報告者： fellow 1 陳筱惠

Name: 黃 O 堯Sex: maleAge: 25-year-oldOccupation: 車床工程師Chart number: 616316Date of admission: 2011/10/07

Decreased urine output for 1 month

Underlying diseases: Chronic kidney disease (the latest crea

level: 2009/10 3.42mg/dl) Nephrotic syndrome associated with

minimal change diseaseDecreased urine output in recent 1

monthNo fever, nausea/vomiting, fatigue,

dyspnea, anorexia, dizziness

Underlying diseases: regular Nephro OPD follow up till 2009, then loss of follow up

Chronic kidney disease (the latest crea level: 2009/10 3.42mg/dl)

Nephrotic syndrome associated with minimal change disease

Hypertension dyslipidemia No diabetes mellitus, heart, liver, or

other significant systemic diseases Current medicine: nil

Allergy: no known allergyAlcohol: quitted for 4 years after

diagnosing MCDBetel-nut: deniedCigarette: 1~2 ppd/day for 9 yearsOver-the-counter medication or

chinese herb: about 2 years

Mother: Thalassemia Grandmother: chronic kidney disease,

no hemodialysis No family history of diabetes mellitus,

malignancy, bleeding diathesis, heart, liver, or hereditary diseases

Vital signs: blood pressure: 157/108mmHg; temperature: 35.8‘C; pulse rate: 86/min; respiratory rate: 18/min

General appearance: chronic ill looking Eye: conjunctiva: pale, sclera: no icteric Neck: supple, no lymphadenopathy or jugular vein

engorgement Chest: symmetric expansion

breathing sound: bilateral clear heart sound: regular heart beats, no S3 or S4,

no murmurs Abdomen: soft, flat, no tenderness, no muscle guarding or

rebounding liver/spleen: impalpable bowel sound: normoactive Extremities: no lower limb pitting edema Skin: intact, no rash

WBC 11.9x1000/ul

Hgb 7.0 g/dl

Hct 20.4 %

MCV 56.2 fl

PLT 287 x1000/uL

Segment 69.4 %

Lymphocyte 14.0 %

Monocyte 2.2 %

Eosinophil 14.0 %

Basophil 0.4 %

BUN 163.5 mg/dl

Crea 25.09 mg/dl

GPT 12 IU/L

NA 140 mEq/L

K 2.7 mEq/L

Cl 100 mEq/L

Albumin 2.45 g/dl

Ca < 5 mg/dl

PH 7.354

PCO2 28.6 mmHg

PO2 118.2 mmHg

HCO3 15.6 mm/L

SaO2 98.2 %

Color Yellow

Turbidity Cloudy

SP. Gravity 1.012

PH 6.0

Leukocyte -

Nitrite -

Protein 4+

Glucose Trace

Ketone -

Urobilinogen 0.1

Bilirulin -

Blood 2+

RBC 20 /uL

WBC 13 /uL

Epithelial cell 5 /uL

Left Kidney Length: 13.6 cm Right Kidney Length: 13.8 cm The both kidneys are large in size with

regular contour. The cortical echogenicity is slightly

increased with adequate thickness. The central sinus is unremarkable. No stone, mass, or hydronephrosis

Left Kidney Length: 11.2 cm Right Kidney Length: 11.6 cm Both kidneys are normal in size with

regular contour. The cortical echogenicity is increased

with adequate thickness. The pelvocalyceal systems are not dilated.

