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PRACTICAL APPROACH TO
PATIENTS WITH NEPHROTIC
SYNDROME

 Vasant Sumethkul M.D.
 Professor of Medicine
 Ramathibodi Hospital
 Mahidol University
ตวอยางผู วย
ตัวอยางผปวย

หญิงไทยโสดอายุ 40 ป มาโรงพยาบาลดวยอาการบวม
และยุบ เปนและหาย มา 1 เดอน
        ป                   ื
มีประวัติปสสาวะเปนฟอง แตไมมีเลือด
และปริมาณปสสาวะออกดี
ไม ี ป
ไ มโรคประจํําตััว และปฏิเิ สธการทานยาเปนประจํํา
                     ป                ป ป
T 36 BP 140/90 HR 80

Not pale, no jaundice, pitting edema 4+

Heart no gallop rhythm

Lung no crepitation
L           i i

Abdomen no palpable mass, fluid thrill

Positive, no superficial vein dilatation
CBC Hb 13.0 WBC 8500 PMN 70 L30
    platelet 240,000

UA sp gr 1020, protein 4+, RBC 3-5
   WBC 0-1, oval fat body 1-2, no cast
          0 1,              1 2,
   spot UPCR = 4

TP 70 G/L, Albumin 20 G/L,
Chol
Ch l 350 mg/dl, Triglyceride 300 mg/dl
           /dl T i l     id        /dl

BUN 10 mg/dl, Serum Cr 1.2 mg/dl
What should you do next ?

1. Screen for ANA, anti DNA,C3, C4, CH 50
                 ,         , , ,

2. Bed rest and furosemide 40 mg/day
                               g y

3.
3 Start prednisolone 1 mg/kg/day

4. Collect
4 C ll t 24 h
            hour urine protein
                   i      t i
other
                       3% 3% 3% 4%            IgA
                                              IgM
           19%
                                              Membranous
                                       29%    ain
                                              cgn

          10%                                 dn
                                              fsgs
                 6%                           lupus
                      4% 1%     18%
                                              mes gn
                                              minimal change

Figure 3: Renal Histopathology of
patients with nephrotic range proteinuria (N=217)
Percent of Secondary Glomerulonephritis

                                                   post inf
    100%
                                                   lupus
     80%
                                                   henoch-sc
     60%                                           MM
     40%                                           light_c
     20%                                           DN

      0%                                           Cryo
                                                   Anti GBM
           <20     20-40          40-60      >60
                                                   Amyloidos
                           Age group (yrs)

 Figure 2: Secondary glomerulonephritis by age (n=391)
ผล   ANA negative, anti DNA negative
     C3, C4 and CH 50 within normal limit
     HBsAg negative, anti HCV negative,
           g g      ,             g   ,
     Anti HIV (with consent) negative

     CXR no pulmonary infiltration
     Stool examination ; no p
                            parasite found

     ผูปวยยังมีอาการบวม และน้าหนักขึ้น 2 กิโลกรัม ใน 1 week
        ู                      ํ
What should you do next ?

1. Bed rest and furosemide 40 mg/day
                               g y

2. Start prednisolone 1 mg/kg/day

3.
3 Advise renal biopsy

4. Repeat serum creatinine
4 R     t           ti i
Percent of Primary GN

   100%
                                              mini
    80%                                       Membranous

    60%                                       fsgs
                                              mes gn
    40%
                                              IgM
    20%                                       IgA
                                              mpgn
     0%
                                              crescentic gn
          <20    20 40
                 20-40          40 60
                                40-60   >60
                    Age group (yrs)




Figure 1: Primary glomerulonephritis by age (n=568)
The patient deny renal biopsy and
received prednisolone 50 mg/daily for 4
weeks.
How can you characterize the response
to corticosteroid ?

1.
1 Diuresis
2. Decrease body weight
3. Decrease proteinuria
3 D             t i  i
4. Increase serum albumin
5. Decrease serum cholesterol
Clinical findings that strongly against
the diagnosis of MCNS

1.   RBC cast
2.   Hypocomplementemia
3.   Gross hematuria
4.
4    Severe uncontrolled hypertension
5.   Rapidly progressive renal failure
6.
6    Organomegaly
     O           l
The patient received prednisolone 50
mg/daily for 8 weeks.
Spot UPCR decrease to 1.5
 p
Serum albumin = 25 G/L.

