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Nephrology Case Presentation

Nephrology Case Presentation. Douglas Stahura D.O. Grandview Hospital November 20, 2001. Case Presentation. 24 y/o AAF referred by PCP c/o fatigue, periorbital edema, lower extremity edema, hematuria, proteinuria

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Nephrology Case Presentation

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  1. Nephrology Case Presentation Douglas Stahura D.O. Grandview Hospital November 20, 2001

  2. Case Presentation • 24 y/o AAF referred by PCP c/o fatigue, periorbital edema, lower extremity edema, hematuria, proteinuria • Pt relates a 5 year history of intermittent gross hematuria usually associated with “colds” • Over past four months has noticed increasing fatigue, swelling “around my eyes, especially in the morning” and swelling in the ankles and legs

  3. Case Presentation • PMH – one pregnancy, uncomplicated • PSH – none • Allergy – none • Meds – Lasix 40 mg QD • Social – married, nursing student, nonsmoker, EtOH socially, caffeine-2 cpd • Family – Father deceased age 50 MVA, Mother DM2 age 56

  4. Case Presentation • ROS • Fatigue, Weight gain 10#, No energy, poor appetite, • Swelling in feet/ankles, worse at end of the day, legs “feel heavy” • Denies CP/PALP/DOE, Cough/Sputum/SOB • Denies N/V/D, +/-Constipation • No recent UTI, hematuria

  5. Case Presentation • Exam: BP 135/85, T-98.6, P-80, R-14 • Wt-146, Ht-5’3” • NAD, pleasant, cooperative • CV,RESP,GI,MS, LYMPH – WNL • SKIN – warm/dry, 3+ pitting up to knees B/L, no rash/purpura,

  6. Case Presentation • Lab data: • Na-133, K-4.1, Cl-103, HCO3-25 • BUN-8, Cr-0.8 • CBC normal • AST-18, ALT-22, ALP-80, ALB-0.6 • UA SG-1.020, pH-5, BLO-2+, PRO-4+, GLU-neg, Ketones-Neg • CXR-normal

  7. Differential Diagnosis • Hematuria, gross • Proteinuria • Hypoalbuminuria • Nephrotic Syndrome • Nephritic Syndrome

  8. Clinical Investigation • Imaging: Renal Ultrasound • Lab: • ANA, dsDNA • ASO titer, ANCA, anti-GBM Ab • Serum/Urine Electropheresis • HBV, HCV, HIV, C3, C4, CH50 • 24 hour Urine: Protein, Creatinine • Renal Biopsy

  9. Clinical Investigation • 24 hour urine • 18 grams protein/24 hours • Creatinine Clearance 120 ml/min

  10. 00:00

  11. IgA Nephropathy • Mesangial proliferative glomerulonephritis characterized by diffuse mesangial deposition of IgA • Described by Berger in Paris 1968 • Common clinical presentation is gross hematuria provoked by mucosal infection • Diagnosis is made by Immunoflorescence

  12. IgA NephropathyPathogenesis • IgA preferentially deposits in glomerulus • Abnormality of host IgA immune system • No consistent evidence for a specific antigen • Bacterial, viral, food • Possibly autoimmune against mesangium • Antigen-independent mechanism – IgA glycosylation

  13. IgA NephropathyPathogenesis • IgA most abundant Ig in the body and provides mucosal defence • Two subclasses: IgA1, IgA2 • Mucosal Ag challenge provokes pIgA by plasma cells in MALT • Bone marrow derived mIgA1

  14. IgA NephropathyPathogenesis • In IgA nephropathy • pIgA1 is deposited in mesangium • pIgA1 is downregulated in mucosa and upregulated in the marrow • Tonsillar pIgA1 is increased • Mesangial proliferation is a result of cytokines and growth factors (PDGF, TGF-beta)

  15. IgA NephropathyClinical Presentation • Macroscopic Hematuria in 2nd & 3rd decades of life (40-50%) • “Synpharingitic” hematuria • Microscopic hematuria +- proteinuria • Nephrotic Syndrome w/ hematuria(5%) • Acute Renal Failure (rare) • Cresentic or tubular occlusion by hematuria • Chronic Renal Failure w/HTN

  16. IgA NephropathyClinical Associations

  17. Clinical POOR Increasing Age Duration of symptoms Severity of proteinuria Hypertension Renal impairment NO IMPACT Gender Serum IgA level Histopathologic POOR Glomerular sclerosis Tubule atrophy Interstitial fibrosis Vascular wall thickening Capillary loop wall IgA deposits IgA NephropathyPrognosis

  18. IgA NephropathyTherapy • Reduce IgA production • Tonsillectomy • Gluten free diet – neither reduce incidence of renal failure • Altering Immune and Inflammation • In cresentic IgAN – plasmapheresis, steroids, and cyclophosphamide – poor long term results • Steroids – alternate day regimen x 2 years • Cyclophosphamide only – no good data

  19. IgA NephropathyTherapy • Altering Immune and Inflammation • Dipyridimole and warfarin – no benefit • Cyclosporine – hemodynamic effect only • Slowly Progressive • Hypertension – Use of ACE-Inhibitor to target 125/75 will reduce proteinuria • Fish Oil – 4-8 grams/day provided renal protection from progressive disease, but did not lower proteinuria

  20. My Patient • Daily Prednisone, then to alternate day therapy – Failed, no decrement of renal function, but no improvement of proteinuria • Fish Oil – unable to comply and quit therapy after 3-4 months • Cytoxan/Methyprednisolone monthly IV pulses x 6 (Lupus Nephritis regimen) • Albumin =4.1 • clinical Nephrotic syndrome resolved

  21. References • Comprehensive Clinical Nephrology, RJ Johnson/J Feehally, Harcourt, 2000 • The Kidney, Brenner and Rector, 6th Ed, Saunders, 2000 • The long term Outcome of Patients with IgA Nephropathy Treated with Fish Oil in a Controlled Trial, Donadio et al., JASN:10;1772-1777, 1999

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