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SYSTEMIC LUPUS

ERYTHEMATOSUS

Eka Amanda Faradillah C014172091


Nurul Fatimah Ramli C014172190
• Name : Miss. BD
• Age : 24 years old
• Address : Maccini
• Occupation : Student
• Religion : Islam
• Marital Status : Single
• Hospital : RSWS
• MR : 680806

Patient Identity
Main complaint : Oral ulcer
Further anamnesis:
• Patient came with complaint of oral ulcer since 10 days ago. Frequency of vomitting 1-
2 times daily, containing food, no blood. Now, consumed Plaquenil 200 mg 1x1,
Methylprednisolon 8 mg 3x1, Lansoprazole 40 mg 2x1
• Fever (+) since 10 days ago, continously, decreased with paracetamol, but fever returns
few hours later.
• Patient feel itchy and redness in the face when exposed to sunlight
• Headache (+) and feels throbbing, cough with phlegm (seropurulent) (+) since 10 days
ago, shortness of breath (+)
• Pain throughout the joints, especially the spine since last 2 months
• Swelling finger joints and feel warm (PIP of digiti 2 of right hand)
• Irregular bowel movement, only 2 times on 15 days
• Urination was normal, foamy urine
• History of diabetes mellitus and hypertension (-)
• Family history with SLE (-)
• History of inpatient repeatedly and last in February 2018 with diagnose SLE

HISTORY TAKING
General Description
General condition: Moderate illness
Nutrition: normoweight
• Vital Signs
• Awareness : Conscious (GCS 15 E4M6V5)
• Blood pressure : 120/88 mmHg
• Heart rate : 68 x/ min, regular, strong
• Respiratory rate : 20 x/min, thoracoabdominal
• Temperature : 37,9°C (axilla)
• VAS : 5/10

PHYSICAL EXAMINATION
PHYSICAL EXAMINATION
• Head : Normocephal, straight black hair, and easy to fell out
• Face : Oedema
• Eyes : Pupils isocor, normal light reflex, conjungtiva pale
no subconjunctival bleeding, no icteric.
• Ear : No abnormalities, otorrhea (-)
• Nose : No abnormalities, epistaxis (-)
• Lips : Oral ulcer (+), cyanosis (-)
• Oral cavity : No abnormalities, gingival hypertrophy (-)
• Throat : No abnormalities, pharyngeal hyperemia (-), T1-T1 quiet.
• Neck : No lymphadenopathy, no enlargement of
thyroid gland, no deviation of the trachea.
PHYSICAL EXAMINATION
LUNG
• I : symmetry left and right
• P : normal vocal fremitus, tenderness (-)
• P : sonor
• A : vesicular breath sound
HEART
• I : Ictus cordis not visible
• P : thrill nor palpable
• P : normal heart borders
• A : normal SI/II heart sound, additional sound (-)
ABDOMEN
• I : distention (-)
• A : normal
• P : hepatosplenomegaly (-), right hipocondrium pain (+), CV tenderness (+)
• P : tympani, ascites (-)
PATIENT PROFILE
• Gait : Normal

• Arm : Oedema on PIP of digiti II of right hand

Fingers joint pain

Left and right shoulder pain


• Leg : Right and left knee : Tumor (-), dolor (+), kolor (-), rubor (-), functio
laesa (-), bony enlargement (-)
ROM Normal
• Spine : Tenderness in CV L5-Sacrum

RHEUMATOLOGICAL STATUS
LABORATORY FINDING
May 13rd, 2018
Leukosit 4.88 x 103 / uL 4000-10.000/µL
Eritrosit 3.08 x 106 / uL 4-6 x 106 / µL

Hemoglobin 8.7 g/dL 12.0 – 16.0 g/dL


Hematokrit 26.8 % 37-48%
Trombosit 103.000 / µL 150.000-400.000 / µL
MCV 87 fL 80-97 fL
MCH 28.2 pg 26.5-33.5 pg
MCHC 32.5 g/dl 31.5-35 g/dl
Neutrofil 81.9 % 52-75%
Limfosit 10.5 % 20-40%
Monosit 7.6 % 2-8%
Eosinofil 0% 1-3 %
Basofil 0% 0-0.1 %
May 8rd, 2018
Coomb test Positive/1 Negative

LABORATORY FINDING
Blood Chemistry May 3rd, 2018
SGOT 157 U/L <38U/L
SGPT 55 U/L <41U/L
Albumin 2.8 gr/dL 3.5-5.0 gr/dL
Ureum 81 mg/dL 10-50 mg/dL
Kreatinin 1.51 mg/dL <1.1 mg/dL
Uric Acid 14.4mg/dL 2.4-5.7 mg/dL
Electrolyte May 3th 2018
Sodium 136 mmol/L 136-145 mmol/L
Potassium 4.4 mmol/L 3.5-5.1mmol/L
Chloride 105 mmol/L 97-111 mmol/L

