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-SLEOral Manifestations and adverse effects

of Lupus in oral cavity

Author: Dr. Boban Fidanoski, DMD

Systemic Lupus Erythematosus is chronic inflammatory multisystem disease of unknown
etiology. It is an autoimmune disease where bodys immune system (antibodies in this case
referred to as autoantibodies) mistakenly attacks its own tissues, causing multi-organ
inflammation and diverse clinical manifestations with domination of peripheral symmetric
polyarthritis of small and large joints. SLE is characterized with periods of exacerbation and
Origins of the name: Systemic Lupus Erythematosus
Lupus is Latin for wolf, Erythro in Greek stands for red, and Systemic is English word
meaning that multiple organs are involved. One theory explains that this disease has gotten
its name because it is similar to the attacks of a wolf on humans with its severity, random
spots of attack and repetitiveness.


SLE results in tissue damage caused by attack of autoantibodies and immune complexes. It
involves polyclonal and antigen-specific T and B lymphocyte hyperactivity. T cell help in
production of autoantibodies is critical for development of full-blown disease.
Proposed Etiology:
Definitive etiology is still unknown. We could only hypothesize what causes this disease is:
genetics, environmental factors (sun exposure to UV light), estrogen (prepubertal and
postmenopausal women have similar incidence to men; men with SLE have higher
concentration of estrogenic metabolites), infection (viral: non-specific stimulant of immune
response, medications (Dilantin-anticonvulsant), oral contraceptive pills are associated with
exacerbation (they should be avoided in SLE patients). 25% of SLE patients have
experienced false-positive tests for syphilis due to circulating lupus anticoagulant in the

Differential Diagnosis:
Diagnostic criteria updated by American College of Rheumatology in 1997 states that at least
4 or more of 11 (7 clinical and 4 laboratory) criteria must be present serially or
Diagnostic Criteria Description
1. Malar rash Classic butterfly rash; sparing of nasolabial folds, no scarring
2. Discoid rash; May cause scarring since invasion of basement membrane
3. Photosensitivity; Skin rush in reaction to sunlight
4. Oral/nasal ulcers; Usually painless
5. Arthritis; Symmetric, involving 2 or more small or large peripheral joints, non-erosive
6. Serositis; Pleuritis or Pericarditis
7. Neurologic disorder; Seizures or Psychosis

8. Renal disorder; Proteinuria
9. Hematologic disorder; Hemolytic anemia, Leukopenia, Lymphopenia, Thrombocytopenia
10. Immunologic disorder; Anti-double stranded DNA Ab (50% of patients), anti-Sm Ab (2560% of patients), anti-phospholipid Ab.
11. Antinuclear antibody (ANA); Most sensitive test (present in 90%of the patients)
Laboratory tests that will determine diagnosis of Lupus:
Serologic diagnosis made by high titre of ANA detected by immunofluorescence, but this
test doesnt determine diagnosis because many other autoimmune disease have positive ANA
Anti-dsDNA antibodies detected by Crithidia test and anti-Sm antibodies are specific for
SLE (95-98% of the cases) and these tests determine the diagnosis of Lupus.

Signs and Symptoms:

1. Musculoskeletal
The most common manifestations of SLE are Arthralgias and Nonerosive Arthritis occurs in
95% of patients, it is symmetric and involves small joints of hands, wrists, and feet).There is
also avascular necrosis (cause of pain, along with arthritis) and myositis.
2. Dermatologic
Abnormalities of the skin, hair or mucous membranes are second most common
manifestation of SLE, occurring in 85% of patients.
The most common skin manifestation is the classic malar butterfly rash, an erythematous
rash covering both cheeks and the bridge of the nose, with sparing of the nasolabial folds.
The second most common skin manifestation is maculopapular rush that can be located
anywhere on the body. Also are present: nasal/genital ulcers, panniculitis, alopecia, urticaria
and purpura.