No obvious evidence of renal stone, mass, or cyst

MINIMAL CHANGE DISEASE 4 PIECES OF TISSUE H&E SECTIONS:

▪ 11 GLOMERULI WITH MILD MESANGIAL HYPERPLASIA ▪ INTERSTITIUM MILD CHRONIC INFLAMMATION▪ TUBULES PROTEIN CASTS ▪ PRESERVED ARTERIES

IMMUNOFLUORESCENCE SECTIONS: 13 GLOMERULI, ALL STAINS NEGATIVE

ELECTRON MICROSCOPIC STUDY:▪ ONE GLOMERULUS WITH DIFFUSE FUSION OF FOOT

PROCESSES WITH VILLOUS TRANSFORMATION▪ NO DEPOSITS

CRESCENTIC GLOMERULONEPHRITIS WITH ADVANCED SCLEROSIS

TUBULOINTERSTITIAL NEPHRITIS 3 CORES OF KIDNEY TISSUE, YELLOWISH,WHITISH,

FRAGMENTED AND SOFT H&E SECTIONS:

▪ 9 GLOMERULI 5 ARE NEARLY TOTAL OR TOTAL OBSOLETE, 3 HAVE HYPERPLASIA WITH CRESCENT AND SEVERE SCLEROSIS

▪ INTESTITIUM MARKED INFLAMMATION AND FIBROSIS COMBINED WITH MARKED TUBULAR ATROPHY

▪ THE ARTERIES AND ARTERIOLES MINIMAL CHANGES.

IMMUNOFLUORESCENCE SECTIONS: 4 GLOMERULI 2 ARE OBSOLETE, ALL NEGATIVE

10/15

RPR -

ASLO < 51.6 U/mL

A/G 1.43

PEP/IFE Protein loss or malnutrition pattern with decrease of protein and albuminNo paraprotein is identified.

IgG 507 mg/dL

IgA 191 mg/dL

IgM 124 mg/dL

IgE 165 mg/dL

C3 76.8 mg/dL

C4 20.8 mg/dL

ANA -

10/8

T-CHOL 222 mg/dL

HBs Ag -

Anti HCV Ab -

10/9

24 urine TP loss

17.34 g/day

24hr Ccr 3.67 ml/min

10/11

i-PTH 255.5 pg/mL

10/17

P-ANCA -

C-ANCA -

10/8 10/11 10/13 10/15 10/17 10/19 10/22

BUN (mg/dL)

149.6 189.7 181.1 179.2 185.8 191.7 192.1

Crea (mg/dL)

23.76 24.1 23.95 23.89 22.64 22.35 20.97

Na (mEq/L) 138 138 139 140 138 137 135

K (mEq/L) 3.1 3.8 3.0 3.3 3.0 2.7 3.2

Ca (mg/dL) < 5 < 5 < 5 5.0 5.2 < 5 5.5

P (mg/dL) 8.7 5.9 8.2 10.3 8.9 9.1 7.6

CO2 (mEq/L)

14.3 13.5 11.0 14.3 15.2 14.6

Alb (g/dL) 2.14 3.22 3.01

U/O (ml/day)

1950 2600 2930 2900 4350 3050

Solu-cortef (100mg) 1pc iv q8h 10/8~10/21

Kidney biopsy10/12

Prednisolone (5mg) 3# po bid 10/21~

Definition: presence of inflammatory infiltrates and edema within the interstitium

Acute interstitial nephritisKidney International (2010) 77, 956–961

The initial event: expression of endogenous nephritogenic antigens or exogenous antigens processed by tubular cells Tamm–Horsfall protein Megalin: a protein localized in the brush

border of proximal tubular cells Components of TBM: tubulointerstitial

nephritis antigen

Cell-mediated immunity: The inflammatory cellular infiltrates that

characterize AIN, mainly composed of T lymphocytes and macrophages.

Increase the production of extracellular matrix and the number of interstitial fibroblasts

Induce an amplification process recruiting more inflammatory cells and eosinophils into the interstitium

Profibrotic cytokines and growth factors: Transforming growth factor-b Platelet-derived growth factor-BB Endothelin-1 Epidermal growth factor Fibroblast growth factor-2

In patients with drug-induced AIN, mean delay between the starting of the offending drug and the appearance of renal manifestations is 10 days.

