The 13th annual Damanhour Nephrology conference November 28-29. 2013!

2.  Female patient,22 years old , housewife , born and lives in
EL Mahmodia
 Newly Married just 1 week ago
 Patient neither diabetic nor hypertensive
 She has no special habit of medical importance
 1 year ago she said that she received steroids for 1 year
, after she consulted a nephrologist for puffy face and
lower limb edema
 Family history is irrelevant

3. Patient referred to us with elevated KFTs

4. History of Present Illness

 The condition started 3 days ago when the patient complained
of sudden sever generalized abdominal pain associated with
low grade fever and constipation
 Patient received (nonspecific) treatment without improvement
 She sought medical advice & was admitted at fever hospital for
1 day and investigations were done
 While there ,she developed anuria & repeated vomiting
 Then she was referred to us with elevated KFTs &anuria

5. Examination

 Patient conscious, alert, oriented.
 No special decubitus
 weight: 69 kg
 Heart rate: 80 Bpm, regular, equal on both sides
 Blood pressure: 160/95
 Respiratory rate: 20/minute
 Temp: 37.5 ° C
 There was pallor, no jaundice, no cyanosis.

6. Cont. Examination

 Puffy face
 Neck veins :Not congested
 Chest :Harsh vesicular breathing & no adventious sounds
& no fine basal crepitations
 Heart :Normal s1,s2 & no murmers
 Abdomen :Lax ,epigastric tenderness
abdominal stretch marks(striae)
 Lower limbs :Trivial,bilateral,pitting l.l edema
 Neurological Exam. :No focal neurological deficit.

7. investigations

8. CBC
Chemistry
HB
HCT
MCV
MCH
6 g/dl
WBCs
Lymph
Mono
Neut
7.5000 /cu mm
30 %
4%
60 %
PLT
34%
83
28
125000 /cu mm
Lipid Profile
S.Cholesterol
197
mg/dl
LDL
116
mg/dl
HDL
29
mg/dl
Triglycride
261
mg/dl
S.Creatinine
11.4
mg/dl
Bl.urea
174
mg/dl
S.Na
133
mEq/L
S.K
4.5
mEq/L
S.Calcium
8.2
mg/dl
S.Albumin
3
g/dl
corrected Ca
9
mg/dl
Po4
4.4
mg/dl
Mg
1.8
mEq/L
PTH
203
pg/ml
ALT
257
IU/L
AST
178
IU/L
T.Bil
0.5
mg/dl
D.Bil
0.2
mg/dl
Prothrombin activity
85 %

9. Urine analysis
Pus
(20-25)
RBCs
(12-15)
Glucose
(+)
Albumin
(++)
Casts
Few granular cast(+)
Crystals
Amoph urate(+)
Bilirubin
(-)
24 urinary protein
1.5 gm/24 hr

10. Immunological markers
Viral Markers
-VE
Anti_ds DNA
ANCA-P
ANCA-C
-VE
+ve
HCV Ab
-ve
HAV Ab
ANA
HBs Ag
-ve
-VE
-VE
Rh.Factor
-ve
PCR for
HBV
5.1×(10)
IU/ml
(low level)
HBe Ag
ESR
ESR 1st hr
2nd hr
PCR
78
110
-VE
HBe Ab
+VE
HBc IgM
-VE
HBc IgG
+VE

11. Cont. Investigations

Pelvi-abdominal U/S:
 Slight hepatosplenomegaly with mild pleural effusion &
mild ascites
 Both kidneys mild enlarged with bilateral symetrical
diffuse medical renoparenchymal change of type 1
nephropathy
Rt. Kidney :12.4 ×6.5 ×3.5 cm
Lt. Kidney : 11.8 ×6.2 ×3.2 cm

12. Patient started H.D sessions via internal
venous catheter

13. &
Renal Biopsy was done

14. But while Waiting result of biopsy…..

15. Cont. present history

 Patient developed compressing chest pain referred to left arm
 ECG was done and revealed antero-lateral wall ischemia
 Patient developed severe left hip joint pain with left leg
tenderness & limitation of movement .
 Patient reported numbness of both hands

16. Renal biopsy revealed

20. Renal biopsy result

21. summary

22 years old female pt.
Not DM,not HTN
HBV +VE
C/O sudden sever abdominal pain
Renal impairment(AKI)
Normocytic normochromic anemia
Chest pain developed &ECG revealed Anterolateral
ischemia
 Arthralgia of left hip joint
 Renal biopsy>>focal ischemic cortical
necrosis&hyalinization with intimal thickening of bl.vs








22. Diagnosis

23. HBV-related polyarteritis
nodosa(PAN) complicated with
acute kidney injury(AKI)

24. Management …

 Patient received 20 sessions of hemodialysis
 Patient received pulse methylprednisolone 500 mg
IV/day for 3 days
 Then continued on 60 mg prednisone/day orally for 4
weeks then tappred gradually
 Antiviral agent was begun lamivudin 150 mg /day orally

25. Follow up

 Now S.creatinine of the pateint ranges from 4.5-5.5
mg/dl without dialysis .