3. Oral
Painless, shallow oral ulcers, most often occur on the hard and soft palate. There is also a
mild involvement of mucosal ulcers as symptom of this disease.
Oral ulcers occur at onset in 11% of patients, while at any time are present in 30% of
patients. The lesions appear as maculae (red patches) that will later transform into irregular
erosions and ulcers which often heal with scarring. Purpuric lesion such as ecchymoses and
petechiae may occur.
In 30% of the cases, pathology of major salivary glands may occur leading to secondary
Sjogrens syndrome and severe Xerostomia

4. Gastrointestinal
Renal involvement occurs in about 50% of patients, with only few % with irreversible
changes. Proteinuria is the most common clinical sign. Other signs are: Pancreatitis, Lupus
Enteropathy, Hepatitis and Hepatomegaly
5. Systemic
Fever, Malaise/Fatigue, Lymphadenopathy, Weight loss
6. Cardio-Vascular
Pericarditis is the most common cardiac manifestation, occurs up to 30% of patients.
Raynauds phenomenon, Thrombosis, Vasculitis, Livedo reticularis, Hemolytic anemia (most
common vascular manifestation, in almost all patients), Leukopenia (50% of patients),
Lymphopenia, Thrombocytopenia.
7. Ophtalmic
Conjunctivitis, Episcleritis, Keratokonjuctivitis (occurs in 20% of patients)
8. Pulmonary
Interstitial lung disease, Pulmonary hypertension, Alveolar hemorrhage, and Pleuritis.
9. Neurological
Depression, Personality disorder, Cerebritis, Transverse myelitis, Seizures, Headache and

Peripheral neuropathy
First symptoms to occur: 1. Fatigue 2. Myalgias 3. Arthtritis
Radiographic characteristics:
Radiographically, the arthritis of SLE is non-erosive. This is helpful for differential diagnosis
with Rheumatoid Arthritis where there is bone erosion on radiographs.

Microscopic features / histoanalysis:

Three histological lesions are most characteristic of SLE:
1. Onion-skin lesions found in arteries of the spleen, which consist of concentric layers of
fibrosis surrounding the vessel
2. Libman-Sacks verrucous endocarditis: vegetations on heart valves
3. Hematoxylin bodies: globular masses of bluish, dense, homogenous material seen on
hematoxylin and eosin stain.
Microscopic features of oral lesions:
Histologically, lesions reveal lichenoid mucositis with perivascular exudate and thickening of
basement membrane.
Prevalence: 0.05%, 15 to 50 per 100,000 population in USA.
female:male ratio = 10:1 (90% of cases are in women) predominantly in young women due

to higher levels of estrogen, while at premenstrual and postmenopauzal women, ratio with
male decreases to 3:1
More common and severe in Blacks and Asians.
Systemic Lupus Erythematosus is a disease without a known cure, so treatment is based on
relieving symptoms, suppressing inflammation, and preventing future pathology.
Symptomatic treatment is tailored for the organ involved and for severity of the disease: use
of topical sunscreen, avoid UV light and estrogens, use of NSAIDs for arthritis, use of
antimalarials for dermatologic manifestations, use of topical steroids for rash, use of systemic
steroids for prevention of end organ damage. Calcium and Vitamin D to fight osteoporosis,
use of Corticosteroids as immunosuppressant drugs for serious organ involvement (e.g.
Cerebritis, nephritis). All medications used to treat SLE require periodic monitoring for
potential toxicities.
Adverse effects of Lupus therapy in oral cavity:
Long term use of medications to control Lupus can induce significant intra-oral pathology.
Corticosteroids: can lead to root canal calcification, delay of tooth eruptions and root
Steroids: can cause necrotizing ulcerative gingivitis.
NSAIDs: can induce gingival bleeding, but because there is a possibility NSAIDs to inhibit
alveolar bone resorption, periodontal health in some patients with Lupus has been found
improved due to intake of these medications.
Cyclosporine: is a common cause of gingival enlargement (hyperplasia).
Immunosuppressive treatment: fights against intra-oral infections but promotes Candidiasis
and Herpes Simplex Virus infections.

Treatment of adverse effects of Lupus therapy in oral cavity :

Preventive dental hygiene care in Lupus patients is very important. Chlorhexidine
mouthwashes could help contain periodontal disease. Mucous membrane ulcers can be
treated with hydrogen peroxide gargle, buttermilk gargle, or steroid impregnated gel.
Intralesional injection of corticosteroids are also effective. Bacterial, viral and fungal
infection should be treated with conventional, proven therapy specific for the infection
present. Dental procedures should not be undertaken on patients with active Lupus, or if
necessary, antibiotic premedication is advised, due to high incidence of bacterial

Lupus prophylaxis before dental hygiene treatment:

Lupus is considered as high-risk category of disease by The American National Guideline
Clearinghouse and American Academy of Dermatology so they recommend that patients with
this kind of condition require antibiotic premedication before dental treatment.