Early steroid treatment improves renal function recovery in patients with drug-induced acute interstitial nephritisKidney Int 2008; 73: 940–946.Acute interstitial nephritis: clinical features and response to corticosteroid therapy.Nephrol Dial Transplant 2004; 19: 2778–2783

Conservative treatment: Larger number of patients and a longer

follow-up revealed that a significant proportion of patients, ranging from not fully recovered their baseline renal function.

Duration of treatment with the offending drug or duration and severity of renal failure have not shown a clear correlation with the levels of serum creatinine at the end of follow-up.

Steroid: early use Intravenous pulses of

methylprednisolone (250 mg daily for 3 consecutive days) followed by oral prednisone (0.5–1 mg/kg/day) tapering off over 4–6 weeks

Anti-TBM disease: Plasmapheresis and cytotoxics

Idiopathic AIN resistant to steroids: cyclophosphamide, cyclosporine, mycophenolate mofetil

80-90%

Pauci-immune (scanty or absent immune deposits): antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV)

Linear deposition of IgG on the capillary wall: anti– glomerular basement membrane (GBM) disease

Immune complex glomerulonephritis with granular immune deposits: lupus nephritis, membranoproliferative nephritis, IgA nephropathy, Henoch-Schonlein purpura, postinfectious glomerulonephritis, or cryoglobulinemiaCrescentic Glomerulonephritis: New Aspects of

PathogenesisSeminars in Nephrology, Vol 31, No 4, July 2011, pp 361-368

Name: 呂 O 敏Sex: femaleAge: 41-year-oldOccupation: 家庭主婦 Chart number: 3766197Date of admission: 2011/09/24

Progressive shortness of breath for 7 days

No systemic diseases before Progressive shortness of breath for 7 days Associated symptoms: chest tightness,

increased weight and general edema (48→57kg in 1 month), decreased urine output, abdominal pain, vomiting, dizziness

No rhinorrhea, productive cough, diarrhea LMD: intermittent fever, hypoalbuminemia,

proteinuria, low C3/C4, equivocal ANA

No heart, liver, or other significant systemic diseases

Current medicine: nil

Allergy: no known allergyAlcohol: denied; betel-nut: denied;

cigarette: deniedOver-the-counter medication or

chinese herb: nil

Sister’s daughter: SLE No family history of diabetes mellitus,

malignancy, bleeding diathesis, heart, liver, kidney, or hereditary diseases

Vital signs: blood pressure: 136/77 mmHg; temperature: 37.4 ‘C; pulse rate: 95/min; respiratory rate: 18/min

General appearance: acute ill looking Eye: conjunctiva: mild pale, sclera: no icteric Face: malar rash Neck: supple, no lymphadenopathy or jugular vein

engorgement Chest: symmetric expansion

breathing sound: bilateral basal crackles heart sound: regular heart beats, no S3 or S4,

no murmurs Abdomen: soft, flat, mild low abdominal tenderness, no

muscle guarding or rebounding liver/spleen: impalpable bowel sound: normoactive Extremities: lower limb pitting edema, grade 3 Skin: intact, no rash

WBC 10.2x1000/ul

Hgb 8.3 g/dl

Hct 24.6 %

MCV 89.5 fl

PLT 286 x1000/uL

Segment 88.0 %

Band 1.0 %

Lymphocyte 6.0 %

BUN 78.7 mg/dl

Crea 2.77 mg/dl

GPT 6 IU/L

NA 139 mEq/L

K 3.9 mEq/L

Sugar 148 mg/dl

T-chol 223 mg/dl

TG 173 mg/dl

Albumin 2.22 mg/dl

CRP 13.87 mg/L

ANA 1:80 (homogenous, speckled)

C3 45.50 mg/dL

C4 17.40 mg/dL

A-DSDNA 178.9 U/mL

Color Dark yellow

Turbidity Turbid

SP. Gravity 1.037

PH 5.5

Leukocyte -

Nitrite -

Protein 4+

Glucose -

Ketone Trace

Urobilinogen 0.1

Bilirulin -

Blood 3+

Granular case +

RBC > 500/uL

WBC 5/uL

Epithelial cell 3/uL

Left Kidney Length: 12.7 cm Right Kidney Length: 12.6 cm The both kidneys are large and swollen in

appearance. The cortical echogenicity increased to the

level of liver. The papillae is prominent. The central sinus is unremarkable.

