SYSTEMIC LUPUS

ERYTHEMATOSUS
Anisa Zulfa F.
Rachma Malina
Tiara Dewi Salindri Pratama
 Systemic Lupus Erythematosus
2

▸ SLE is a fluctuating multisystem disease with a diversity of clinical

presentations. Abnormal immunologic function and formation of
antibodies against “self-antigens” underlie the pathogenesis of SLE.
▸ SLE (or lupus for short) is a multisystem, autoimmune disease, involving
complex pathogenetic mechanisms that can present at any age. It most
commonly presents in women in the reproductive age group
▸ The hallmark of SLE is the development of autoantibodies to cellular
nuclear components, resulting in chronic inflammatory autoimmune
disease. Symptoms and organ involvement depend on the nature of the
autoantibodies.
3
ETIOLOGI

▸ Genetic (several human leukocyte antigen genes, non major

histocompatibility complex genes: immunoglobulin receptor genes and
mannose-binding protein genes)
▸ Environmental
UV light
Drugs (Isoniazid, procainamide, hydralazine)
Chemicals (hydrazine, aromatic amines)
Diet
Environmental estrogens
Infection with viruses or bacteria (the Epstein Barr virus)
▸ Hormonal factors
“
Pathophysiology
“
“
DIAGNOSIS
▸The diagnosis is based on the clinical symptoms and the
laboratory findings and based on Updating the American
College of Rheumatology Revised Criteria for the
Classification of Systemic Lupus Erythematosus

▸The proposed classification is based on 11 criteria. For the

purpose of identifying patients in clinical studies, a person
shall be said to have systemic lupus erythematosus if any 4
or more of the 11 criteria are present, serially or
simultaneously, during any interval of observation
“
Arthritis Rheum 1997;40:1725
“
“
“
“
 “
Epidemiology of Lupus

The incidence of SLE varies among ethnic groups with the annual incidence in adults ranging
from 1.9 to 5.6 per 100,000 persons per year. The disease occurs predominantly in women,
with a reported ratio approaching 10:1.
Epidemiology of Lupus in Indonesia

Anonim, 2017
 “
Treatment
A combination of treatment strategies called treatment pillars is needed.
The pillars is should be continuous so that the goal of treatment is
absolute.
The pillars:
I. Educations and conselling
II. Rehabilitation
III. Pharmacology treatments:
a. NSAIDs
b. Antimalarials
c. Corticosteroids
d. Immunosupressant / cytotoxic drugs
e. Another treatment

Anonim, 2011
 “
General approach to the management of systemic lupus
erythematosus

Dipiro, 2015
 “
SLE treatment strategies

Gordon carolin et al, 2018

 “
Medications are used in SLE therapy

Dipiro, 2015
“
Treatment of SLE Symptomps

Vulam et al, 2016

 “
SLE tyerapy in pregnancy

Things that must be considered for handling SLE before, during

pregnancy and postpartum:
1. If a person with SLE wants to become pregnant is recommended
at least after 6 months of controlled disease activity or in a state of
total remission. In lupus nephritis a period of longer than 12 months
of total remission. This can reduce lupus recurrence during
pregnancy.
2. Medicamentosa:
a) The dose of corticosteroids is as small as possible, not
exceeding 7.5 mg / day of prednisone or equivalent.
b) DMARDs or other medicines should be given with caution
Anonim, 2011
 “
Treatment during pregnancy and breastfeeding
Drugs Pregnancy Breastfeeding
NSAIDs Yes (avoid after 32 weeks) Yes
Antimalarias Yes Yes
Corticosteroids Yes (no more than 7,5 mg/day) Yes (maks 20 mg/day)
Cyclosporine Yes Unknown
Azathioprine Yes (no more than 1,5-2 mg/kg/day) Unknown

Mycophenolate No No
Methotrexate No, must be stop min 3 month before No
conception
Cyclophosphamide No No

Biologic agents No No

Warfarin No (considerate after fisrt trimester with Yes

caution)
Heparin Yes Yes

Low dose aspirin Yes Yes

Anonim, 2011
 “
Follow up and monitoring

Vulam et al, 2016

 “
Follow up and monitoring (Cont…)

Vulam et al, 2016

CASE
 “
A female 30 years old was hospitalized (June 12, 2018) due to fever. Fever has
been felt since one week ago and patient felt high fever in the night. Patient has
been used acetaminophen but the fever has not recovered.
Besides that, patient feels dizziness (+), eyes are hot and swollen, hair falls out for
five months ago, mouth feels sore when eating and drinking, bleeding in the gums
even though she was not brushing her teeth , cough (- mucus) since one week ago,
shortness of breath (+), heartburn, and diarrhea with watery stools since six days
ago.
Assessment With
SOAP Method
PATIENT IDENTITY

