case presentation staci smith do gvh nephrology. case presentation 44-year-old white right-handed...
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Case Presentation
Staci Smith DO
GVH Nephrology
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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
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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
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Case Presentation
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
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Case Presentation
Positive ROS : Recent URI two weeks ago 10 pound weight gain with LE edema Hematuria Foamy urine Abdominal pain - Zantac
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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.
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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
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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
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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
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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
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Differential Diagnosis
hematuria proteinuria glomerulonephritis red blood cell casts
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Red Blood Cell Casts
Glomerular hematuria Dysmorphic rbc’s
glomerular damage rule out urologic causes
Nephritic syndrome HTN RBC casts Proteinuria Edema
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Differential Diagnosis: Glomerulonephritis 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
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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
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Differential Diagnosis: Glomerulonephritis 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
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Renal Biopsy : Ig A Nephropathy Light microscopy - Mesangial hypercellularity
IgA is predominantly polymeric IgA1 mainly derived from the mucosal
immune system
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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
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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
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IgA Deposition is Common
IgA deposition in other forms of glomerulonephritis thin basement membrane nephropathy lupus nephritis minimal change disease diabetic nephropathy
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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
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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
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IgA Nephropathy
EM electron dense deposits that are limited to the
mesangial regions
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IgA Nephropathy
mesangial glomerulonephritis showing segmental areas of increased mesangial matrix and cellularity
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Conditions associated with IgA nephropathy
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
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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.
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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
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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
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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
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Ig A Pathophysiology
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
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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
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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
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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)
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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
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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
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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
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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
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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
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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
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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
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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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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