What should you do next ?
1.
1 Continue prednisolone 50 mg/daily to
   16 weeks
2.
2 Add cyclophosphamide 100 mg/day
           l h h id            /d
3. Add cyclosporin 150 mg/day
4. Advise renal biopsy
The patient agree for renal biopsy at

8 weeks, the results are shown here.

What is your diagnosis ?
After renal biopsy, the patient receive
prednisolone 30-40 mg/daily and
cyclophosphamide 100 mg/day for
 y p p                    g y
another 8 weeks.

At 16 weeks, proteinuria is 2+
spot UPCR = 0 5
              0.5
Serum albumin increase to 38 G/L

What should you do next ?
1. Increase prednisolone to 40 mg/day and
   continue cyclophosphamide 100 mg/day for
   another 4 weeks

2. Add cyclosporin ; keep trough level
    75-120 ng/dl

3. Continue low dose of Prednisolone and
   cyclophosphamide and add ACEI keep BP <
   130/80

4. Repeat renal biopsy
Response to therapy of MCNS

1. Complete remission:

   Proteinuria trace or negative X 2

   24 hour Proteinuria < 300 mg/day
                i   i          /

2.
2 Partial remission:

   Proteinuria < 1-2 gram/day
                     g      y

   Proteinuria < 50 % of baseline with normal

   serum albumin
Steroid non responsive (resistance) MCNS

1. Proteinuria > 3 5 gram /d after 16 weeks
1 P t i     i    3.5      /day ft        k

2.
2 Patients who do not have criteria for partial

   remission after adequate therapy
                      q          py
If renal biopsy shows FSGS :

What is the different approach ?
If renal biopsy shows
  Membranous
  glomerulonephritis :

What is the different approach ?
SUMMARY

1. The prediction of renal pathology from
   clinical setting is not accurate

2. The long term prognosis of p
          g      p g          patient with
   nephrotic syndrome is generally good

3. Over immunosuppression should be
   avoided
Thank you for your attention
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome
Practical approach to_nephrotic_syndrome

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Practical approach to_nephrotic_syndrome