No stone, mass, or hydronephrosis is noted.

LUPUS NEPHRITIS, CLASS IV-G(A/C); ACTIVITY (7/24), CHRONICITY (1/12) GROSS D:

▪ 7 CORES OF KIDNEY TISSUE, WHITE AND SOFT

MICRO D:▪ H & E SECTIONS: 16GLOMERULI, MODERATE HYPERPLASIA WITH

NEUTROPHILS AND KARYORRHEXIS▪ THE INTERSTITIUM: MODERATE INFLAMMATORY CELLS

INFILTRATE WITH MILD FIBROSIS.▪ THE TUBULES: MILD ATROPHY WITHOUT TUBULITIS▪ THE ARTERIOLES: MIINIMAL CHANGES▪ IMMUNOFLUORESCENCE SECTIONS: 12 GLOMERULI, 4+IGG, C3

AND C1Q & 2+IGA & 1+IGM IN DIFFUSE PATTERN.▪ ELECTRON MICROSCOPIC STUDY: 2 GLOMERULUS, PROMINENT

MESANGIAL AND FOCAL SUBENDOTHELIAL DEPOSITS

9/26

RPR -

ASLO < 51.6 IU/Ml

IgG 824 mg/dL

IgA 355 mg/dL

IgM 111 mg/dL

IgE 42.7 mg/dL

Indirect Coombs

-

Coombs -

Anti-RNP 217 U/ml +

Anti-Sm 140 U/ml +

9/27

Cardiolipin IgG

6.74 U/ml

Cardiolipin IgM

6.01 U/ml

9/26

24 urine TP loss

2.1 g/day

24hr Ccr 4.75 ml/min

9/27

P-ANCA -

C-ANCA -

9/29

Cryoglobulin IgG 1+

Haptoglobin 7.21 mg/dL

9/26 9/29 10/1 10/3 10/6 10/12

BUN (mg/dL)

113.1 121.2 98.0 156.4 73.1 72.2

Crea (mg/dL)

5.33 8.08 7.32 10.27 4.87 1.60

Na (mEq/L)

144 141 138 131 137 139

K (mEq/L) 4.3 5.6 5.8 5.1 4.4 3.8

Ca (mg/dL)

6.8 7.0 8.2 7.0 8.6 8.3

P (mg/dL) 7.0 10.4 9.0 8.7 5.7 3.3

CO2 (mEq/L)

21.8 20.4 23.5 29.9

Alb (g/dL) 2.20 2.95

U/O (ml/day)

40 90 0 50 20 1410

BW (kg) 55.4 49.8 49.1 51.6 50.62 47.6

Hemodialysis 9/26~10/10

Solu-cortef 100mg 1pc iv q8h 9/26~10/6

Solu-medrol (500mg) 1pc ivf qd 10/6~10/8

Prednisolone (5mg) 4# po qd10/8~

Kidney biopsy 10/7

No outcome difference

intraglomerular distribution

10-year follow-up evaluation 24 SLE patients with active segmental GN in 50%

or more of the glomeruli 35 SLE patients with diffuse GN

The incidence of end stage renal disease was significantly greater in patients with segmental GN of 50% or more compared with those with diffuse GN (9/24 [38%] versus 5/35 [14%], P < .05)

The incidence of remission with stable renal function was greater in patients with diffuse GN (22/35 [63%] versus 9/24 [38%], P < .05).

Significance of histologic patterns of glomerular injury upon long-term prognosis in severe lupus glomerulonephritis.Kidney Int. 2001;59:2156-63.

Pathology of lupus nephritisSeminars in Nephrology, Vol 27, No 1, January 2007, pp 22-34