▸Name : Mrs. I
▸Age : 30 years old
▸Sex : Female
▸Job : Employees of an agency
▸MR number : 19 10 18
▸The Date of : June 12th, 2018
hospitalized
26
SUBJECTIVE
▸ Main complaint : Fever ▸ Other Medical History:
Peptic ulcer (+), asthma (-), BD (-)
▸ Others complaint: DM (-)
Dizziness (+)
Eyes are hot and swollen ▸ Family History: -
Alopecia
Mouth feels sore when eating and drinking ▸ Habit:
Bleeding gums even though she was not Consumption of herbs (-)
brushing her teeth drug consumption (6 months) (-)
Cough (- mucus)
shortness of breath (+) ▸ Social History:
Heartburn Exposure to cigarette smoke at work
Chest pain
Muscle and joint pain
Diarrhea with watery stools
27
OBJECTIVE

Physical examination

Status Present Vital Signs Others

Nutrition status: Enough BP : 130/80 mmHg Face: Malar rush (+)

Height : 157 cm HR : 128 x/minute Eye : Konjungtiva anemis (+/+)
Weight : 50 kg RR : 36 x/minute Mouth: stomatitis (+), bleeding
BMI : 20,31 Kg/m2 Temp : 40,1 0C gums (+)
GC: compos mentis Extremity: edema in both legs
28
CONT...
Laboratory tests
Parameter Normal Result Comment
WBC 4,00 – 10,0 2,48 [10^3/uL] Abnormal
RBC 4,00 – 6,00 3,58 [10^6/uL] Abnormal
HB 12,0 – 16,0 8,6 g/dL Abnormal
HCT 37,0 – 48,0 27,6 % Abnormal
MCV 80,0 - 97,0 77,1 [fl] Normal
MCH 26,5 - 33,5 24 [pg] Normal
PLT 150-400 95 [10^3/uL] Abnormal
Neutrofil 50-70 80,6% Abnormal
Limfosit 20-40 14,2% Normal
Eosinofil 1-3 0 Normal
Basofil 0-1 0,3% Normal
29
CONT...

Parameter Result Normal

Blood Glucose 102 70 – 180 mg/dl

Creatinine 0,7 0,5 – 1,0 mg/dl

Ureum 19 15-40 mg/dl

SGPT 114 5 – 35 U/L

SGOT 149 5-35 U/L

30
CONT....

Immunology test

Anti ds-DNA –Ncx

Titer : 700 IU/mL (positif) ▸ Diagnosis: Systemic Lupus

Erythematosus
Note:
Negative : < 100 IU/mL
Positive : ≥ 100 IU/mL
31
PRESCRIPTION MEDICATION

Drugs/ Strength and Frequency Administration Indication

IVFD Asering 20 tpm IV Acidosis concering dehydration and loss of

base from the body
PRC 400cc 200cc/day IV Anemia

Furosemide 40 mg/24 h IV Edema

Ciprofloxacin inf/12 h IV Suspect infection

Methylprednisolone 125 mg/8 h IV Lupus

Pantoprazole 40 mg/24 h IV heartburn

Acetaminophen 500mg q 8 h PO Fever, dizzy

Codein 10 mg q 8 h PO dry cough

32
ASSESSMENT AND PLAN

CLINICAL
S/O Drugs/ medication ASSESMENT DRP PLAN
PROBLEM

Anemia Hb 8,6 g/dl, PRC 200cc/day appropriate indication Monitoring blood profiles
plt 95 [10^3/uL]

