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Case Presentation

Case Presentation. Staci Smith DO GVH Nephrology. Case Presentation. 44-year-old white right-handed male that presented to GVH with complaints of hematuria had associated abdominal pain and took a Zantac for it no history of known CKD and has not actually seen a doctor in 25 years

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Case Presentation

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  1. Case Presentation Staci Smith DO GVH Nephrology

  2. Case Presentation • 44-year-old white right-handed male that presented to GVH with complaints of hematuria • had associated abdominal pain and took a Zantac for it • no history of known CKD and has not actually seen a doctor in 25 years • no history of other hematuria,prostate trouble, dysuria, nocturia,incontinence • not on any outpatient anticoagulation • 20 years of tobacco abuse

  3. Case Presentation • recent upper respiratory infection 2 weeks ago with fevers and chills • foamy urine • denies any recent known UTI, over-the-counter NSAIDs, history of nephrolithiasis, contrasted procedure, physical exercise

  4. PAST MEDICAL HISTORY: OA Tobacco abuse URI 2 weeks ago PAST SURGICAL HISTORY: Right knee scope ALLERGIES: None FAMILY HISTORY: Htn No medical renal dz MEDS: None SOCIAL HISTORY: Cleans gutters / construction work Positive tobacco 1-2 ppd x 20 yrs Social EtOh only Case Presentation

  5. Case Presentation • Positive ROS : • Recent URI two weeks ago • 10 pound weight gain with LE edema • Hematuria • Foamy urine • Abdominal pain - Zantac

  6. Case Presentation : Physical Exam • VS:126/87- 97.9 F (T-max 99.2)- HR 84- RR 20- 96%sat • GEN-Awake, alert and oriented x3. Family is at bedside and updated. No acute distress. • HEENT: Poor dentition. Atraumatic, normocephalic. Extraocular muscles intact. Sclerae are anicteric. Mucous membranes are now moist • CHEST: Respirations clear to auscultation bilaterally. No wheezing, rhonchi or crackles • ABDOMEN: Soft, nontender, nondistended. Positive bowel sounds. No perotoneal signs or Foley • EXTREMITIES: No clubbing, cyanosis. Plus 2 pitting edema. There is no asterixis • SKIN: There is no rash or cellulitis • NEUROLOGIC: Cranial nerves II-XII grossly intact.

  7. Case Presentation : Labs • 133 109 13 122 CCa 9.6 Alb 1.6 5 22 2.0 No Phos or Mg UA is cloudy with positive nitrite, large blood, 300 protein, too numerous to count red cells, trace bacteria, 1-5 white cells

  8. Case Presentation : Labs • CT of the abdomen and pelvis was negative for any stone, multiple loops of fluid-filled nondilated small bowel without obstruction or contracted GB • Urine Pr/ Cr ratio- 10 • CPK was negative at 148. • LFTs normal. Amylase and lipase are normal. • TSH is 3.03 • Total cholesterol 277 • total triglycerides 264, HDL 26, LDL 198 • 7.8 11.3 165 S 79-L 11-M 8 – E 2 33.6

  9. Case Presentation : Labs • All negative • Complements • ANA, dsDNA • Hepatitis profile • SPEP/ UPEP with IFE • Rheumatoid factor/ Anti CCP • Anti GBM • P and C ANCA’s • HIV • ASO

  10. Differential Diagnosis • Back to our case • 44 yo with hematura, proteinuria, and lower extremity edema two weeks after an URI • No previous know CKD or AKI • Cr now 2.0 • 10 grams proteinuria • Hyperlipidemia

  11. Differential Diagnosis • hematuria • proteinuriaglomerulonephritis • red blood cell casts

  12. Red Blood Cell Casts • Glomerular hematuria • Dysmorphic rbc’s • glomerular damage • rule out urologic causes • Nephritic syndrome • HTN • RBC casts • Proteinuria • Edema

