1. Case presentation Hooman N 2011

2. 10- yr-old, Girl, Khalkhal First Presentation Sep 2010 – prolonged fever, PND, anemia (Hb=10.6), AKI ( Cr=1.4  0.8)  (Tx Antibiotic) 2 nd 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

6. 3 rd ad. (march 2011) – Abdominal pain ( Dx. Acute Cholecystitis) – HB electrophoresis  nr 4 th 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 5 th ad( July 2011) – Productive cough (Dx. Sinusitis) 6 th ad(Sep2011) – Productive cough( Dx. Bronchopneumonia)  referred to our center

7. 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)

9. MRI w/wo contrast

10. 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)

11. 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)

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

13. Induction therapy Antibiotic ( Imipnem, vancomycin) Methylprednisolon Pulses (5X500mg) – Severe leukopenia – Serum Cr =0.8 – Hyperetensive IVIG ( 10 gr) – (WBC  4000  6000) Cell Cept ? – CNS lupus ( Halluciantion, blindness, severe headache) (nr BP) Cyclophosphamide ( IV, 500 mg)

15. 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