nephrotic syndrome 01.10.09.ppt - siriraj hospital · practical approach to patients with nephrotic...

PRACTICAL APPROACH TO PATIENTS WITH NEPHROTIC SYNDROME

Vasant Sumethkul M.D.Professor of MedicineRamathibodi HospitalRamathibodi HospitalMahidol University

ตัวอยางผปวยตวอยางผูปวย

หญิงไทยโสดอายุ 40 ป มาโรงพยาบาลดวยอาการบวม

ป 1 ื และยุบ เปนและหาย มา 1 เดอน

มีประวัติปสสาวะเปนฟอง แตไมมีเลือด

และปริมาณปสสาวะออกดี

ไ โี ป ํ ั ป ิ ป ป ํไมมโีรคประจําตัว และปฏิเสธการทานยาเปนประจํา

T 36 BP 140/90 HR 80

Not pale, no jaundice, pitting edema 4+Not pale, no jaundice, pitting edema 4+

Heart no gallop rhythmHeart no gallop rhythm

L i iLung no crepitation

Abdomen no palpable mass, fluid thrill

Positive, no superficial vein dilatation

CBC Hb 13.0 WBC 8500 PMN 70 L30platelet 240,000

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

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

3 Start prednisolone 1 mg/kg/day3. Start prednisolone 1 mg/kg/day

4 C ll t 24 h i t i4. Collect 24 hour urine protein

3% 4%3% 3%

other

IgA4%

19%

3% IgA

IgM

Membranous

29%

Membranous

ain

10%

cgn

dn

18%1%4%6%

fsgs

lupus18%1%4%

mes gn

minimal change

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

Percent of Secondary Glomerulonephritis

100%post inf

lupus

60%

80%henoch-sc

MM

20%

40% light_c

DN

0%

20%

Cryo

Anti GBM<20 20-40 40-60 >60

AmyloidosAge group (yrs)

Figure 2: Secondary glomerulonephritis by age (n=391)

ผล ANA negative, anti DNA negativeC3, C4 and CH 50 within normal limitHBsAg negative, anti HCV negative,g g , g ,Anti HIV (with consent) negative

CXR no pulmonary infiltrationStool examination ; no parasite found; p

ผูปวยยังมีอาการบวม และน้าํหนักขึ้น 2 กโิลกรัม ใน 1 weekู

What should you do next ?

1. Bed rest and furosemide 40 mg/dayg y

2. Start prednisolone 1 mg/kg/day2. Start prednisolone 1 mg/kg/day

3 Advise renal biopsy3. Advise renal biopsy

4 R t ti i4. Repeat serum creatinine

Percent of Primary GN

80%

100%mini

60%

80% Membranous

fsgs

mes gn

20%

40%mes gn

IgM

IgA

0%

<20 20-40 40-60 >60

mpgn

crescentic gn<20 20 40 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 ?to corticosteroid ?

1 Diuresis1. Diuresis2. Decrease body weight3 D t i i3. Decrease proteinuria4. Increase serum albumin5. Decrease serum cholesterol

Clinical findings that strongly against the diagnosis of MCNS

1. RBC cast2. Hypocomplementemia2. Hypocomplementemia3. Gross hematuria4 Severe uncontrolled hypertension4. Severe uncontrolled hypertension5. Rapidly progressive renal failure6 O l6. Organomegaly

The patient received prednisolone 50 mg/daily for 8 weeks. Spot UPCR decrease to 1.5pSerum albumin = 25 G/L.

What should you do next ?1 Continue prednisolone 50 mg/daily to1. Continue prednisolone 50 mg/daily to

16 weeks2 Add l h h id 100 /d2. Add cyclophosphamide 100 mg/day3. Add cyclosporin 150 mg/day4. 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 yanother 8 weeks.

At 16 weeks, proteinuria is 2+spot UPCR = 0 5spot UPCR = 0.5Serum 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

i i /24 hour Proteinuria < 300 mg/day

2 Partial remission:2. Partial remission:

Proteinuria < 1-2 gram/dayg y

Proteinuria < 50 % of baseline with normal

serum albumin

Steroid non responsive (resistance) MCNS

1 P t i i > 3 5 /d ft 16 k1. Proteinuria > 3.5 gram /day after 16 weeks

2 Patients who do not have criteria for partial2. Patients who do not have criteria for partial

remission after adequate therapy q py

If renal biopsy shows FSGS :If renal biopsy shows FSGS :

What is the different approach ?

If renal biopsy showsIf renal biopsy shows Membranous glomerulonephritis :

What is the different approach ?What is the different approach ?

SUMMARY

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

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

3. Over immunosuppression should be avoided

Thank you for your attention