case 3 55-year man presenting with nephrotic syndrome and rprf
DESCRIPTION
Case 3 55-year man presenting with nephrotic syndrome and RPRF. Dr. Vinita Agrawal Additional Professor Department of Pathology SGPGIMS, Lucknow. Clinical presentation. 55 year gentleman Not a known diabetic/ hypertensive Edema- 1month Decreased urine output- 2weeks - PowerPoint PPT PresentationTRANSCRIPT
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Case 355-year man presenting with nephrotic syndrome and RPRF
Dr. Vinita AgrawalAdditional Professor
Department of PathologySGPGIMS, Lucknow
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Clinical presentation
• 55 year gentleman • Not a known diabetic/ hypertensive• Edema- 1month• Decreased urine output- 2weeks• Fever with chills- 2weeks• Breathlessness-2weeks• Anorexia, nausea, recurrent vomiting- 2weeks
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Clinical presentation
• No history of–Hematuria– systemic symptoms: cough, expectoration,
joint pains, rashes, sore throat
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On examination
• PR-110/min• BP- 180/90 mm Hg• Pallor+, edema+• Chest- bilateral crepitations• No organomegaly
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InvestigationsHematological
Parameter Value
Hb 8.8gm/dL
TLC 9300/dL
DLC N62L28E06M04
Platelet 1,20,000/dL
Reticulocyte 1.1%
GBP Normocytic normochromic
Biochemical
Parameter Value Normal
S. Creatinine 7.0 0.5-1.2mg/dl
S. Protein 6.9 6-8.4 g/dl
S. Albumin 3.4 3.5-5.5g/dl
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InvestigationsSerology
Parameter Value NormalS. C3 68.8 60-120
mg/dlS. C4 17.6 15-25
mg/dl
Urine examinationParameter ValueOutput 1800pH 5Protein 4+RBC’s 15-20/hpfWBC’s 12-15/hpfCulture Positive
(E.faecalis)
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Clinical diagnosis• 55 year-male patient with nephrotic range
proteinuria – Recent onset– Renal failure– Active sediments
RPRF with nephrotic range proteinuria? Crescentic GN
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Renal histology
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Salient histological features
Enlarged glomeruliDiffuse endocapillary proliferationNeutrophilic exudationWidespread splitting of GBMCapillary BM thickening (wire loop-like)Hyaline thrombiAcute tubular necrosis
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Histological diagnosis and differential diagnosis
Proliferative Glomerulonephritis(MPGN)
• MPGN type I • C3 glomerulopathy • Cryoglobulinemia• Lupus Nephritis• Secondary GN- HCV or HBV related?
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IgG (3+) IgA (4+)
C3 (4+)
IgM, C1q negative K, λ equal intensity
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EM findings
• Focal foot process effacement• Extensive electron dense deposits: sub-
endothelial and in capillary lumens• Glomerular basement membrane duplication
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Further work-up
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InvestigationsSerology
Parameter Value NormalANA negativeAnti-GBM ab. negative <15au/mlANCA negative MPO <3au/ml
PR3 <3au/ml S. Cryoglobulins negativeHIV negativeHBsAg negativeHCV negative
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Final Diagnosis• Cryoglobulinemia
x S. Cryoglobulins negativex No hyper-gammaglobulinemia
• Lupus Nephritisx No systemic manifestationsx ANA negativex Full-house on IF absent
• Secondary causes- HCV or HBV x Serology negative
• C3 glomerulopathy and dense deposit diseasex Presence of IG’s on IFx S. C4 low (marginally)x No dense deposits on ultrastructure
• MPGN type I
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Final Diagnosis
• IgA Nephropathy with MPGN-like pattern (M1E1S0T0)/HAAS Class IV ? Features
Extensive sub-endothelial depositsHyaline thrombi and wire loop-like lesions
• MPGN type I (IgA-dominant)
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IgA Nephropathy with MPGN-like pattern
• Rare; few case reports• Usually reported in children* • IgA-dominant post-infectious GN may have a
MPGN-like pattern and hyaline thrombi ǂ
* Iitaka, K. IgA-associated GN with MPGN-like pattern in 2 children. Clin.Exp. Nephrol. 2003; 7:284-9.* Akira K et al. Infantile IgAN showing features of MPGN. Tohoku J Exp. Med. 2012;228:253-58.
ǂWorawichawong S et al. IgA-dominant postinfectious GN. Hum. Pathol. 2011; 42: 279-84 Satoskar A et al. Staph. infection-associated GN mimicking IgAN . Clin J Am Soc Nephrol. 2013;1: 1179.
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• No description of MPGN-like pattern • Peripheral capillary deposits (23%) had no
prognostic significance
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Summary
• Diagnostic dilemma- IgAN with MPGN- like pattern/ MPGN (IgA-dominant)
• Limited description in literature• No association with the common secondary
causes
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Follow-up
Intensive treatment- Steroids (3 months)
RemissionMaintenance on Azoran + ACE receptor blockers• Recent visits (Feb. and August 2013)• S. creatinine- 1.2mg%, no proteinuria or active
urinary sediments