A PREGNANT PATIENT WITH CEREBRAL LUPUS

Information about A PREGNANT PATIENT WITH CEREBRAL LUPUS

Published on December 10, 2009

Author: puguh

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A PREGNANT PATIENT WITH CEREBRAL LUPUS : , 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 INTRODUCTION : , INTRODUCTION Chronic Autoimmune Rheumatic Disease Multiorgan disease Autoantibodies CEREBRAL LUPUS SLE Slide 3: All ages , >>16 – 55 years old ♀:♂ = 8 :1 – 13 : 1 4 - 250 cases / 100.000 population Woman with SLE, the fertility is not affected SLE & Pregnancy : ↑ incidence of : flare, prematurity, intrauterine fetal growth retardation (IUGR), perinatal & maternal death 70% in SLE patient, mortality 7 – 13 % Severe to mild  myelitis / CVA to subclinic neurocognitive dysfunction SLE Cerebral Lupus SLE & Pregnancy Epidemiology Slide 4: Survival rates 90 – 95% in 2 years, 82 – 90 % in 5 years, > 81 % in 10 years. Immunosuppressive agent & corticosteroid Prognosis Slide 5: 5 Mrs N, 18 y.o married, Javanese, moslem, stay at Surabaya Admitted to dr.Soetomo hospital on Jan 21th 2009 Chief complaint : FEVER PATIENT IDENTITY CASE Slide 6: 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 2nd pregnancy Body & hinge pain (+), headache (+), oral ulcer (+), hair loss (+), facial rash (+), photosensitive (+) History of Past Ilness Family History History of diabetes mellitus, hypertension and abortus was not found 1st pregnancy : normal delivery No family with the same disease Slide 7: , 7 General condition weak; delirium; GCS 4-x-6; BP 110/80 mmHg; pulse rate 112 bpm; axillar temp 38.1°C; RR 26 x/m Head and neck : Anemia (-); icterus (-); dyspnea (-); cyanosis (-); malar rash / butterfly appearance (+); Oral ulcer (+) Thorax : no abnormality Abdominal : gravid (+) Extremities : edema -/- Physical Examination Jan 21th ,2009 Slide 8: , 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 21th ,2009 Slide 9: 9 Radiologic Imaging Slide 10: 10 Initial Assesment Cerebral lupus + S.Sepsis (unknown source of infection ) + G2P1-1 + 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 : D5 infusion :2:2 Ceftriaxone injection 2x1g Paracetamol 3x 500mg Hypokelemia correction with KCL 50mg in RL 500 cc / 24 hour Slide 11: 11 Slide 12: 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 ) + G2P1-1 + Hypokalemia P: Bedrest Enteral diet 6x150 cc Rl : D5 infusion 2:2 Ceftriaxone injection 2x1 g Pulse dose Methylprednisolon 500 mg/u in 100 cc PZ in 3 day Neurology : decline of awareness without either meningeal sign or focal neurologic deficit due to metabolic encephalopathy, Unspesific seizure possibly due to hypokalemia Adv : EEG if transportable Psychiatric : 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 Slide 13: 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, C4 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 CONSENT ) Feb 5th ,2009 (day 15) Feb 10th ,2009 (day 20) Feb 1th ,2009 (day 12) Slide 14: 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. The 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 15th ,2009 (day 25) Mar 2th ,2009 (day 35) Feb 25th ,2009 (day 30) Slide 15: Discussion sle Difficult Diagnosis Slide 16: TISSUE NECROSIS Autoreactive T cell TH cell B cell Auto Ag Auto Ab Complement Activation Platelet Agregation Hagemen factor activation VASCULITIS TROMBUS NEUROENDOCRINE (SEX HORMONE) GENETIC PATOGENESIS ENVIRONMENT (UV, INFECTION) IMMUNE COMPLEX Slide 17: COMPLEMENT ACTIVATION C3, C4 Case Case IMMUNE COMPLEX Diagnosis American Rheumatism Association (ARA ) criteria : Diagnosis American Rheumatism Association (ARA ) criteria Slide 19: , Bleeding Dysfunctional Plexus coroides Anti neural Antibody Mechanism of cerebral lupus Neuroendocrine Immune System CNS Injury Vascular Oclusion CEREBRAL LUPUS Slide 20: , Acute confusional state, cognitive dysfuntion, Psychosis Mood disorder Anxiety Movement disorder Seizure Headache (migrain intracranial hypertention ) CVA Transversal myelitis Dimyelinating syndrome Aseptic meningitis Cranial nerves neuropathy Polyneuropathy Plexopathy Focal neuropathy GBS Otonomic dysfuntion Myasthenia gravis CNS PNS Clinical Manisfestation Neuropsychiatric syndrome Slide 21: , DIAGNOSIS OF CEREBRAL LUPUS ARA Criteria Neuropsychiatric sign Laboratory test, neuropsychology test, imaging = non SLE Slide 22: LUPUS SEVERE FLARE TREATMENT FAILURE AMNIONIC FLUID INDEX <5 CM PROGRESIVE ECLAMPSIA HELLP SYNDROME SEVERE DECOMP CORDIS SEVERE TROMBOCYTOPENIA PSYCHOSOCIAL FETAL DISTRESS TERMINATION ? Flare Abortus Prematurity IUFD PREGNANCY Case SEVERE FLARE TREATMENT FAILURE AMNIONIC FLUID INDEX <5 CM Slide 23: , Cerebral lupus Lupus with pregnancy Methylprednisolon Dexametason Cyclophosphamide Prednison Prednisolon Methylprednisolon Azathioprine Cyclosporin A Low dose aspirin Pulse dose methylprednisolon 500mg iv  tapp off 1 mg / kg bb / day Cyclosphosphamide 500 mg ( informed consent ) The family refused for termination the pregnancy Case TREATMENT Slide 24: , Time & onset Disease manifestation Disease activity Treatment SLE Factors Non SLE factors PROGNOSIS OF SLE Case The cerebral lupus was getting better with the treatment (clinical sign improvement) Dubious ad bonam for the mother, but dubious ad malam for the baby Summary : , Summary A 18 year old female patient with cerebral lupus and pregnancy The diagnosis of cerebral lupus was based on anamnesis, physical examination and laboratory examination according to ARA clasification criteria which is supported by the neuropsychiatric syndrome of SLE. The Patient was planned to be terminated the pregnancy but the family refused it. The patient received medical treatment with corticosteroid (methylprednisolon) and immunosuppressive agent (cyclophosphamide) with a good respone, shown as a clinical sign improvement of cerebral lupus Slide 26: Thank you Monument of Sidoarjo city “alun – alun”

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