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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”