  13. Postinfectious GN IgA nephropathy Thin basement membrane Henoch-Schönlein purpura Mesangial proliferative GN SLE Goodpasture’s dz Vasculitis Wegener’s, Churg-Strauss Cryoglobulinemia HIV Membranoproliferative glomerulonephritis Rapidly progressive GN Fibrillary glomerulonephritis Focal glomerulosclerosis Membranous nephropathy Amyloidosis Multiple Myeloma DM HUS Differential Diagnosis: Glomerulonephritis

  14. Differential Diagnosis: Glomerulonephritis • UA with C and S • Cbc with differential • Renal panel • Urine Pr/Cr • Renal US • Renal Duplex • Cystography • Urine eosinophils • SPEP / UPEP with IFE

  15. ANA, dsDNA SLE Cryoglobulins, RF Cryoglobulinemia, HCV Anti-GBM Anti-GBM dz Goodpasture’s ANCA’s c-ANCA: Wegener’s p-ANCA: PAN, Churg Strauss, MPA Complements C3, C4, CH50 ASO Post Strept GN Hepatitis profile MPGN HIV HIV, FSGS Renal Biopsy Differential Diagnosis: Glomerulonephritis

  16. Renal Biopsy : Ig A Nephropathy • Light microscopy - Mesangial hypercellularity • IgA is predominantly polymeric IgA1 • mainly derived from the mucosal immune system

  17. IgA Nephropathy : Berger’s Disease • the most common lesion found to cause primary glomerulonephritis • peak incidence in the second and third decades of life • 2:1 male to female • greatest frequency in Asians and Caucasians • relatively rare in blacks

  18. IgA Nephropathy • large undiagnosed "latent" IgA nephropathy in the general population • the process of mesangial IgA deposition is likely to be separate from the induction of glomerular injury • IgA deposition does not necessarily need to be followed by nephritis

  19. IgA Deposition is Common • IgA deposition in other forms of glomerulonephritis • thin basement membrane nephropathy • lupus nephritis • minimal change disease • diabetic nephropathy

  20. IgA Nephropathy • Many patients are detected on routine urine screening • asymptomatic hematuria and/or proteinuria • higher prevalence • active urine testing program • low threshold for renal biopsy

  21. IgA Nephropathy • IgA nephropathy is established only by kidney biopsy • Immunofluorescence microscopy • demonstrating large, globular mesangial IgA deposits • also seen with HSP • IgA often accompanied by C3 and IgG in the mesangium

  22. IgA Nephropathy • EM • electron dense deposits that are limited to the mesangial regions

  23. IgA Nephropathy • mesangial glomerulonephritis showing segmental areas of increased mesangial matrix and cellularity

  24. Idiopathic (most cases) Hepatic cirrhosis Gluten enteropathy HIV infection Minimal change disease Membranous GN Wegener’s granulomatosis Dermatitis herpetiformis Seronegative arthritis - eg, ankylosing spondylitis Small cell carcinoma Disseminated tuberculosis Mycosis fungoides Conditions associated with IgA nephropathy

  25. IgA Nephropathy • Initiating event in the pathogenesis is the mesangial deposition of IgA • Codeposits of IgG and complement • commonly seen • may contribute to disease severity • Between episodes of gross hematuria • persistent microhematuria, proteinuria, or both. • Gross hematuria has also followed • tonsillectomy, vaccinations, strenuous physical exercise, and trauma.

  26. Increased Plasma IgA Levels • Not alone is not sufficient to produce mesangial IgA deposits • Found in 50% of cases • IgA is probably accumulated and deposited because of a systemic abnormality rather than a defect intrinsic to the kidney

  27. IgA Nephropathy • Two common presentations • episodic gross hematuria • 40-50% • upper respiratory tract infection, or, less often, gastroenteritis • persistent microscopic hematuria • 30-40% • asymptomatic, with erythrocytes (RBCs), RBC casts, and proteinuria discovered on urinalysis

  28. IgA Nephropathy • Nephrotic range proteinuria is uncommon • occurring in only 5% of patients • Indicates more advanced disease • Approximately 1-2% of all patients with IgA nephropathy develop ESRD each year • Hypertension seldom occurs at the time of initial presentation