LABORATORY FINDING
Urinalysis May 4rd, 2018
Color Clear Yellow Yellow
Blood 3+ Negatif
Billirubin Negatif Negatif
Urobilinogen Normal Normal
Keton Negatif Negatif
Protein 2+ Negatif
Nitrit Negatif Negatif
Glucose Negatif Negatif
pH 6 4.5-8.0
SG (Mass) 1.015 1.005-1.035
Leukosit Negatif Negatif
Vitamin C 0 Negatif

LABORATORY FINDING
LABORATORY FINDING
Urinalysis May 7th , 2018
Protein Esbach Negatif Negatif
EKG May 3rd, 2018
Chest Xray May 3rd, 2018

• Platelike athelectasis dextra


PROBLEM LIST
Planning Treatment

1. Systemic lupus erythematous severe • CBC test • Methyprednisolone


(ACR 5/11) control 8mg/8 hours/oral
Based on: • ESR test • Cavit D3 /8
Oral Ulcer control hours/oral
Photosensitivity • C3 and C4 • Allopurinol 100
Malar Rash • ANA test mg/24 hours/oral
Arthritis • Anti-dsDNA • Nistatin drops/12
Hematologic disorders ( hemolytic test hours/oral
anemia, thrombocytopenia, • RF & anti- • Lansoprazole 30
lymphopenia) CCP mg/24 hours/oral
• Reticulocyte,
peripheral
blood smear
PROBLEM LIST
Planning Treatment

2. Hypoalbuminemia • Blood
chemistry test
Based on; control
Albumin 2.8 gr/dL

3. Hyperuricemia asimptomatic • Blood • Allopurinol


chemistry test 100mg/24h/oral
Based on: control
Uric acid 14.4 mg/dL and clinical
manifestation (-)
DISCUSSION
“Systemic Lupus
Erythematosus”
DEFINITION

Systemic Lupus Erythematosus (SLE) is a heterogeneous


autoimmune disease characterized by the production of
antibodies to components of the cell nucleus in association
with a diverse array of clinical manifestations and
involvement of many different organs.

Kasper D et al, 2015. (Harrison’s Principle of Internal Medecine, 19th ed.)


EPIDEMIOLOGY

• Ninety percent of patient are women of child-bearing years.


People of all genders, ages and ethnic groups are susceptible
• Prevalence of SLE in the United States is 20 to 150 per
100.000 women depending on race and gender; highest
prevalence is in African-American and Afro-Caribbean
women, and lowest prevalence is in the white men

Kasper D et al, 2015. (Harrison’s Principle of Internal Medecine, 19th ed.)


ETIOPATHOGENESIS
Genetic, gender, hormon,
and environmental factor

Immunological
dysregulation

Cleareance
dysfunction
Defective antigen
Dendritic cell presentation

B-Cell T-Cell

Antinuclear Antibody

- Abnormal complement
- Increased apoptosis
dysfunction Tissue
- Defective cytokine
- Defective phagocyte Damage production
activation
Anthony S Fauci et al, 2013. (Harrison’s Rheumatology, 3 th ed.)
Suspicion of SLE need to be considered if there is 2
(two) or more criteria as listed;

1. Young woman with 2 organs included or more


2. Constitusional symptoms : fatigue, fever (with no infection
evidence), and weight loss
3. Musculosceletal : arthitis, arthralgia, myositis
4. Skin : butterfly rash/malar rash, photosensitivity,
mucousmembrane lesion, allopesia, Raynaund phenomen,
pupura, utrikaria, vaskulitis
5. Renal : hematuria, proteinuria, silinderuria, nefrotik syndrome

Vu Lam NC, V G Maria, L Marzela. SLE:Primary Care Approach to Diagnosis and Management. American Family Physician. 2018 : Vol 94
6. Gastrointestinal : nausea, vomit and abdominal pain
7. Lungs: pleurisy, pulmonal hypertension, parenkim
lesion
8. Cor : pericarditis, endocarditis, myocarditis
9. Reticulo-endotel : organomegaly (lymphadenopathy,
splenomegaly, hepatomegaly)
10. Hematology : anemia, leukopenia and
thrombocytopenia
11. Neuropsychiatry : psychosis, seizure, organic brain
syndrome, myelitis transversus, cognitive impairment,
cranial and peripheral neuropathy

Vu Lam NC, V G Maria, L Marzela. SLE:Primary Care Approach to Diagnosis and Management. American Family Physician. 2018 : Vol 94
ACR 1997 Criteria for
Systemic Lupus
Erythematosus

