a pregnant patient with cerebral lupus
DESCRIPTION
A Pregnant Patient With Cerebral LupusTRANSCRIPT
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A PREGNANT PATIENT WITH CEREBRAL LUPUS
Puguh Widagdo
Joewono Soeroso
CASE PRESENTATION
Department of Internal MedicineDr Soetomo Teaching Hospital- Airlangga University School of Medicine
SURABAYA
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INTRODUCTION
Chronic Autoimmune Rheumatic Disease
Multiorgan disease
Autoantibodies
CEREBRAL LUPUS
SLE
All ages , >>16 – 55 years old ♀:♂ = 8 :1 – 13 : 1 4 - 250 cases / 100.000 population
Woman with SLE, the fertility is not affectedSLE & 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
Survival rates 90 – 95% in 2 years, 82 – 90 % in 5 years, > 81 % in 10 years.
Immunosuppressive agent & corticosteroid
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Mrs N, 18 y.omarried, Javanese, moslem, stay at Surabaya
Admitted to dr.Soetomo hospital on Jan 21th 2009
Chief complaint :FEVER
Mrs N, 18 y.omarried, Javanese, moslem, stay at Surabaya
Admitted to dr.Soetomo hospital on Jan 21th 2009
Chief complaint :FEVER
PATIENT IDENTITYCASE
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History of Present IlnessHigh 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 found1st pregnancy : normal delivery
No family with the same disease
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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
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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
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Radiologic Imaging
Conclusion : No abnormality
Jan 21th ,2009
Chest x-ray Conclusion : No abnormalityCt – Scan Skull
Conclusion : No abnormality
Feb 5th ,2009
Obstetry USGConclusion : BP 38, FL 21, amnion fluid ( - )
Feb 10th ,2009
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Initial Assesment
Cerebral lupus + S.Sepsis (unknown source of infection ) +
G2P1-1 + Hypokalemia
Planning :Planning :
Urinalysis and urine sediment, ANA test, CRP, C3, C4Blood and urine culture
DX:DX:
TX:TX: O2 4 lpmBed restEnteral diet HCHP 6x150 ccRL : D5 infusion :2:2Ceftriaxone injection 2x1gParacetamol 3x 500mgHypokelemia correction with KCL 50mg in RL 500 cc / 24 hour
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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: BedrestEnteral diet 6x150 ccRl : D5 infusion 2:2Ceftriaxone injection 2x1 gPulse 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 hypokalemiaAdv : EEG if transportable
Psychiatric : mental organic disorder due to SLEAdv : treat organic cause and Haloperidol 2 x 0,5 mg or Haloperidol injection (1/2 ampul i.v) if agitated
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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 CeftazidimeTx :~ 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)
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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-0-0Chloroquinolon 1 x 100 mgCa2 vit D31x1
Feb 15th ,2009 (day 25)
Mar 2th ,2009 (day 35)
Feb 25th ,2009 (day 30)
TISSUE NECROSIS
Autoreactive T cell TH cell
B cell
Auto AgAuto Ab
ComplementActivation
PlateletAgregation
Hagemen factor activation
VASCULITIS
TROMBUS
NEUROENDOCRINE(SEX HORMONE)
GENETIC
PATOGENESIS
ENVIRONMENT(UV, INFECTION)
IMMUNE COMPLEX
Febris Leukocytosis ↑ ESR ↑ CRPBlood culture (+)
Febris Leukocytosis ↑ ESR ↑ CRPBlood culture (+)
COMPLEMENT ACTIVATION
C3, C4
SepsisSepsis
INFECTION
IMMUNE COMPLEX
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
(+)
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BleedingDysfunctional
Plexus coroides
Anti neural Antibody
Mechanism of cerebral lupusMechanism of cerebral lupus
NeuroendocrineImmune System
CNS Injury
Vascular Oclusion
CEREBRAL LUPUS
,Acute confusional state, cognitive dysfuntion, Psychosis Mood disorder Anxiety Movement disorder Seizure Headache (migrain intracranial hypertention )CVATransversal myelitisDimyelinating syndromeAseptic meningitisCranial nerves neuropathy
Polyneuropathy
Plexopathy
Focal neuropathy
GBS
Otonomic dysfuntion
Myasthenia gravis
CNS PNS
Clinical Manisfestation
Neuropsychiatric syndrome
Acute confusional state, cognitive dysfunction, Psychosis (+)
Mood disorder (+)Anxiety (+)Movement disorder (+)Seizure (+)
CASE
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DIAGNOSIS OF CEREBRAL LUPUS
ARA Criteria Neuropsychiatric sign
Laboratory test, neuropsychology test, imaging = non SLE
LUPUS
SEVERE FLARETREATMENT FAILUREAMNIONIC FLUID INDEX <5 CM
PROGRESIVE ECLAMPSIAHELLP SYNDROMESEVERE DECOMP CORDISSEVERE TROMBOCYTOPENIAPSYCHOSOCIALFETAL DISTRESS
Flare Abortus Prematurity IUFD
Flare Abortus Prematurity IUFD
PREGNANCY
SEVERE FLARETREATMENT FAILUREAMNIONIC FLUID INDEX <5 CM
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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
TREATMENT
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Time & onset
Disease manifestation
Disease activity
Treatment
Early diagnosis
Respone to treatment
Race
Sex, age
Social economy
SLE Factors Non SLE factors
PROGNOSIS OF SLE
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
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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
Thank you
Monument of Sidoarjo city
“alun – alun”