Lupus Malar rash, Methylprednisolone Overdose of corticosteroids Recommendation of topical and oral
arthralgia, iv 125 mg/8 h In mild SLE , high dose of of corticosteroids and sunscreens.
Anti-dsDNA 700 corticosteroids is not Selected medicine:
IU recommended. For mild SLE • Methylprednisolone 8 mg PO /day
(without organ damage) use low •Betametason cr 0,1%
dose of corticosteroids. • Hydroksiklorokuin 200 mg PO/ day
Patient gets more than amount of •Sunscreens SPF 15
drugs she requires
Untreated medical condition.
In this case HCQ / chloroquine has
not been prescribed.
Malar rush: topical glucocorticoids
should be given and sun protection
factor min. SPF 15
33
ASSESSMENT AND PLAN
CLINICAL Drugs/
S/O ASSESMENT DRP PLAN
PROBLEM medication
Suspect infection Neutrofil 80,6% Ciprofloxacin inf Treatment without indication recommendation for stop antibiotic
there is no infection diagnosis.
Ciprofloxacine can induce SLE
Edema - Furosemide appropriate indication Continue furosemide 40 mg/ 24 h
Fever, dizziness Temeprature acetaminophen appropriate indication temperature checking, if symptoms
40,1oC are resolved, suggest to stop
medication
Heartburn - Pantorprazole appropriate indication Continue pantoprazole 40 mg/24 h
until the symptoms relief
Cough (-mucus) - Codein appropriate indication Continue codein 10mg/8 h untuk
the symptoms relief
Liver disfunction  ALT 149 dan - Untreated medical condition Recommendation for additional
SGPT 114 Indications of liver disfunction, vitamin such as proliver 1 cap for 3
probably due to illness times a day
34
MONITORING THERAPEUTIC EFFECT

▸ Monitoring decrease of SOGT and / or SGPT

▸ Decreased of symptoms intensity patient (fever, heartburn, muscle
and joint pain)
▸ Regular monitoring of blood profiles
 MONITORING OF ADVERSE EFFECT
No. Drugs Parameter of Monitoring Monitoring Frequency
1. Methylprednisolone Glucose level, total cholesterol levl and bond Every three to six months
density
2. Hydroxyschloroquine Ophtalmonologic examination Every six to twelve months to monitor for
macular damage

3. Furosemide  Hypovolemia During medication use

 Diuresis
 Nausea vomiting
4. Acetaminophen Liver damage (long-term medication) During medication use

5. Codein  constipation During medication use

 Drowsiness
6. Pantoprazole  Headache During medication use
 Abdominal pain
 Vomiting
36
PATIENT EDUCATION

▸ Explanation of what is lupus and its causes

▸ Explain about problem of physical activity because patients need
knowledge of the problem and how to reduce or prevent recurrence, such as
protect the skin from exposure ultra violet light by using sunscreens,
umbrellas or hats; and do regular exercises.
▸ Patients must pay attention if they have an infection.
▸ Patients need to regulate the diet because patients cannot be overweight,
osteoporosis or dyslipidemia.
▸ Explain about drug use includes; type, dosage, duration of administration,
mineral and vitamin supplementation

Indonesian rheumatology association, 2011

37

Daftar Pustaka
▸ Anonim, 2017, Situasi Lupus di Indonesia, Pusat Data dan Informasi Kementerian Kesehatan RI, Jakarta
▸ Anonim, 2011, Diagnosis dan Pengelolaan Lupus Eritematosus Sistemik, Perhimpunan Rheumatologi
Indonesia, Jakarta
▸ Dipiro, J.T., R.L. Telbert., G.C. Yee., G.R. Matzke., B.G. Wells., & L.M. Posey. 2015. Pharmacotherapy A
Pathophysiologic Approach. Ninth Edition. The McGraw-Hill Companies. USA
▸ Nguyet-cam Vu Lam, Md; Maria V. GHETU, MD; And MARZENA L. Bieniek,2016, Systemic Lupus
Erythematosus: Primary Care Approach to Diagnosis and Management, American Family Physician;94(4)
▸ Gordon caoline, Amissah-Arthur, Gayed Mary, et al, 2018, The British Society for Rheumatology guideline
for the management of systemic lupus erythematosus in adults, British Society for Rheumatology;57
▸ Gill JM, Quisel AM, Rocca PV, Walters D, 2003, Diagnosis of systemic Lupus Erythematosus, , American
Family Physician;68(11)
▸ Weckerle, C.E, 2011, The Unexplained Female Predominance of Systemic Lupus Erythematosus: Clues
from Genetic and Cytokine Studies, Clin Rev Allergy Immunol;40(1)
38
REFERENSI

Park H-J, et al, 2013, Low-Dose

Cyclophosphamide Therapy for
Thrombocytopenia in Lupus
39
REFERENCE

The British
Society for
Rheumatology
guideline for
the management
of systemic lupus
erythematosus
in adults, 2018
40
REFERENCE

Systemic Lupus
Erythematosus:
Primary Care
Approach to Diagnosis
and Management
41
REFERENCE

Transfusion of
Packed Red Cells
Indications, Triggers
and Adverse Events,
2015
42
REFERENCE

