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U01-4411 and U06- 18160

This case study shows the progression of focal proliferative necrotizing glomerulonephritis with crescent formation in a patient with a history of Wegener's and membranous glomerulopathy. Treatment includes high-dose prednisone, plasmapheresis, and Rituximab.

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U01-4411 and U06- 18160

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  1. U01-4411 and U06-18160 #726961800

  2. U01-4411 • 66 y.o. male • ? Wegener’s

  3. 72 yo male. PMhx: prostate CA (radical prostatectomy - deemed curative), COPD. 2001: • Hemoptysis, bilateral pulmonary infiltrates, active urine sediment, sCr=150, pANCA (+). • Renal biopsy: some IgG subepithelial immune complexes consistent with membranous, but 3/14 glomeruli show focal proliferation and necrosis, 1/14 crescent. Dx: WG Tx: 1 year of cyclophosphamide and prednisone, then d/c’d. 2001-2005 • Followed by nephrologist • Clinically quiescent disease, stable sCr: 100-120

  4. IF • IgG- Mild to moderate coarse capillary loop staining. • IgA- Trivial to trace capillary loop staining. • IgM- Trivial to trace capillary loop staining. • C3- Trivial to trace capillary loop staining. • C1q- Negative. • Kappa- Moderate coarse capillary loop staining. • Lambda- Trivial to trace capillary loop staining. • Fibrin- Negative. • Albumin- Negative.

  5. IgG

  6. Kappa

  7. DiagnosisRenal Biopsy: • Combined membranous glomerulopathy and focal proliferative necrotizing glomerulonephritis with crescent formation. • Rule out SLE.

  8. Comment • There have been several reports of such a combination(Taniguchi, Chronic nephrology 52:253-255, 1991 ; NDT 12:1017-1027, 1997). • Most of the cases described had systemic lupus. • Many light microscopic features suggest lupus in our case but IF positivity for only 2 reactants would be distinctly unusual.

  9. Comment • Our case is intriguing in that fluorescence positivity appears monoclonal or oligoclonal with positive IgG and kappa but negative lambda.

  10. U06-18160 • Rising creatinine • Pulmonary/renal « syndrome » • Previous kidney biopsy UAH 5 years ago

  11. January 2006: sCr=133 on routine testing August 18, 2006: sCr=182, P/C=70 mg/mmol August 28, 2006. Evaluated by new nephrologist. • Completely asymptomatic, physical exam significant only for hypertension (180s). • sCr=253 (eGFR=23), active urine. U/S: normal; CXR: clear • Started on Prednisone 60 mg daily.

  12. Sept 18, 2006: • -Develops ?scant hemoptysis and SOB. bp: 190 systolic, hypoxic requiring 4L O2, CXR: LLL consolidation. • -Admitted to UAH Pulmonary ward • -Bronchoscopy: NO hemorrhage. • -Blood and BAL culture: Strep pneumoniae. Tx: Levofloxacin. • -Cr=365, active urine sediment, P/C=260 mg/mmol • -pANCA weakly positive, anti-GBM negative. Aggressively fluid resuscitated

  13. Sept 20, 2006 -sCr=360 (non-oliguric), hypoxia/cough improving.  Transfer to nephrology Sept 22, 2006: sCr=440 : << RENAL BIOPSY >>

  14. IF • IgG- Negative. • IgA- Negative. • IgM- Negative. • C3- Mild vascular staining. • C1q- Negative. • Kappa- Negative. • Lambda- Negative. • Fibrin- Mild interstitial staining. • Albumin- Negative.

  15. C3

  16. Fibrin

  17. EM • Will be ready next week

  18. DiagnosisRenal Biopsy: • Focal proliferative and necrotizing glomerulonephritis progressing toward end-stage renal disease. • A previously documented membranous glomerulopathy seen in 2001 is less apparent now.

  19. Comment • This biopsy appears to show a more advanced stage of the process seen in the previous biopsy in 2001. • Now the membranous process is less apparent • The disease process seems to be mainly the focal proliferative and necrotizing GN, which has now progressed to nearly end-stage disease.

  20. Dx: Recurrent ANCA-associated GN Tx: Given recent pneumonia and oral thrush, hesitant to use cyclophosphamide. Therefore, continued high dose prednisone, started plasmapheresis and Rituximab Sept 28: sCr(peak)=549 Sept 29: plasmapheresis started sCr trending down to low 500s. No HD yet

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