 Female patient,22 years old , housewife , born and lives in
EL Mahmodia
 Newly Married just 1 week ago

 Patient neith...
Patient referred to us with elevated KFTs
History of Present Illness


 The condition started 3 days ago when the patient complained
of sudden sever generalized a...
Examination

 Patient conscious, alert, oriented.
 No special decubitus
 weight: 69 kg
 Heart rate: 80 Bpm, regular, ...
Cont. Examination


 Puffy face

 Neck veins :Not congested
 Chest :Harsh vesicular breathing & no adventious sounds
&...
investigations
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

L...
Urine analysis
Pus

(20-25)

RBCs

(12-15)

Glucose

(+)

Albumin

(++)

Casts

Few granular cast(+)

Crystals

Amoph urat...
Immunological markers

Viral Markers

-VE

Anti_ds DNA

ANCA-P
ANCA-C

-VE

+ve

HCV Ab

-ve

HAV Ab

ANA

HBs Ag

-ve

-V...
Cont. Investigations

Pelvi-abdominal U/S:

 Slight hepatosplenomegaly with mild pleural effusion &
mild ascites
 Both ...
Patient started H.D sessions via internal
venous catheter
&
Renal Biopsy was done
But while Waiting result of biopsy…..
Cont. present history

 Patient developed compressing chest pain referred to left arm
 ECG was done and revealed antero...
Renal biopsy revealed
Renal biopsy result
summary

22 years old female pt.
Not DM,not HTN
HBV +VE
C/O sudden sever abdominal pain
Renal impairment(AKI)
Normocytic ...
Diagnosis
HBV-related polyarteritis

nodosa(PAN) complicated with
acute kidney injury(AKI)
Management …

 Patient received 20 sessions of hemodialysis
 Patient received pulse methylprednisolone 500 mg
IV/day fo...
Follow up

 Now S.creatinine of the pateint ranges from 4.5-5.5
mg/dl without dialysis .
Effect of HBV on kidney



1) Most common lesion seen is Membranous Nephropathy(MN)
The lesion is a result of the deposit...
Case Commentary
POLYARTERITIS NODOSA(PAN)



 Polyarteritis nodosa is a rare autoimmune systemic
disease featuring spont...
causes and risk factors for
PAN



 Polyarteritis nodosa is most common in middle-age
people.

 Its cause is unknown, b...
Symptoms and Signs of PAN


 The American College of Rheumatology established
criteria for the classification of PAN in ...
Criteria of PAN

1)

Elevated kidney blood tests (BUN greater than 40 mg/dl or
creatinine greater than 1.5 mg/dl)

2)

He...
Criteria of PAN

6)

Weight loss greater than or equal to 4 kg

7) Diastolic blood pressure greater than 90 mm Hg (high
b...
Diagnosis:

 There is no single diagnostic test for PAN
 Tests that can confirm the diagnosis include:




Arteriog...
Angiography

 Conventional angiography is the preferred.

 Positive findings include aneurysms and stenosis
of medium-s...
Biopsy

 Most accessible sites for biopsy include the skin, sural
nerve, testes, and skeletal muscle
 PAN typically aff...
Our Questions Now
Cytotoxic drugs (cyclophosphamide)
with or against

Plasmapheresis
Role , regimen

steroid regimen
Antiviral drugs
whi...
Treatment

HBV-Related PAN

 HBV-related PAN is a special situation. Standard therapies for
PAN, including glucocorticoi...
Treatment (cont.)

 Stronger immunosuppression using a combinations
of steroids and cyclophosphamide is typically
avoide...
Treatment (cont.)

 Prednisone (1 mg/kg/d) is administered for the first week.
 Alternatively, methylprednisolone pulse...
Case presentation fadl
Case presentation fadl
Case presentation fadl
Case presentation fadl
Case presentation fadl
Case presentation fadl
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Case presentation fadl

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

Published in: Education, Health & Medicine
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Case presentation fadl

  1. 1.  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
  2. 2. Patient referred to us with elevated KFTs
  3. 3. 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
  4. 4. 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.
  5. 5. 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.
  6. 6. investigations
  7. 7. 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 %
  8. 8. 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
  9. 9. 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
  10. 10. 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
  11. 11. Patient started H.D sessions via internal venous catheter
  12. 12. & Renal Biopsy was done
  13. 13. But while Waiting result of biopsy…..
  14. 14. 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
  15. 15. Renal biopsy revealed
  16. 16. Renal biopsy result
  17. 17. 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       
  18. 18. Diagnosis
  19. 19. HBV-related polyarteritis nodosa(PAN) complicated with acute kidney injury(AKI)
  20. 20. 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
  21. 21. Follow up   Now S.creatinine of the pateint ranges from 4.5-5.5 mg/dl without dialysis .
  22. 22. 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
  23. 23. 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
  24. 24. 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%)
  25. 25. 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:
  26. 26. 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)
  27. 27. 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)
  28. 28. 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)
  29. 29. 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
  30. 30. 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
  31. 31. Our Questions Now
  32. 32. Cytotoxic drugs (cyclophosphamide) with or against Plasmapheresis Role , regimen steroid regimen Antiviral drugs which to give(interferon-α2b, lamivudine or entecavir) & the regimen
  33. 33. 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.
  34. 34. 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.
  35. 35. 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

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