U01-4411 and U06-18160 #726961800. U01-4411 66 y.o. male ? Wegener’s.

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Presentation transcript:

U and U #

U y.o. male ? Wegener’s

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 Followed by nephrologist Clinically quiescent disease, stable sCr:

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.

IgG

Kappa

Diagnosis Renal Biopsy: Combined membranous glomerulopathy and focal proliferative necrotizing glomerulonephritis with crescent formation. Rule out SLE.

Comment There have been several reports of such a combination (Taniguchi, Chronic nephrology 52: , 1991 ; NDT 12: , 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.

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

U Rising creatinine Pulmonary/renal « syndrome » Previous kidney biopsy UAH 5 years ago

January 2006: sCr=133 on routine testing August 18, 2006: sCr=182, P/C=70 mg/mmol August 28, 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.

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

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

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

C3

Fibrin

EM Will be ready next week

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

Comment This biopsy appears to show a more advanced stage of the process seen in the previous biopsy in 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.

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