A PREGNANT PATIENT WITH CEREBRAL LUPUS Puguh Widagdo Joewono Soeroso , CASE PRESENTATION Department of Internal Medicine D...
INTRODUCTION <ul><li>Chronic Autoimmune Rheumatic Disease </li></ul>, Multiorgan  disease Autoantibod ies CEREBRAL LUPUS S...
<ul><li>All ages  ,  >>16 – 55 years old </li></ul><ul><li>♀ :♂ =  8 :1 – 13 : 1 </li></ul><ul><li>4 - 250 cases / 100.000...
<ul><li>Survival rates  </li></ul><ul><ul><li>90 – 95% in 2 years,  </li></ul></ul><ul><ul><li>82 – 90 % in 5 years,  </li...
Mrs N, 18 y.o married, Javanese,  moslem, stay at Surabaya Admitted to  dr.Soetomo  hospital on Jan 21 th  2009 Chief comp...
History of Present Ilness High fever since 5 days before admission, shiver (-), not getting better with fever  lowering   ...
, <ul><li>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...
, 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 ...
Radiologic Imaging Conclusion : No abnormality Jan 21 th  ,2009 Chest x-ray   Conclusion : No abnormality Ct – Scan Skull ...
Initial Assesment Cerebral lupus + S.Sepsis (unknown source of infection ) +  G 2 P 1-1  + Hypokalemia Planning : Urinalys...
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, BU...
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,  ...
Conference with Obgyn Dept :   Considering   severe flare of SLE ( cerebral lupus ) which is unresponsive with the treatme...
Discussion
TISSUE  NECROSIS Autoreactive  T cell TH cell B cell Auto Ag Auto Ab Complement Activation Platelet Agregation Hagemen  fa...
COMPLEMENT  ACTIVATION C3, C4 Case Case IMMUNE COMPLEX <ul><li>Febris  </li></ul><ul><li>Leukocytosis </li></ul><ul><li>↑ ...
Diagnosis American Rheumatism Association  (ARA ) criteria REF CASE REF CASE Malar rash (+) Renal disorder (-) Discoid ras...
, Bleeding D y sfunctional Plexus coroides Anti neural  Antibody Mechanism of cerebral lupus Neuroendocrine Immune System ...
, <ul><li>Acute  confusional  state,  c ognitive  d ysfuntion, Psyc h osis   </li></ul><ul><li>Mood disorder   </li></ul><...
, DIAGNOSIS OF CEREBRAL LUPUS ARA Criteria Neuropsychiatric sign Laboratory test, neuropsyc h ology test,   imaging = non ...
LUPUS   <ul><li>SEVERE FLARE </li></ul><ul><li>TREATMENT FAILURE </li></ul><ul><li>AMNIONIC FLUID INDEX  <5 CM </li></ul><...
, Cerebral lupus Lupus with pregnancy <ul><li>Met h ylprednisolon </li></ul><ul><li>Dexametason </li></ul><ul><li>Cyclopho...
, <ul><li>Time & onset </li></ul><ul><li>Disease   manifestation </li></ul><ul><li>Disease activity </li></ul><ul><li>Trea...
Summary , <ul><li>A 18 year old female patient with cerebral lupus and pregnancy </li></ul><ul><li>The diagnosis of cerebr...
Thank you Monument of Sidoarjo city “ alun – alun”
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A Pregnant Patient With Cerebral Lupus

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A Pregnant Patient With Cerebral Lupus

