CBD & BOOK

READING
Dipresentasikan oleh: Migi Pradysta
Pembimbing : Prof. Dr. dr. Nyoman Kertia, SpPD-KR
 Nn. Dian Ernawati, 28 tahun

No CM : 01802579
Alamat : Yogyakarta
Pendidikan : S1
Pekerjaan : Mahasiswa
Suku : Jawa
Agama : Islam
Dirawat di : Dahlia 1
Indikasi rawat inap: Terapi
Keluhan utama :
Gaduh gelisah sejak 2 hari sebelum masuk rumah sakit
RPS :
2 hari sebelum masuk rumah sakit Os gaduh gelisah, mengigau dan melantur.
Os mulai menyakiti diri sendiri dengan mencubit dan mencakar lengan hingga
kemerahan. Os sulit tidur 2 malam terakhir. Os tidak selera makan dan minum.
Demam (-) batuk (-) sesak nafas (-) mual (-) muntah (-). BAB dan BAK normal.
Hari masuk rumah sakit keluhan menetap, gaduh gelisah (+), bicara tidak
nyambung (+) kejang (-), pingsan (-), demam (-), makan dan minum sulit, Os
dibawa ke UGD RSS.
Os adalah penderita SLE tegak sejak 3 minggu yang lalu, saat dirawat di
Dahlia 4 dengan kriteria ARA yang positif demam (+), artritis (+), vaskulitis (+),
kelainan hematologi (+), ANA (+) dan ds DNA (+). Os pulang mondok 1 minggu
yang lalu dengan terapi cellcept 3x500 mg, cefixim 2x200 mg, MP 16 mg 2-2-0,
curcuma 3x1, lansoprazole 30 mg 1x1 tab, chloroquin 1x150 mg, betahistin 8
mg 3x1, sistenol 3x1 tablet, inpepsa 3x1.
RPD :
Riwayat alergi (-)
Riwayat gangguan jiwa (-)
Riwayat kejang, epilepsi (-)

RPK :
Herediter (-)
Familial (-)
Infeksi (-)

Riwayat Pribadi :
Os adalah seorang mahasiswi. Os belum menikah. OS kesulitan
ekonomi (-) , biaya ditanggung JKN non PBI
Anamnesis Sistem
Keadaan umum
Tampak gelisah
Kulit
Tidak ada perubahan warna kulit, gatal (-), ruam (-), kelainan kuku (-), infeksi
kulit (-)
Kepala
Sefalgia (-), vertigo (-), nyeri (-), sinus (-), trauma kapitis (-)
Mata
Tidak ada riwayat mata berwarna kuning
Telinga
Tidak ada keluhan pendengaran, tinnitus (-), secret tidak ada kelainan, nyeri (-)
Hidung
Pilek (-), obstruksi (-), epistaksis (-), bersin (-)
Mulut dan tenggorokan
tidak ada keluhan sukar atau nyeri menelan atau mengunyah, tonsilitis (-),
stomatitis (-), saliva tidak ada kelainan, suara serak (-)
Anamnesis Sistem (lanjutan)
Leher
pembesaran gondok (-), pembengkakan kelenjar getah bening (-)
Dada
Tidak pernah mengalami gejala asma, Batuk (-),dahak (-),sesak nafas (-),
hemoptisis (-)
Jantung
Tidak ada keluhan sesak, berdebar-debar, ataupun nyeri dada kiri, ortopnu (-),
hipertensi (-)
Vaskuler
Tidak ada keluhan ataupun riwayat penyakit vaskuler
Gastrointestinal
Os tidak selera makan dan minum,tidak mual, tidak muntah.
Genitourinaria
benjolan (-), nokturia (-), disuria (-), polakisuria (-), poliuria (-), retensi urin (-),
anuria (-), hematuria (-)
Anamnesis Sistem (lanjutan)
Muskuloskeletal
Nyeri sendi (+), nyeri otot (-), kejang otot (-), kelemahan otot (-), nyeri tulang (-),
riwayat gout (-)
Payudara
perdarahan (-), discharge (-), benjolan (-)
Neurologik
Gaduh gelisah (+), bicara tidak nyambung(+), gangguan saraf otak (-), paralysis (-),
anastesi (-), parestesi (-), ataksia (-), gangguan fungsi luhur(-)
Endokrin
Tidak ada keluhan pembesaran kelenjar gondok, gangguan pengaturan suhu,
maupun perubahan rambut, tremor (-), diabetes (-), akromegali (-)
Psikiatrik
Sulit tidur (+)
Pemeriksaan Fisik
KU : Sedang, kesadaran delirium, gizi kurang
TB 38 cm, BB 148 kg, IMT 17,35 kg/m
VS : TD 110/70 mmHg, tidur, manset di lengan kanan, large
adult cuff
N 100 x/menit, irama teratur, isi dan tekanan cukup
R 20 x/menit, irama teratur, tipe pernapasan
thorakoabdominal
T 36,8 C, suhu aksila
Kepala : Insp. : konj. pucat (-), sklera ikterik (-), malar rash
(+)
Palp. : tidak ada nyeri tekan, tak teraba massa
Leher : Insp. : JVP tak meningkat
Palp. : lnn ttb
Thorax :
Pulmo : Insp. : simetris, KG (-),
retraksi (-)
Palp. : stem fremitus
kanan = kiri
Perk. : sonor (+)
Ausk. : vesikuler (+) RBK
Pemeriksaan Fisik
Abdomen : Insp. : datar
Ausk. : peristaltik (+) N
Perk. : timpani di seluruh regio
Palp. : NT (-), H/L ttb
Extremitas : Insp. : edema