Anthony S Fauci et al, 2013. (Harrison’s


Rheumatology, 3th ed.)
EVALUATION OF SLE PROGRESSION BY MEX-SLEDAI
2

< 2 : mild ; 2-5 : moderate ; >5 : severe


Anthony S Fauci et al, 2013. (Harrison’s
Rheumatology, 3th ed.)
Mild Moderate Severe
• Skin • Mild – moderate Nefritis • Severe Nefritis (Class IV,
• Trombositopenia (trombosit III+V, IV+V or III-V with
Manifestation
20-50x10^3/mm^3
• Arthritis • Serositis mayor
impaired renal function
• Refractory Trombositopenia
(<20 x 10^3 / mm^3)
• Refractory Hemolitic Anemia
Therapy
Induction Therapy • Associated with lung
Klorokuin or MTX
MP iv (0,5-1gr/day for 3 days (haemorrhagic)
And/Or following by: • Vaskulitis abdomen
KS (low dose) AZA (2mg/Kg/day) or MMF (2-
OAINS 3gr/day)
+
KS (0,5-0,6 mg/Kg/day for 4-6
weeks then lowered slowly Induction Therapy
TR MP iv (0,5-1gr/day for 3 days)
CYC iv (0,5-0,75 gr/m^2/month
x 7 dose)
MaintenanceTherapy RP
AZA (1-2mg/Kg/day) or RS TR
MMF (1-2 gr/day)
+ Maintenance Therapy Needed Rituximab
KS (lowered until 0,125 CYC iv (0,5-0,75 Calcineurin
mg/Kg/2 days dose) gr/m^2/3 months for one Inhibitor
year) IVIg
Rekomendasi Perhimpunan Reumatologi Indonesia Untuk Diagnosis dan Pengelolaan Lupus Eritematosus Sistemik
General practitioner
PRIMARY HEALTHCARE CENTRE SUSPECTED SLE

Reconciliation

MILD SLE RHEUMATOLOGIST / INTERNIST


• Diagnose
• Activity review and disease stage
SLE with complication / • Medication planning
increase activity • Monitoring disease’s activity regularly

Moderate and Severe SLE


Refractere SLE/life threatening

Rekomendasi Perhimpunan Reumatologi Indonesia Untuk Diagnosis dan Pengelolaan Lupus Eritematosus Sistemik
PROGNOSIS

• Over the past decade, the five-year survival rate of


patients with SLE has improved to more than 95%
because of more effective recognition and treatment
of infectious and renal complication

Vu Lam NC, V G Maria, L Marzela. SLE:Primary Care Approach to Diagnosis and Management. American Family Physician.
Criteria De nition

Malar rash Fixed erythema, at or raised, over the malar eminences, tending to spare the nasolabial folds

Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring occurs in
Discoid rash
older lesions

Photosensitivity Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation

Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by a physician

Arthritis Non-erosive arthritis involving two or more peripheral joints, characterised by tenderness, swelling or effusion

a. Pleuritis: convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion or
Serositis
b. Pericarditis: documented by ECG or rub or evidence of pericardial e usion

a. Persistent proteinuria >0.5 g per day or >3+ if quantitation is not performed or


Renal disorder
b. Cellular casts: may be red cell, haemoglobin, granular tubular, or mixed

a. Seizures:intheabsenceofo endingdrugsorknownmetabolicderangements(eg,uraemia, acidosis, or electrolyte


imbalance) or
Neurological disorder
b. Psychosis:intheabsenceofo endingdrugsorknownmetabolicderangements (eg, uraemia, acidosis, or
electrolyte imbalance)

a. Haemolyticanaemiawithreticulocytosis,or
2.Leucopenia: <4000/mm3, or
Haematologic disorder
3.Lymphopenia: <1500/mm3, or
d. rombocytopenia: <100 000/mm3 in the absence of o ending drugs

a. Anti-DNA:antibodytonativeDNAinabnormaltitre,or
b. Anti-Sm:presenceofantibodytoSmnuclearantigen,or
c. Positive ndingofantiphospholipidantibodiesbasedon:(1)anabnormalserum
Immunologic disorder
concentration of IgG or IgM anticardiolipin antibodies, (2) a positive test result for lupus anticoagulant using a
standard method, or (3) a false positive serologic test for syphilis known to be positive for at least 6 months and
con rmed by Treponema pallidum immobilisation or uorescent treponemal antibody absorption test

An abnormal titre of antinuclear antibody by immuno uorescence or an equivalent assay at any point in time
Antinuclear antibody
and in the absence of drugs known to be associated with ‘drug-induced lupus’ syndrome
THANK YOU

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