A Pregnant Patient With Cerebral Lupus

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”