Survival in patients with SLE is 90 to 95% at 2 years, 82 to 90% at 5 years, 71 to 80% at 10
years, and 63 to 75% at 20 years. Disability in SLE patients is common. 20% of patients
experience remissions. Infections due to immunosuppressive therapy and renal failure are the
leading causes of death in the first decade of disease. Thromboembolic events are frequent
causes of death in the second decade.
Subsets of Lupus:
1. Idiopathic
2. Discoid
3. Subacute cutaneous (ANA negative)
4. Late-onset
5. Neonatal
6. Drug-induced

Quotes about Lupus:

"Homo homini Lupus est." Plautus, year 254 before Christ. (Latin phrase meaning: Man
is a wolf to his fellow-man)

Its never Lupus Dr. Gregory House M.D.

Written literature:
Andreoli, T.E. et al. (1997): Cecil Essentials of Medicine; (4-th ed.)-W.B.Saunders
Fauci, A.S. et al. (1998): Harrisons principles of internal medicine; (14-th ed.)-The
McGraw-Hill Companies INC.,U.S.A.
Shiau, C.J., Toren, A.J. (2006): The Toronto Notes 2006: Comprehensive Medical
References, 26-nd Ed., Canada.
Tierney, L.M. (1997): Pocket guide to the essentials of diagnosis and treatment; (1-th ed.)Lange medical book, U.S.A.
Younger-Lewis,C.; Complete home medical guide;Canadian Medical Association (1-st ed.),
Dk Publishing Inc.
Internet resources:
Long, R.G. et al. (1998): Oral manifestations of systemic diseases, The Mount Sinai Journal
of Medicine(N.Y.-USA) Vol. 65, No.5-6
Sultan, S.M. et al. (1999): A review of gastrointestinal manifestations of systemic lupus
erythematosus, (Oxford Journal of Rheumatology, United Kingdom) Vol. 38, No.10
Norfolk lupus group:
author: Boban Fidanoski; researched for the purposes of the studies at the CCDH and
published on-line in July-August 2007

manifestasi oral pada penyakit lupus eritematosus

Lupus erithematosus adalah suatu kondisi inflamasi yang berhubungan dengan sistem
imunologis yang menyebabkan kerusakan multi organ.. Lupus Eritematosus didefinisikan
sebagai gangguan autoimun, dimana sistem tubuh menyerang jaringannya sendiri.
Klasifikasi Menurut Myers SA and Mary HE, (2001) lupus eritematosus dibagi ke dalam 4
bagian besar, yaitu :
1. Chronic Cutaneous Lupus Erythematosus (CCLE)
Dibagi lagi ke dalam 2 subtipe :
a. Discoid Lupus Erythematosus (DLE)
Dibagi juga dalam beberapa subtipe yang jarang terjadi:
1) Palmar-palmar Lupus Erythematosus
2) Oral Discoid lupus Erythematosus
3) Lupus Erythematosus panniculitis

b. Hypertrophic Lupus Erythematosus (HLE)

2. Subacute Cutaneous Lupus Erythematosus (SCLE)
Memiliki subtype yang jarang terjadi yaitu : Neonatal lupus Erythematosus (NLE)
3. Systemic Lupus Erythematosus (SLE)
4. Drug-Induced Lupus Erythematosus (DILE)

Menurut European Assosiation of Oral Medicine (2005) lupus eritematosus diklasifikasikan

