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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
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Case Conference 02/14/2014 YuvarajThangaraj, 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 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 • IV Drug abuse • Tobacco abuse • 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
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 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