Download Pauci-immune

CASE CONFERENCE
02/14/2014
Yuvaraj Thangaraj, MD
Nephrology Fellow
Division 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 stains
were evaluated. Sections contain
18 glomeruli. Three are globally sclerotic. All but two others show
necrosis and/or crescents. The capillaries are not
generally patent with marked collapse and necrosis. The tubules show
severe atrophy and focal destruction. There is
severe interstitial fibrosis and chronic inflammation. Medium sized
arteries show fibrinoid necrosis, endarteritis and
exoarteritis. Attached EM and IF files show pauci-immune
glomerulonephritis.
PMH
1) IV Drug abuse
2) Tobacco abuse
3) 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 mmHg
Temp: 37.3 °C (99.1 °F)
Pulse: 110
Resp: 18
SpO2: 95 %
Constitutional: young white male-Not in distress
Eyes: PERRL
ENT: No pharyngeal congestion/erythema
Neck : Trachea midline, R chest vascath without drainage or surrounding erythema
CV: s1s2 positive, no m/r/g
Pulm: 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 ?
Infection
Related Pauci-immune
Crescentic GN
Crescentic GN
Complement
Decreased
Normal
ANCA
Negative
Positive
Light microscopy
Crescents
Crescents
IF
positive
negative
EM
Deposits
No deposits
PATHOGENESIS OF IRGN
PATHOGENESIS OF VASCULITIS
Ref : Comprehensive textbook of Nephrology, 4th edition: Richard J Johnson
New pathophysiological insights and treatment of ANCA-associated vasculitis
Benjamin 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