menjadi :
1. Discoid Lupus Erythematosus (DLE)
2. Systemic Lupus Erythematosus (SLE)
3. Bullous form
4. Neonatal form (NLE)
5. Acute Cutaneous form (ACLE)
6. Subacute Cutaneous form (SCLE)
7. Chronic Cutaneous form (CCLE)
8. Childhood onset (CSLE)
9. Drug Induced (DILE)
Lupus Erithematosus merupakan penyakit yang jarang terjadi. Di seluruh dunia diperkirakan
terdapat 5 juta orang mengidap lupus, sedangkan di Amerika Serikat diperkirakan antara
270.000-1.500.000 orang mengidap lupus. Penyakit lupus ditemukan baik pada wanita
maupun pria, tetapi wanita lebih banyak dibanding pria yaitu 9:1, umumnya pada usia 18-65
tahun tetapi paling sering antara usia 25-45 tahun, walaupun dapat juga dijumpai pada anak
usia 10 tahun . SLE ditemukan lebih banyak pada wanita keturunan ras Afrika-Amerika, Asia,
Hispanik, dan dipengaruhi faktor sosioekonomi. Sebuah penelitian epidemiologi melaporkan
insidensi rata-rata pada pria ras kaukasia yaitu 0,3-0,9 (per 100.000 orangper tahun); 0,7-2,5
pada pria keturunan ras Afrika-Amerika; 2,5-3,9 pada wanita ras Kaukasia; 8,1-11,4 pada
wanita keturunan ras Afrika-Amerika. Menelusuri epidemiologi SLE merupakan hal yang
sulit karena diagnosis dapat menjadi sukar dipahami .
Etiologi lupus eritematosus, seperti halnya penyakit autoimun lain, adalah tidak diketahui .
Terdapat dua teori mengenai etiologi lupus, yaitu teori yang pertama menyebutkan bahwa
pada perkembangan penyakit mulai dari gambaran awal sampai timbul kerusakan didasari
oleh produksi sirkulasi autoantibodi menjadi suatu nukleoprotein, yaitu antinuclear
antibodies (ANA). Proses awal tidak diketahui tetapi kemungkinan terjadi mutasi gen yang
berhubungan dengan sel yang mengalami apoptosis yang melibatkan limfosit, kemudian
limfosit bereaksi menyerang selnya sendiri. Teori lainnya menyatakan autoantibodi lupus
eritematosus merupakan lanjutan dari reaksi silang antigen eksogen seperti retrovirus RNA .
Manifestasi Klinis

Manifestasi klinis lupus eritematosus secara umum penyakit lupus eritematosus sistemik atau
lebih dikenal dengan istilah lupus, memiliki manifestasi klinis yang bervariasi, dan
melibatkan multiorgan yaitu sekitar 80% melibatkan persendian, kulit, dan darah; sekitar 3050% melibatkan ginjal, jantung, sistem saraf, sekitar 50 % melibatkan ganguan
gastrointestinal, sekitar 20 % melibatkan gangguan optalmik, dan sekitar 10-30% melibatkan
trombosis arteri dan vena.
Secara umum tanda dan gejala dari lupus diantaranya adalah :
1. kelelahan (fatigue)
2. demam (fever)
3. penurunan berat badan atau sebaliknya
4. malar-rash (butterfly-shaped rash) pada muka
5. lesi di kulit yang bertambah buruk bila terpapar matahari
6. ganguan mulut
7. alopecia
8. raynauds phenomenom
9. nafas yang memendek
10. nyeri dada
11. dry eyes
12. ankietas
13. depresi
14. memory loss
Manifestasi pada kulit dapat berupa lesi ruam diskoid dan ruam malar. Ruam diskoid adalah
ruam pada kulit leher, kepala, muka, telinga, dada, punggung, dan ekstremitas yang
menimbul dan berbatas tegas, dengan diameter 5-10 mm, tidak gatal maupun nyeri. Pada
kepala dapat menyebabkan alopecia yang permanen. Ruam malar adalah ruam yang
menyerupai kupu-kupu pada wajah. Ruam-ruam tersebut dipicu oleh paparan cahaya
matahari... Lesi-lesi tersebut penyebarannya bersifat sentrifugal dan dapat bersatu sehingga
berbentuk ruam yang tidak beraturan. Dapat ditemukan pula berupa lesi kronis malignan,
meskipun jarang, tetapi mengarah pada kanker kulit nonmelanoma. Lesi mirip lichen
planus (LP) juga dapat ditemukan dan seringkali tumpang tindih antara LE dengan LP atau
lesi dapat timbul juga karena penggunaan terapi dengan antimalaria. Penyembuhan dari lesi
diskoid akan meninggalkan jaringan yang atropi dan jaringan parut.