vasculitis + +
+ +

Palp. : akral hangat, tidak ada nyeri tekan

Pemeriksaan Penunjang
Darah rutin Ginjal Hati
Hb 10,2 g/dl BUN 22 mg/dl GOT 28 U/L
AL 14,08 .103/L Crea 0,94 mg/dl GPT 68 U/L
AT 272 .103/L mg/dl Alb 4,35 g/dL
AE 3,73 .106/L
Hmt 30,6 %
Elektrolit Urin Rutin
S 93,3 % Na 139 warna yellow
K 3,18 mmol/L pH 6,5
L 3,3 %
Cl 105 mmol/L BJ >1,030
M 2,7 %
Mg 2,8 mmol/L Blood 1+
E 0,1 %
Ca 2,18 mg/dL LE (-)
B 0,5 %
mmol/L nitrit (-)
MCV 82,2 fL eritro 20
Glukosa Leu 19
MCH 27,4 pg GDS 99
mg/dl Epitel 20
Leu 19
Protein spesifik epitel 35,2
CRP kuantitatif 12 mg/L silinder 1,2
Procalsitonin 0,25 bacteri 420
Kristal 0,4
EKG 12/3/17 : sinus rhytm, heart rate 94 x/menit , normo axis
Ro thorax 3/7 :
pulmo tak tampak kelainan
Cor CTR 0,6 (inspirasi kurang)
SLEDAI SCORE :
Gangguan neurologis 8
Gangguan ginjal 0
Vasculitis 8
Hemostatis dan trombositopenia 0
Miositis 0
Arthritis 4
Malar Rash 2
Serositis 0
Demam 0
Leukopenia 0

TOTAL 22
Severe flare (SLEDAI sebelumya 14)
ASSESMENT
Systemic Lupus Eritematosus Severe Flare
dengan manifestasi suspect Neuropsikiatrik
Lupus
Hipokalemia ringan
Terapi :
IVFD NaCl 0,9% 20 tpm
Inj. MP 750 mg /24 jam (3 hari)
Cellcept 3x500 mg
Chloroquin 1x150mg
Inj. Haloperidol 5 mg/12 jam
Inj. Diazepam 10 mg/12 jam
Sistenol 3x1 tab kp
Aspar K 3x1
Konsul senior reumato:
Injeksi MP 750 mg/ 24 jam (3 hari)
Terapi yang lain diteruskan
Konsul psikiatri:
A : Gangguan mental organik DD
delirium
P : Haloperidol tab 1,5 mg/12 jam atau
inj.2,5 mg/12 jam. Raber psikiatri
Plan :
MSCT kepala
Konsul neurologi:
A : - Suspect neurotik, stress related dan
somatoform dissorders DD
neuropsikiatrik lupus
- Tidak ditemukan defisit
neurologis fokal
P : - Head CT scan (untuk menyingkirkan
lesi struktural)
- Haloperidol 2x1 mg
- Raber neuroinfeksi
NEUROPSYCHIATRIC LUPUS
 BACKGROUND

Neuropsychiatric systemic lupus erythematosus (NPSLE) is a generic

definition referring to a series of neurological and psychiatric
symptoms directly related to systemic lupus erythematosus (SLE)
No laboratory or radiological biomarker nor other formal system
exists for establishing a diagnosis and guiding therapy decisions in
neuropsychiatric SLE (NPSLE)
 EPIDEMIOLOGI

It is well-established that SLE is substantially increased in females of

child-bearing age (female : male ratio is 815:1)
There is limited evidence to support an association between gender
and NPSLE
NP manifestations are more frequently seen in African descendants,
Hispanics, and Asians than in White individuals
NP manifestations usually occur early in the course of SLE, and in 39
50 % of patients it is the presenting symptom of SLE
KLASIFIKASI
 PATOGENESIS
1. Autoimmune or Inflammation

Characterized by brain dysfunction due to autoantibodies or

inflammatory mediators with either a disrupted blood
brain barrier (BBB) or intrathecal formation of immune
complexes and the presence of inflammatory mediators
2. Vascular injury and occlusion

