LE edema;
fatigue, malaise; joint aches and pains,
purpuric rash
Diagnostics
Labs: UA, elevated Cr
Serologic testing: elevated dsDNA, low C3/C4
Specimen: subnephrotic proteinuria, nephritic
urinary sediment (dysmorphic RBC &/or RBC
casts)
Renal biopsy: >50% of glom. On
LM, subendothelial Immune
complex deposits
CXR: should be clear
Labs: leukocytosis and anemia; elevated
ESR, CRP, Cr, BUN
Serologic testing: anti-GBM
Specimen: macrophages in sputum,
subnephrotic proteinuria, nephritic urinary
sediment (dysmorphic RBC &/or RBC casts)
Renal biopsy: linear anti-GBM (IgG) deposits
on immunofluorescence
CXR: pulmonary infiltrates
Therapeutics
high-dose corticosteroids, cyclophosphamide
vs. Mycophenolate
plasmapharesis, steroids (prednisone),
cyclophosphamide

21. © 2016 Virginia Mason Medical Center
Acknowledgements
Team A!
-Zach Mattes
-Meghan Caballero
-Owen Thompson
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