Case PresentationBy

Ahmed Ezzat Fadl

Resident of Nephrology Damanhour Medical National Institute

( D.M.N.I )

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

Patient referred to us with elevated KFTs

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

History of Present Illness

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.

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.

Cont. Examination

investigations

6 g/dl34%8328

HBHCTMCVMCH

7.5000/ cu mm30% 4%

60%

WBCsLymphMonoNeut

125000 /cu mm PLT

11.4 mg/dl S.Creatinine

174 mg/dl Bl.urea

133 mEq/L S.Na

4.5 mEq/L S.K

8.2 mg/dl S.Calcium

3 g/dl S.Albumin

9 mg/dl corrected Ca

4.4 mg/dl Po4

1.8 mEq/L Mg

203 pg/ml PTH197 mg/dl S.Cholester

ol

116 mg/dl LDL

29 mg/dl HDL

261 mg/dl Triglycride

257 IU/L ALT

178 IU/L AST

0.5 mg/dl T.Bil

0.2 mg/dl D.Bil

85% Prothrombin activity

CBC Chemistry

Lipid Profile

Urine analysis

(20-25)

(12-15)

(+)

(++)

Few granular cast(+)

Amoph urate(+)

(-)

Pus

RBCs

Glucose

Albumin

Casts

Crystals

Bilirubin

24 urinary

protein

1.5 gm/24 hr

-VE ANA

-VE Anti_ds DNA

-VE-VE

ANCA-PANCA-C

-ve Rh.Factor

+ve HBs Ag

-ve HCV Ab

-ve HAV Ab

5.1(×10) IU/ml

(low level)

PCR for HBV

78110

ESR 1st hr 2nd

hr

Viral MarkersImmunological markers

PCR

ESR-VE HBe Ag

+VE HBe Ab

-VE HBc IgM

+VE HBc IgG

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

Cont. Investigations

Patient started H.D sessions via internal venous catheter

Renal Biopsy was done &

But while Waiting result of biopsy..…

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

Cont. present history

Renal biopsy revealed

Renal biopsy result

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

summary

Diagnosis

HBV-related polyarteritis

nodosa(PAN) complicated

with acute kidney injury(AKI)

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

Management…

Now S.creatinine of the pateint ranges from 4.5-

5.5 mg/dl without dialysis .

Follow up

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

4) IgA NephropathyIn 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

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

Case CommentaryPOLYARTERITIS NODOSA(PAN)

Polyarteritis nodosa is most common in

middle-age people.

Its cause is unknown, but it has been reported

after hepatitis B infection(30%)

causes and risk factors for 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:

Symptoms and Signs 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)

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)

Criteria of PAN

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)

Diagnosis:

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

Angiography

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

Biopsy

Our Questions Now

Cytotoxic drugs (cyclophosphamide)

with or against

Plasmapheresis

Role , regimen

steroid regimen

Antiviral drugs

which to give(interferon-α2b, lamivudine or entecavir)

& the regimen

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.

Treatment

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.

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

Treatment (cont.)

Thank You