A pregnant 18-year-old female patient presented with cerebral lupus and was admitted to the hospital with fever and inability to speak or stand. She was diagnosed with cerebral lupus based on meeting ARA criteria and exhibiting neuropsychiatric symptoms of SLE. Her pregnancy was considered high-risk due to a severe lupus flare and low amniotic fluid index, but termination was refused by her family. She was treated with corticosteroids and cyclophosphamide, showing improvement in her cerebral lupus symptoms.

1. A PREGNANT PATIENT WITH CEREBRAL LUPUS Puguh Widagdo Joewono Soeroso , CASE PRESENTATION Department of Internal Medicine Dr Soetomo Teaching Hospital- Airlangga University School of Medicine SURABAYA

5. Mrs N, 18 y.o married, Javanese, moslem, stay at Surabaya Admitted to dr.Soetomo hospital on Jan 21 th 2009 Chief complaint : FEVER PATIENT IDENTITY CASE

6. History of Present Ilness High fever since 5 days before admission, shiver (-), not getting better with fever lowering agent . Suddendly 1 day before admission, the patient became silent couldn’t speak and stand. Patient was in 3 months of the 2 nd pregnancy Body & hinge pain (+), headac h e (+), oral ulcer (+), hair loss (+), facial rash (+), photosensitive (+) History of Past Ilness Family History History of diabetes mellitus, hypertension and abortus was not found 1 st pregnancy : normal delivery No family with the same disease

8. , Laboratory Examination Hb 11,2 g/dl ; WBC 14000/l ; PLT 124.000 u/l ; HCT 35,3 %; RBS 104 mg/dl; BUN 31 mg/dl; Cr 1.7 mg/dl; SGOT 30 u/l; SGPT 24 u/l Total prot. 5.5 g/dl; Albumin 2.9 g/dl ; Total bil 0.8 mg/dl; direct bil 0.2 mg/dl; Potassium 2,67 mmol/l; Sodium 136,7 mmol/l. Blood gas analysis (BGA): pH 7,38; pCO2 20 mmHg; pO2 107 mmHg; HCO3 11,8mmol/l; BE -13mmol; SO2 98 % Jan 21 th ,2009

9. Radiologic Imaging Conclusion : No abnormality Jan 21 th ,2009 Chest x-ray Conclusion : No abnormality Ct – Scan Skull Conclusion : No abnormality Feb 5 th ,2009 Obstetry USG Conclusion : BP 38, FL 21, amnion fluid ( - ) Feb 10 th ,2009

10. Initial Assesment Cerebral lupus + S.Sepsis (unknown source of infection ) + G 2 P 1-1 + Hypokalemia Planning : Urinalysis and urine sediment, ANA test, CRP, C3, C4 Blood and urine culture DX: TX: O2 4 lpm Bed rest Enteral diet HCHP 6x150 cc RL : D 5 infusion :2:2 Ceftriaxone injection 2x1g Paracetamol 3x 500mg Hypokelemia correction with KCL 50mg in RL 500 cc / 24 hour

12. Jan 22 nd ,2009 (day 2) S: Agitation scream, delirium (GCS 4x6), weak . ESR 50 mm/hour SGOT 45 u/l, SGPT 42 u/l, BUN 30 mg/dl, Cr 1,7 mg/dl, Na 136,7 mmol/ L K 2,35 mmol/L O: T 120/80, HR 96 bpm RR 25 x/m , t 37,8 ºC A: Cerebral lupus + suspected sepsis (un known source of infection ) + G 2 P 1-1 + Hypokalemia P: Bedrest Enteral diet 6x150 cc Rl : D 5 infusion 2:2 Ceftriaxone injection 2x1 g Pulse dose Methylprednisolon 500 mg/u in 100 cc PZ in 3 day Neurolog y : decline of awareness without e ither meningeal sign or focal neurologic deficit due to metabolic encephalopathy , Unspesific seizure possibly due to hypokalemia Adv : EEG if transportable Psychia tric : mental organic disorder due to SLE Adv : treat organic cause and Haloperidol 2 x 0,5 mg or Haloperidol injection (1/2 ampul i.v) if agitated

13. Jan 25 th ,2009 (day 5) Agitation Scream, Weak GCS 224, ANA Test moderate (+) (45 UNIT) BUN 29 mg/dl Cr 0,73 mg/dl, K 2,9 mmol/l, C3 3 mg/dl, C 4 13 mg/dl Blood Culture : Staphylococcus coagulase negative, sensitive with sefoperazone-sulbactam Dx : CEREBRAL LUPUS + SEPSIS + G2P1-1 + HYPOKALEMIA Tx : ~ 2nd day, Methylprednisolon 60 mg iv, Sefoperazone – Sulbactam Injection 3x1g Blood culture : Corynebacterium spp (+), sensitive with Ceftazidime Tx :~ 5th day, Ceftazidime inj 3x1g SKULL CT-SCAN WITHOUT CONTRAST : NO ABNORMALITY ABDOMINAL USG : AMNIOTIC FLUID (-) TX : ~ 15TH DAY, CYCLOFOSFAMIDE DRIP 500 mg IN 500CC PZ IN 5 HOUR ( INFORMED CON S ENT ) Feb 5 th ,2009 (day 15) Feb 10 th ,2009 (day 20) Feb 1 th ,2009 (day 12)

14. Conference with Obgyn Dept : Considering severe flare of SLE ( cerebral lupus ) which is unresponsive with the treatment and amniotic fluid index (-). The pregnancy should be terminated . T he family refuse it. Patient could eat and drink minimally , GCS 456 Patient could eat and drink minimally, minimal daily activity GCS 4-5-6 Discharge from hospital terapy : Methylprednisolon 8mg-0-0 Chloroquinolon 1 x 100 mg Ca2 vit D31x1 Feb 15 th ,2009 (day 25) Mar 2 th ,2009 (day 35) Feb 25 th ,2009 (day 30)

15. Discussion

16. TISSUE NECROSIS Autoreactive T cell TH cell B cell Auto Ag Auto Ab Complement Activation Platelet Agregation Hagemen factor activation VAS C ULITIS TROMBUS NEUROENDOCRINE (SEX HORMONE) GENETIC PATOGENESIS ENVIRONMENT (U V, INFECTION ) IMMUNE COMPLEX

18. Diagnosis American Rheumatism Association (ARA ) criteria REF CASE REF CASE Malar rash (+) Renal disorder (-) Discoid rash (-) Neurologic disorder (+) Photosensitivity (+) Hematologic disorder (-) Oral ulcer (+) Immunologic disorder Anti ds DNA Anti SM Not Examined Arthritis (+) Serositis (-) Antinuclear antibody ANA - test (+)

19. , Bleeding D y sfunctional Plexus coroides Anti neural Antibody Mechanism of cerebral lupus Neuroendocrine Immune System CNS Injury Vascular Oclusion CEREBRAL LUPUS

21. , DIAGNOSIS OF CEREBRAL LUPUS ARA Criteria Neuropsychiatric sign Laboratory test, neuropsyc h ology test, imaging = non SLE

26. Thank you Monument of Sidoarjo city “ alun – alun”