u01-4411 and u06-18160 #726961800. u01-4411 66 y.o. male ? wegener’s

U01-4411 and U06-18160

#726961800

U01-4411

• 66 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: WGTx: 1 year of cyclophosphamide and prednisone, then d/c’d.2001-2005•Followed by nephrologist•Clinically quiescent disease, stable sCr: 100-120

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

DiagnosisRenal 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 52:253-255, 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.

Comment

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

U06-18160

• 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, 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.

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, 2006

-sCr=360 (non-oliguric), hypoxia/cough improving.

Transfer to nephrology

Sept 22, 2006: sCr=440 : << RENAL BIOPSY >>

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

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.

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.

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)=549Sept 29: plasmapheresis started  sCr trending down to low 500s. No HD yet