Characterized by a thrombotic process of the large and

small (microangiopathy) intracranial vessels due to
autoantibody- mediated vascular injury, immune complexes,
complement deposition, leukoagglutination, and accelerated
atherosclerosis.
 DIAGNOSIS
Given the absence of a gold standard in the diagnostic approach, NPSLE remains a
diagnosis per exclusionem and is mainly based on expert opinion
In all patients, there is an obligation to first exclude other causes such as infection,
coincidental disease processes, metabolic abnormalities, or drug side effects
Among all circulating autoantibodies, aPL, including aCL, LAC, and b2-
glycoprotein antibodies, provide the greatest diagnostic information in NPSLE,
especially in patients with focal NP events such as cerebrovascular disease and
seizures
It has been suggested serum antiribosomal P antibodies are specifically related to
lupus psychosis
Autoantibodies to aquaporin 4 can help in the diagnostic process of a patient
presenting with myelopathy and optic neuritis
The measurement of autoantibodies or cytokines in CSF is not recommended
in clinical practice at this time due to the lack of specificity
 Magnetic resonance imaging (MRI) is the neuroimaging technique of
choice in NPSLE to localize abnormalities in the brain and spine, allowing
the identification of lesions associated with NPSLE (e.g. infarcts or
myelopathy)
However, MRI is nonspecific and non sensitive enough to rule out
abnormality
THERAPY
PRIMARY PREVENTION
DRUGS
1. Antimalarial drugs
. several studies using antimalarials have demonstrated that immunologic
effects can reduce the risk of CVD, such as improvement of
dyslipidemias, prevention of diabetes, and reducing aPL titers

. antimalarials had an independent protective effect on the development of

metabolic syndrome over time, showing its potential as an
atheroprotective drug

. antithrombotic effect is another known additional benefit of

antimalarials

. Another potential benefit attributed to antimalarial drugs in SLE is the

protective effect against seizures
2. Statins
. Some immunomodulating effects, such as reduction of inflammatory
cytokines and adhesion molecules, prevention of endothelial cell
activation induced by aPL, inhibition of T-cell function, and reduction of the
number and activity of inflammatory cells in atherosclerotic plaque
have been attributed to statins

. For some authors, SLE is considered a coronary heart disease risk factor
equivalent to diabetes The Lupus Atherosclerosis Prevention Study, a 2-
year trial of atorvastatin in 200 adult patients with SLE without clinical CVD,
showed no benefit in the primary or secondary atherosclerosis outcomes

. The use of statins is recommended in these patients when the LDL cholesterol
level is >100 mg/dL
MANAJEMEN INFLAMMATORY
NPSLE
1. Corticosteroid
.Offers the most immediate anti-inflammatory effect of all
immunosuppressive therapies and is subsequently the more widely used
therapy to control mildsevere flares of SLE
.Glucocorticoids may aggravate underlying metabolic abnormalities and
other factors contributing to the risk of clinical accelerated atherosclerosis,
which may lead to early cerebrovascular disease.
each 2-month exposure to high-dose prednisone was associated with
a 1.2-fold increase in the risk of stroke in SLE
doses <7.5 mg/day, as well as methylprednisolone pulses, may not be
associated with damage accrual
2. Cyclophosphamide
.Several case series have described positive effects of treatment with
cyclophosphamide in severe NPSLE manifestations in both adults and
children
.Cyclophosphamide has significant side effects

3. Azathioprine
.Nowadays, azathioprine (23 mg/kg/day) is mainly used in SLE patients
presenting with arthritis, mucocutaneous manifestations, and
serositis, and as maintenance therapy in lupus nephritis
.Due to its relatively mild side effect profile, azathioprine is frequently used
for maintenance or as a glucocorticoid-sparing agent
4. Mycophenolate Mofetil
In SLE patients, mycophenolate mofetil is widely used as the first-line option for
both induction and maintenance therapy of lupus nephritis
Several observational studies have suggested the potential benefit of mycophenolate
mofetil in nonrenal manifestations of SLE, especially in hematological and
dermatological manifestations; how- ever, none focused on NP manifestations

5. Metotrexate
Methotrexate is routinely used in SLE patients with musculoskeletal and skin
manifestations
This drug is used very rarely in NPSLE and evidence is limited to several case
series reporting the effect of intrathecal methotrexate
5. Cyclosporine
The place of cyclosporin A in the treatment of SLE patients is limited and
most data regarding these agents come from experience with lupus
nephritis
No studies explicitly describe the effect of cyclosporin A on NP symptoms
in SLE

6. Rituximab
Currently, rituximab is widely used as an alternative therapy in patients
with active SLE who are nonresponsive to standard immunosuppressive
therapy
Current data support the use of rituximab as a second-line therapy in
patients with severe refractory NPSLE
7. Intravenous Immunoglobulin
IVIGs in SLE are severe cases nonrespondent to conventional
immunosuppressive drugs, patients with active SLE and concomitant
infection.
IVIGs may be used in severe refractory NPSLE not responding to
conventional immunosuppression, or even as the first- choice agent
when patients are pregnant or if symptoms are life-threatening and a
concomitant infection is pre- sent.
TERIMAKASIH