CASE CONFERENCE 02/14/2014

Yuvaraj Thangaraj, MDNephrology FellowDivision of Nephrology ,HTN and Renal Transplantation

HISTORY OF PRESENT ILLNESS

24 y/o male with PMH significant for opioid abuse(iv drug user), tobacco abuse presented to OSH with symptoms of 20 pound weight loss, progressive fatigue, N/V for several weeks duration and 1 episode of hematuria

He was found to have a creatinine of 11, BUN 105, Hemoglobin 7

UA showed microscopic hematuria and Ultrasound showed increased echogenicity and no evidence of Hydronephrosis

ANA : mildly positive, ANCA 1:20 positive, SPEP and UPEP negative

No protein quantification done

He was started on HD for solute clearance and volume management

Noted to have enterococcus in blood culture and urine culture

TTE showed good valvular and left ventricular function

Biopsy showed pauci-immune necrotizing crescentic GN

RENAL BIOPSY

Multiple levels with H&E, PAS and PAMS stainswere evaluated. Sections contain18 glomeruli. Three are globally sclerotic. All but two others shownecrosis and/or crescents. The capillaries are notgenerally patent with marked collapse and necrosis. The tubules showsevere atrophy and focal destruction. There issevere interstitial fibrosis and chronic inflammation. Medium sizedarteries show fibrinoid necrosis, endarteritis andexoarteritis. Attached EM and IF files show pauci-immuneglomerulonephritis.

PMH

1)IV Drug abuse2)Tobacco abuse3)Opioid abuse

PSH

None

FH

None significant

ALLERGY

None

REVIEW OF SYSTEMS

20 lb weight loss

Fatigue

Nausea

Vomiting

Poor appetite

Hematuria

Decreased urine output

PHYSICAL EXAM

Vital Signs:

BP: 137/91 mmHgTemp: 37.3 °C (99.1 °F)Pulse: 110 Resp: 18 SpO2: 95 %

Constitutional: young white male-Not in distressEyes: PERRLENT: No pharyngeal congestion/erythemaNeck : Trachea midline, R chest vascath without drainage or surrounding erythema CV: s1s2 positive, no m/r/gPulm: CTA B/L, no wheezes, rales or rhonchi, symmetric air entry GI: soft, abdominal wall edema, No tenderness Skin: No rashes or skin discolouration

RENAL FUNCTION PANEL CBC

URINALYSIS

SUMMARY

24 y/o male iv drug abuser presents with AKI

UA – microscopic hematuria

USG – increased echogenicity

ANA and ANCA - weekly positive

Renal biopsy - consistent with PNCGN

Blood culture and urine culture - positive for enterococcus

CLINICAL CONUNDRUM

Is this Pauci-immune Crescentic Necrotizing Vasculitis (PCNGN) or Infection Related Crescentic GN (IRGN) ?

How do we approach ?

Decreased Normal

Negative Positive

Crescents Crescents

positive negative

Infection Related-CrescenticGN

Pauci-immuneCrescenticGN

Complement

ANCA

Light microscopy

IF

EM Deposits No deposits

PATHOGENESIS OF IRGN

Ref : Comprehensive textbook of Nephrology, 4th edition: Richard J Johnson

PATHOGENESIS OF VASCULITIS

New pathophysiological insights and treatment of ANCA-associated vasculitisBenjamin Wilde, Pieter van Paassen, Oliver Witzke and Jan Willem Cohen Tervaert

A major differential diagnosis of IRGN and particularly infectious endocarditis–associated GN is ANCA-induced pauci-immune necrotizing and crescentic glomerulonephritis

Crescentic and necrotizing glomerulonephritis is the most common pattern of glomerular injury in patients with infectious endocarditis–associated GN

CONCLUSION

Infective endocarditis related GN is usually not associated with immune complex deposit

Infection related GN (other than Infective endocarditis related GN) is usually associated with immune complex deposit

Pauci-immune necrotizing GN is the most common pathologic finding in renal biopsy in IE

Opana ER is a recently reformulated extended-release form of oxymorphone (an opioid pain reliever) intended for oral

administration

Fourteen of the 15 patients reported injecting reformulated Opana ER

Seven patients were treated for sepsis

The new formulation contains inactive ingredients not found in the original formulation, including polyethylene oxide (PEO) and polyethylene glycol