26. Effect of HBV on kidney

1) Most common lesion seen is Membranous Nephropathy(MN)
The lesion is a result of the deposition of IC HBe Ag-Ab complex in the B.M
(subepithelial)
2) Also it can cause MPGN
The HBs Ag-Ab complex is too large to filter through the B.M so it lodges in the
inner surface of the capillary wall (subendothlial)
3) Polyarteritis nodosa
Necrotizing vasculitis of medium sized vesseles that is not a direct form of
glomerulnephritis
3) IgA Nephropathy
In significant liver injury there is increase circulating IgA and may deposite in
the kidney
5)
FSGS: no immune complex & it may be due to deposition of HBV in the
renal tissues

27. Case Commentary
POLYARTERITIS NODOSA(PAN)

 Polyarteritis nodosa is a rare autoimmune systemic
disease featuring spontaneous inflammation of
medium & small sized arteries
 . The most common areas of involvement include the
muscles, joints, bowels, nerves, kidneys, and skin

28. causes and risk factors for
PAN

 Polyarteritis nodosa is most common in middle-age
people.
 Its cause is unknown, but it has been reported after
hepatitis B infection(30%)

29. Symptoms and Signs of PAN

 The American College of Rheumatology established
criteria for the classification of PAN in 1990
 Patient is said to have PAN if at least three of the
following 10 criteria are present:

30. Criteria of PAN

1)
Elevated kidney blood tests (BUN greater than 40 mg/dl or
creatinine greater than 1.5 mg/dl)
2)
Hepatitis B virus tests positive (for surface antigen or antibody)
3)
Arteriogram (angiogram) showing the arteries that are dilated
(aneurysms) or constricted by the blood vessel inflammation
4)
Biopsy of tissue showing the arteritis (typically inflamed
arteries)
5)
Livedo reticularis (a mottled purplish skin discoloration over the
extremities or torso)

31. Criteria of PAN

6)
Weight loss greater than or equal to 4 kg
7) Diastolic blood pressure greater than 90 mm Hg (high
blood pressure)
8) Testicular pain or tenderness (occasionally, a site
biopsied for diagnosis)
9)
Muscle pain, weakness, or leg tenderness
10) Nerve disease (either single or multiple)

32. Diagnosis:

 There is no single diagnostic test for PAN
 Tests that can confirm the diagnosis include:



Arteriogram
Tissue biopsy
Complete blood count (CBC)
Erythrocyte sedimentation rate (ESR) or C-reactive
protein (CRP)

33. Angiography

 Conventional angiography is the preferred.
 Positive findings include aneurysms and stenosis
of medium-sized vessels
 Aneurysms are most commonly found in the
kidney, liver, and mesenteric arteries, and their
presence is associated with more severe and
extensive disease

35. Biopsy

 Most accessible sites for biopsy include the skin, sural
nerve, testes, and skeletal muscle
 PAN typically affect medium sized or small arteries and
sparing arteriols , capillaries , venules & does not cause
glomerulonephritis but may cause ischemic renal injury
 Granulomatous inflammation does not occur in PAN, and
its presence suggests other diagnoses
 Kidney biopsy carries a risk of aneurysmal rupture and
bleeding

36. Our Questions Now

37. Cytotoxic drugs (cyclophosphamide)
with or against
Plasmapheresis
Role , regimen
steroid regimen
Antiviral drugs
which to give(interferon-α2b, lamivudine or entecavir)
& the regimen

38. Treatment

HBV-Related PAN
 HBV-related PAN is a special situation. Standard therapies for
PAN, including glucocorticoids and
cyclophosphamide, enhance prognosis and control of the
polyarteritis.
 Antiviral medications are essential in the treatment of these
patients
 For hepatitis B–related PAN, treatment consists of
corticosteroids for early, initial control followed by
plasmapheresis and antiviral agents.

39. Treatment (cont.)

 Stronger immunosuppression using a combinations
of steroids and cyclophosphamide is typically
avoided in these cases as it can enhance viral
replication
 Plasma exchanges are used as adjunctive therapy??
with antivirals.

40. Treatment (cont.)

 Prednisone (1 mg/kg/d) is administered for the first week.
 Alternatively, methylprednisolone pulse (15 mg/kg/d for 1-3 d) is
used in severely ill patients. Steroids are then tapered rapidly and
withdrawn at the end of 2ndweek
 Antiviral agents are begun after steroid withdrawal to enhance
immunologic clearance of HBV-infected hepatocytes and favor
seroconversion.
 Agents studied included vidarabine, which was replaced by
interferon-α2b,and later by lamivudine and recently entecavir
 It is recommended that lamivudine be continued for 6 months or
stopped at the time of seroconversion to hepatitis B surface
antibody