Case presentation

Hooman N2011

• 10- yr-old , Girl, Khalkhal• First Presentation Sep 2010

– prolonged fever, PND, anemia (Hb=10.6), AKI ( Cr=1.40.8) (Tx Antibiotic)

• 2nd adm. Oct– fever, Gener. LANP( reactive, EBV IgM +),– Headache + transient hemiparalysis - Abd. Sono.

- Horseshoe kidney + 2 large cyst at LT, urolithiasis- Mild splenomegaly

- sinus CT ( Max. sinusitis), Lung CT(Nr), Brain CT(- VCUG (Nr) Polycitrat K, pheobarbital, ursobil

• 3rd ad. (march 2011)– Abdominal pain ( Dx. Acute Cholecystitis)– HB electrophoresisnr

• 4th ad. (June 2011)– Abdominal pain (Dx. Pyelonephritis)– Hair loss– Bicytopenia(WBC=2300, Hb=10.4) BMA(nr)– Abdominal CT?– NCV axonal lesion of left ulnar nerve

• 5th ad( July 2011)– Productive cough (Dx. Sinusitis)

• 6th ad(Sep2011)– Productive cough( Dx. Bronchopneumonia) referred to

our center

Findings in our center– Persistent high fever (T=40C)– Productive cough– Arthralgia, severe Myalgia, muscle weakness– Genre LANP, mild Hepatosplenomegaly– Pancytopenia ( Hb=10.5, WBC=1200,

Plt=100000),ESR=90– Renal failure (Cr:2.5mg/dl),uric acid=10, UO– Oral ulcer– Severe intolerable headache (MRI)– Mood disorder, aggressive, – Episodes of blurred vision and blindness, red eye– Chest pain ( ECG ischemic changes)

MRI w/wo contrast

Lab tests

• Ca=13• P=4• Mg=1.9• AlKP=330• PTH<1• VITD=50• ACE=66 (8-52)

• C3 Low• C4 Normal• ANA(+v)• Anti ds DNA>800• C-ANCA,P-ANCA(-v)• CCP-Ab(-v)• RF-IgG(-v)

• Ferritin (High)= 2125• Fibrinogen=Nr• FDP(High)=15• D-dimer(high)= 3.1

• Wright, Coomb’s wright(-) • 2ME(-)• PCR for Mycobacteria(-)• PCR for Brucella(-)• Cultures(BM,BC,UC)(-)

• Anti- cardiolipin(IgG,IgM) (-)• Anti-PR3(-)• Anti-MPO(-)• Anti-phospholipid (IgG,IgM)(-)• Anti-RNP70(-)• Anti-SS-B(La)(-)• Anti-SS-A(RO)=131(nr<25)

Questions

• SLE or other vacuities?• What is the best induction therapy?• What is the best maintenance therapy?• What is your idea about renal biopsy?

Induction therapy• Antibiotic ( Imipnem, vancomycin)• Methylprednisolon Pulses (5X500mg)– Severe leukopenia– Serum Cr =0.8– Hyperetensive

• IVIG ( 10 gr)– (WBC 40006000)

• Cell Cept ?– CNS lupus ( Halluciantion, blindness, severe

headache) (nr BP)• Cyclophosphamide ( IV, 500 mg)

Maintenance?• Monthly CYP or Oral cell cept ??• Prednisolon 50 mg/d• Antihypertensive ( Metoprolol, ACEI, Amelodipin)• Hydrochloroquin???• -----------------------------------------------------------------• Productive cough • Abdominal pain• Severe weakness (unable to walk)• Oral ulcer• Red eye