  29. Platelet Derived Growth Factors Made by mesangial cells Increased PDGG receceptors in glomerular dz Infusion of glomerular transfection with PDGF leads to mesangial proliferation TGF- beta Made by mesangial cells Pro fibrotic Antiinflammatory and immunosuppresive Ig A Pathophysiology

  30. Morbidity and Mortality • follow a benign course in most cases • at risk for slow progression to ESRD • approximately 15% of patients by 10 years • 20% by 20 years

  31. Ig A Nephropathy Outcomes • 20-30% progress to ESRD over 20 years • 1-2% per year • Clinical predictors of poor renal outcome • Absence of gross hematuria • Male • Older onset age • HTN • Heavy proteinuria • Elevated Cr >2-2.5

  32. Ig A Nephropathy Outcomes • Therapy remains to be defined • Antibiotics • Tonsillectomy • Cyclophosphamide, dipyridamole • High dose immunoglobulin therapy • Statins • Fish Oils • Ace inhibition • Cellcept (mycophenolate mofetil)

  33. Ig A Nephropathy Outcomes • Ace inhibitors • Effectively reduce proteinuria in glomerular dz • ACE-I better than other anti – HTN meds to preserve GFR in Ig A • Questionable addititive effect with ARBS

  34. Ig A Nephropathy Outcomes • Fish Oil • Meta analysis concluded there may be a minor benefit in heavy proteinuria • Dillon 1997 JASN • Low does omega 3 fatty acids as effective as high dose • Cellcept • Inhibits de novo guanosine nucleotide synthesis • Established use for transplant • Not that much improvement for Ig A

  35. Ig A Treatment Summary • If Uprot <0.5 g/d and CrCl >70 • Observe and consider ACEI or ARB • If Uprot > 0.5 g/d and Cr Cl > 70 • ACEI/ARB for target bp 125/75 • If Uprot 1-3 g/d with Cr Cl >70 • Maximal ACEI/ ARB • Consider 6 months of high dose steroids and taper for 6 mo • If Uprot >3 g/d and CrCl <70 or declining • Steroids plus Cytotoxics • Possible maintenance with AZA or MMF

  36. Transplant Recipients • high recurrence rate in renal transplant recipients who have IgA nephropathy • 25-60% • Increased risk of allograft loss in living related donor • disappearance of the deposits from donor kidneys with IgA nephropathy when transplanted into donors without the disease

  37. Patient Progression • Cr continued to worsen with disease progression • March Cr: 2.0 • April Cr: 3.09 – 4.2 • Initiation of Cytoxan and Steroids ( 2 cycles) • ACEI caused hyperkalemia • Fish Oil, BB, Lasix , Zaroxolyn, Statin, PPI, Oscal • May Cr :5.2 • June 18th: 8.76 • ESRD with hemodialysis initiation • Uncontrollable edema and pulmonary edema despite diuretics

  38. Question 1 • Which of the following is the most predictive for progression of Ig A • A- elevated levels of IgA • B- elevated Cr at baseline diagnosis • C- male gender • D- absence of gross hematuria

  39. Question 1 • Which of the following is the most predictive for progression of Ig A • A- elevated levels of IgA • B- elevated Cr at baseline diagnosis • C- male gender • D- absence of gross hematuria

  40. Question 2 • You are seeing a 30yr Asian woman with bx proven Ig A. Her Uprotein is 3.5g/d despite maximal ACEI, Bp is 100/70, Cr stable at 1.6 for the past year. Diffuse foot process effacement is seen on EM. What is the next step for management? • A- Add ARB • B- Add MMF • C- Add steroids • D- Add fish oils • E-Tonsillectomy

  41. Question 2 • You are seeing a 30yr Asian woman with bx proven Ig A. Her Uprotein is 3.5g/d despite maximal ACEI, Bp is 100/70, Cr stable at 1.6 for the past year. Diffuse foot process effacement is seen on EM. What is the next step for management? • A- Add ARB • B- Add MMF • C- Add steroids • D- Add fish oils • E-Tonsillectomy

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