Dipiro, et al, 2008

43
QUESTIONS
▸Perdani Adnin M / 18/432942/PFA/01842
1. Which one is the best corticosteroid for this patient?
▸Corticosteroids are divided based on work action. The choice of corticosteroids that is
appropriate for SLE patients is recommended for medium-acting corticosteroids such as Methil
Prednisolone, prednisone and prednisolone.. In other article said that mimic naturally occurring
hormones excreted by the adrenal gland and help regulate blood pressure and immune function.
These agents decrease the swelling and pain associated with inflammation, which can occur in
a lupus flare (Mhaidhof and Hilas, 2012). Because of their serious long-term side effects,
corticosteroids should be used at the lowest possible dose and only for periods necessary to
control an active exacerbation of lupus. The dose given to lupus when experiencing flares, malar
rash and some symptoms of active lupus are given high-dose corticosteroids. These flare-ups
are normally managed with corticosteroids until symptoms are put into remission again and
medications may be adjusted (Sweet et al, 2013)
44

▸Perdani Adnin M / 18/432942/PFA/01842

2.The Patient has liver damage but patient gets paracetamol for her treatment. How
about the liver function? Is there any option drug for replace paracetamol?
▸Paracetamol use for treatment in fever symptoms. Fever is a common
manifestation that often appears in SLE patients, so paracetamol therapy is
needed in this condition. Patients cannot get NSAID therapy because NSAIDs can
aggravate the peptic ucer suffered by the patient.
▸To overcome liver problems, sistenol can be given. Sistenol is a combination of
paracetamol and acetylcysteine. The administration of this drug is followed by
strict monitoring of the patient's AST and ALT levels. As is known that
paracetamol is a drug that triggers hepatotoxics, but now the patient's fever can
be controlled by giving paracetamol. This combination is chosen, because
acetylcysteine is a hepatoprotector to keep patients' liver functions back better
and can act as an antidote for paracetamol.
45
Novita Lumintu Sari / 18/432940/PFA/018420

▸3.How about the relation between smoking and SLE?

▸Smoking is an environmental factors can induced lupus with some kind

of stress on the body and trigger with genetic disposision.
46
Linda Dimyati / 18/432935/PFA/01835
▸4. In the presentation, the patient have arthritis symptom. What is the
difference between arthritis and lupus, and explained about the mechanism?
▸Rheumatoid arthritis and lupus arthritis affect peripheral joints. Science is present with
pain, swelling, and stiffness, and both arthritis have systemic manifestations. Even though
they present with the same symptoms, which are different.
▸The process of inflammation due to an autoimmune process in RA, is indicated by
laboratory tests in the presence of RF (Rheumatoid Factor) and anti-CCP in the blood. RF is
an antibody to the Fc component of IgG. B cells, T cells, and pro-inflammatory cytokines play
an important role in the pathophysiology of RA. This happens because the results of
differentiation of T cells stimulate the formation of IL-17, namely cytokines that stimulate
synovitis. Sinovitis is inflammation of the synovial membrane, the tissue that lines and
protects the joint.
▸From here it can be seen clearly the difference between RA and SLE. If SLE pathophysiology
is more dominant due to interferon alfa, RA is predominant in interleukin 17.

(Dipiro 2015)
47
Linda Dimyanti / 18/432935/PFA/01835
▸How the mechanism action of hormonal factor can caused the lupus
disease? Explained about the mechanism.
▸SLE is characterized by a 9: 1 female to male ratio of disease incidence, with a higher female
predominance event during peak reproductive years. Many lines of evidence have implicated IFN-α
in SLE pathogenesis. IFN-α is a pleiotropic type I interferon with the potential to break immunologic
self-tolerance by activating antigen-presenting cells after uptake of self material.
▸One of the major differences between men and women is the ability to carry out placental
reproduction. Elevated IFN-α is thought to play a pathogenic role in SLE, and this cytokine is
expressed by the placenta, and the gene cluster encoding this cytokine has undergone a dramatic
evolution in placental mammals. IFN-α contributes to the success of placental reproduction and
that potential upregulation of this cytokine system in females could both increase reproductive
fitness and simultaneously increase susceptibility to SLE.
▸ Another major difference between men and women is the number of X chromosomes, and both
the number of X chromosomes and genetic variants on the X chromosome are related to the risk
of development of SLE. Two functional X chromosomes, either by sex or by translocation or
duplication, appear to confer a greater risk of SLE than one X chromosome.
Weckerle, C.E, 2011
THANKYOU