  1. 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
  2. 2. INTRODUCTION <ul><li>Chronic Autoimmune Rheumatic Disease </li></ul>, Multiorgan disease Autoantibod ies CEREBRAL LUPUS SLE
  3. 3. <ul><li>All ages , >>16 – 55 years old </li></ul><ul><li>♀ :♂ = 8 :1 – 13 : 1 </li></ul><ul><li>4 - 250 cases / 100.000 population </li></ul><ul><li>Woman with SLE , the fertility is not affected </li></ul><ul><li>SLE & Pregnancy : ↑ incidence of : flare, p rematurity, i ntrauterine </li></ul><ul><li>f etal g rowth r etardation (IUGR), p erinatal & maternal d eath </li></ul><ul><li>70% in SLE patient, mortality 7 – 13 % </li></ul><ul><li>Severe to mild  myelitis / CVA to subclinic </li></ul><ul><li>neurocognitive d y sfunct i on </li></ul>SLE Cerebral Lupus SLE & Pregnancy Epidemiology
  4. 4. <ul><li>Survival rates </li></ul><ul><ul><li>90 – 95% in 2 years, </li></ul></ul><ul><ul><li>82 – 90 % in 5 years, </li></ul></ul><ul><ul><li>> 81 % in 10 years. </li></ul></ul><ul><li>Immunosup pressive agent & corticosteroid </li></ul>Prognosis
  5. 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. 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
  7. 7. , <ul><li>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 </li></ul><ul><li>Head and neck : </li></ul><ul><li>Anemia (-); icterus (-); dyspnea (-); cyanosis (-); malar rash / butterfly appearance (+); Oral ulcer (+) </li></ul><ul><li>Thorax : no abnormality </li></ul><ul><li>Abdominal : gravid (+) </li></ul><ul><li>Extremities : edema -/- </li></ul>Physical Examination Jan 21 th ,2009
  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 21 th ,2009
  9. 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. 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
  11. 11.
  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 ) + 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. 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. 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. 15. Discussion
  16. 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
  17. 17. COMPLEMENT ACTIVATION C3, C4 Case Case IMMUNE COMPLEX <ul><li>Febris </li></ul><ul><li>Leukocytosis </li></ul><ul><li>↑ ESR </li></ul><ul><li>↑ CRP </li></ul><ul><li>Blood culture (+) </li></ul>Sepsis INFECTION
  18. 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. 19. , Bleeding D y sfunctional Plexus coroides Anti neural Antibody Mechanism of cerebral lupus Neuroendocrine Immune System CNS Injury Vascular Oclusion CEREBRAL LUPUS
  20. 20. , <ul><li>Acute confusional state, c ognitive d ysfuntion, Psyc h osis </li></ul><ul><li>Mood disorder </li></ul><ul><li>Anxiety </li></ul><ul><li>Movement disorder </li></ul><ul><li>Seizure </li></ul><ul><li>Headac h e (migrain intracranial hypertention ) </li></ul><ul><li>CVA </li></ul><ul><li>Transversal myelitis </li></ul><ul><li>Dimyelinating syndrome </li></ul><ul><li>Aseptic meningitis </li></ul><ul><li>Cranial nerves neuropathy </li></ul><ul><li>Polyneuropathy </li></ul><ul><li>Plexopathy </li></ul><ul><li>Focal neuropathy </li></ul><ul><li>GBS </li></ul><ul><li>Otonomic dysfuntion </li></ul><ul><li>Myasthenia gravis </li></ul>CNS PNS Clinical Manisfestation Neuropsychiatric syndrome <ul><li>Acute confusional state, </li></ul><ul><li>c ognitive d ysfun c tion, </li></ul><ul><li>Psyc h osis (+) </li></ul><ul><li>Mood disorder (+) </li></ul><ul><li>Anxiety (+) </li></ul><ul><li>Movement disorder (+) </li></ul><ul><li>Seizure (+) </li></ul>CASE
  21. 21. , DIAGNOSIS OF CEREBRAL LUPUS ARA Criteria Neuropsychiatric sign Laboratory test, neuropsyc h ology test, imaging = non SLE
  22. 22. LUPUS <ul><li>SEVERE FLARE </li></ul><ul><li>TREATMENT FAILURE </li></ul><ul><li>AMNIONIC FLUID INDEX <5 CM </li></ul><ul><li>PROGRESIVE ECLAM P SIA </li></ul><ul><li>HELLP SYNDROME </li></ul><ul><li>SEVERE DECOM P CORDIS </li></ul><ul><li>SEVERE TROMBOCYTOPENIA </li></ul><ul><li>PSYC H OSOCIAL </li></ul><ul><li>FETAL DISTRESS </li></ul>TERMINATION ? <ul><li>Flare </li></ul><ul><li>Abortus </li></ul><ul><li>Prematurity </li></ul><ul><li>IUFD </li></ul>PREGNANCY Case <ul><li>SEVERE FLARE </li></ul><ul><li>TREATMENT FAILURE </li></ul><ul><li>AMNIONIC FLUID INDEX <5 CM </li></ul>
  23. 23. , Cerebral lupus Lupus with pregnancy <ul><li>Met h ylprednisolon </li></ul><ul><li>Dexametason </li></ul><ul><li>Cyclophosphamide </li></ul><ul><li>Prednison </li></ul><ul><li>Pre d nisolon </li></ul><ul><li>Methylprednisolon </li></ul><ul><li>Azat hioprine </li></ul><ul><li>Cyclosporin A </li></ul><ul><li>Low dose aspirin </li></ul><ul><li>Pulse dose methylprednisolon 500mg iv  tapp off 1 mg / kg bb / day </li></ul><ul><li>Cyclosphosphamide 500 mg ( informed con s ent ) </li></ul><ul><li>The family refused for termination the pregnancy </li></ul>Case TREATMENT
  24. 24. , <ul><li>Time & onset </li></ul><ul><li>Disease manifestation </li></ul><ul><li>Disease activity </li></ul><ul><li>Treatment </li></ul>SLE Factor s Non SLE factor s PROGNOSIS OF SLE Case <ul><li>The cerebral lupus was getting better with the </li></ul><ul><li>treatment (clinical sign improvement) </li></ul><ul><li>Dubious ad bonam for the mother, but dubious ad malam for </li></ul><ul><li>the baby </li></ul><ul><li>Early diagnosis </li></ul><ul><li>Respon e to treatment </li></ul><ul><li>Race </li></ul><ul><li>Sex, age </li></ul><ul><li>Social economy </li></ul>
  25. 25. Summary , <ul><li>A 18 year old female patient with cerebral lupus and pregnancy </li></ul><ul><li>The diagnosis of cerebral lupus was based on anamnesis, physical examination and laboratory examination a ccording to ARA clasification criteria which is supported by the neuropsychiatr ic syndrome of SLE. </li></ul><ul><li>The Patient w as planned to be terminated the pregnancy but the family refused it. </li></ul><ul><li>The patient received medical treatment with corticosteroid (methylprednisolon) and immunosup p ress ive agent (cyclophosphamide) with a good respone , shown as a clinical sign improvement of cerebral lupus </li></ul>
  26. 26. Thank you Monument of Sidoarjo city “ alun – alun”

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