  • 1. PRACTICAL APPROACH TO PATIENTS WITH NEPHROTIC SYNDROME Vasant Sumethkul M.D. Professor of Medicine Ramathibodi Hospital Mahidol University
  • 2. ตวอยางผู วย ตัวอยางผปวย หญิงไทยโสดอายุ 40 ป มาโรงพยาบาลดวยอาการบวม และยุบ เปนและหาย มา 1 เดอน ป ื มีประวัติปสสาวะเปนฟอง แตไมมีเลือด และปริมาณปสสาวะออกดี ไม ี ป ไ มโรคประจํําตััว และปฏิเิ สธการทานยาเปนประจํํา ป ป ป
  • 3. T 36 BP 140/90 HR 80 Not pale, no jaundice, pitting edema 4+ Heart no gallop rhythm Lung no crepitation L i i Abdomen no palpable mass, fluid thrill Positive, no superficial vein dilatation
  • 4.
  • 5.
  • 6. CBC Hb 13.0 WBC 8500 PMN 70 L30 platelet 240,000 UA sp gr 1020, protein 4+, RBC 3-5 WBC 0-1, oval fat body 1-2, no cast 0 1, 1 2, spot UPCR = 4 TP 70 G/L, Albumin 20 G/L, Chol Ch l 350 mg/dl, Triglyceride 300 mg/dl /dl T i l id /dl BUN 10 mg/dl, Serum Cr 1.2 mg/dl
  • 7.
  • 8. What should you do next ? 1. Screen for ANA, anti DNA,C3, C4, CH 50 , , , , 2. Bed rest and furosemide 40 mg/day g y 3. 3 Start prednisolone 1 mg/kg/day 4. Collect 4 C ll t 24 h hour urine protein i t i
  • 9.
  • 10.
  • 11.
  • 12. other 3% 3% 3% 4% IgA IgM 19% Membranous 29% ain cgn 10% dn fsgs 6% lupus 4% 1% 18% mes gn minimal change Figure 3: Renal Histopathology of patients with nephrotic range proteinuria (N=217)
  • 13. Percent of Secondary Glomerulonephritis post inf 100% lupus 80% henoch-sc 60% MM 40% light_c 20% DN 0% Cryo Anti GBM <20 20-40 40-60 >60 Amyloidos Age group (yrs) Figure 2: Secondary glomerulonephritis by age (n=391)
  • 14. ผล ANA negative, anti DNA negative C3, C4 and CH 50 within normal limit HBsAg negative, anti HCV negative, g g , g , Anti HIV (with consent) negative CXR no pulmonary infiltration Stool examination ; no p parasite found ผูปวยยังมีอาการบวม และน้าหนักขึ้น 2 กิโลกรัม ใน 1 week ู ํ
  • 15. What should you do next ? 1. Bed rest and furosemide 40 mg/day g y 2. Start prednisolone 1 mg/kg/day 3. 3 Advise renal biopsy 4. Repeat serum creatinine 4 R t ti i
  • 16. Percent of Primary GN 100% mini 80% Membranous 60% fsgs mes gn 40% IgM 20% IgA mpgn 0% crescentic gn <20 20 40 20-40 40 60 40-60 >60 Age group (yrs) Figure 1: Primary glomerulonephritis by age (n=568)
  • 17.
  • 18. The patient deny renal biopsy and received prednisolone 50 mg/daily for 4 weeks. How can you characterize the response to corticosteroid ? 1. 1 Diuresis 2. Decrease body weight 3. Decrease proteinuria 3 D t i i 4. Increase serum albumin 5. Decrease serum cholesterol
  • 19. Clinical findings that strongly against the diagnosis of MCNS 1. RBC cast 2. Hypocomplementemia 3. Gross hematuria 4. 4 Severe uncontrolled hypertension 5. Rapidly progressive renal failure 6. 6 Organomegaly O l
  • 20.
  • 21.
  • 22.
  • 23.
  • 24.
  • 25. The patient received prednisolone 50 mg/daily for 8 weeks. Spot UPCR decrease to 1.5 p Serum albumin = 25 G/L. What should you do next ? 1. 1 Continue prednisolone 50 mg/daily to 16 weeks 2. 2 Add cyclophosphamide 100 mg/day l h h id /d 3. Add cyclosporin 150 mg/day 4. Advise renal biopsy
  • 26.
  • 27.
  • 28.
  • 29. The patient agree for renal biopsy at 8 weeks, the results are shown here. What is your diagnosis ?
  • 30.
  • 31.
  • 32.
  • 33. After renal biopsy, the patient receive prednisolone 30-40 mg/daily and cyclophosphamide 100 mg/day for y p p g y another 8 weeks. At 16 weeks, proteinuria is 2+ spot UPCR = 0 5 0.5 Serum albumin increase to 38 G/L What should you do next ?
  • 34. 1. Increase prednisolone to 40 mg/day and continue cyclophosphamide 100 mg/day for another 4 weeks 2. Add cyclosporin ; keep trough level 75-120 ng/dl 3. Continue low dose of Prednisolone and cyclophosphamide and add ACEI keep BP < 130/80 4. Repeat renal biopsy
  • 35.
  • 36. Response to therapy of MCNS 1. Complete remission: Proteinuria trace or negative X 2 24 hour Proteinuria < 300 mg/day i i / 2. 2 Partial remission: Proteinuria < 1-2 gram/day g y Proteinuria < 50 % of baseline with normal serum albumin
  • 37. Steroid non responsive (resistance) MCNS 1. Proteinuria > 3 5 gram /d after 16 weeks 1 P t i i 3.5 /day ft k 2. 2 Patients who do not have criteria for partial remission after adequate therapy q py
  • 38. If renal biopsy shows FSGS : What is the different approach ?
  • 39.
  • 40.
  • 41.
  • 42.
  • 43.
  • 44.
  • 45.
  • 46.
  • 47.
  • 48. If renal biopsy shows Membranous glomerulonephritis : What is the different approach ?
  • 49.
  • 50.
  • 51.
  • 52.
  • 53.
  • 54.
  • 55.
  • 56.
  • 57. SUMMARY 1. The prediction of renal pathology from clinical setting is not accurate 2. The long term prognosis of p g p g patient with nephrotic syndrome is generally good 3. Over immunosuppression should be avoided
  • 58. Thank you for your attention