Penyakit lupus pada sistem saraf pusat (SSP) berhubungan dengan beberapa sindrom
neurologik yang berbeda. Manifestasi neuropsikiatrik lupus bervariasi dari ringan (seperti
sakit kepala) sampai berat (seperti stroke). Spektrum manifestasi klinis lupus SSP sangat luas
sehingga merupakan suatu sindrom klinis utama pada lupus SSP yaitu berupa vaskulitis SSP
yang merupakan inflamasi pada pembuluh darah otak karena aktivitas lupus, dan merupakan
satu dari dua sindrom spesifik lupus SSP yang dibuat oleh American College of
Rheumatology.Manifestasi utama dari Lupus SSP :
1. Disfungsi kognitif ( tidak dapat berpikir jernih, defisit memori)
2. Sakit kepala
3. Seizure
4. berubahnya kewaspadaan mental (stupor atau koma)
5. Meningitis aseptik
6. Stroke (gangguan suplai darah pada bagian bagian otak yang berbeda)
7. Periperal neuropathy ( contoh : hilang rasa,rasa geli, rasa terbakar pada tangan dan kaki)
8. Gangguan pergerakan
9. Myelitis (gangguan pada spinal cord)
10. visual alternation
11. Autonomic neuropathy (contoh: reaksi flushing atau mottled skin)
Diagnosis lupus sulit ditegakan karena gejala dan tanda tiap individu bisa berbeda, bisa
berubah dari seiring berjalanya waktu dan overlap dengan penyakit lain yang memiliki gejala
yang sama. Alsan inilah yang sangat dipertimbangkan oleh para klinisi untuk benar-benar
mendiagnosa lupus, bila tanda dan gejala sudah jelas mengarah ke lupus. Untuk membedakan
lupus dengan penyakit lain, ahli medis dariAmerican Rheumatism Association (ACR) telah
nenetapkan 11 kriteria kelainan yang terjadi dalam mendiagnosis lupus eritematosus yaitu
bila ada 4 poin dari 11 manifestasi kelainan. Kriteria ini dikemukan oleh Dr Graham Hughes
pada tahun 1982 yaitu :

1. ruam malar
2. ruam diskoid
3. fotosensitifitas
4. ulser pada rongga mulut
5. artritis
6. serositis
7. gangguan pada ginjal
8. gangguan pada sistem saraf
9. gangguan perdarahan
10. gangguan imunologis
11. antibodi antinuklear
Pemeriksaan penunjang dilakukan untuk menyingkirkan diagnosa penyakit lain, mengetahui
fungsi organ yang menjadi predileksi serangan lupus (tes fungsi ginjal) atau memantau
perjalanan penyakit. Pemeriksaan laboratorium yang berguna diantaranya adalah hematologi
lengkap, tes fungsi ginjal dan hati, urinalisis, dan pemeriksaan serologi.


Antinuclear Antibodi (ANA) Diindikasikan untuk Reumatoid

Tidak spesifik untuk SLE
Antibody t double-stranded

Disarankan untuk sistemik lupus Eritromatosus

Prediktif bila ginjal terlibat

Anti-Smith antibody

Prediktif bila ginjal terlibat

Anti-Ro antibody

Disarankan bila ada sekunder sindroma Sjgrens

Antiphospolipid antibody

Meningkatkan resiko tromboembolisme

Tabel. Tes serologi untuk penyakit lupus eritromatosus

Tujuan penatalaksanaan pada penderita lupus adalah untuk mencegah dan melawan proses
inflamasi yang terjadi, meningkatkan keadaan umum penderita, mengontrol lesi kulit yang
ada, mengurangi bekas luka, dan untuk mencegah pertumbuhan lesi lebih lanjut. Penderita
lupus juga perlu mengetahui kemungkinan adanya manifestasi sistemik yang beresiko serius,

sehingga perlu dilakukan pemeriksaan klinis dan pemeriksaan laboratorium secara reguler
Pengobatan sesuai standar medis meliputi pemberian kortikosteroid (topikal atau intralesi)
dan antimalaria (hydroxychloroquine), Diantara agen imunosupresif (cyclophosphamide,
azathioprine dan mycophenolet) dapat digunakan pada kasus lupus yang berat, namun juga
memiliki efek samping yang berat .diantaranya adalah resiko rentan terkena infeksi,
kerusakan hati, infertilitas dan kanker
Pasien lupus yang memiliki lesi di kulit harus memakai pelindung kulit dari paparan sinar
ultraviolet dengan menggunakan sun-block (SPF) minimal SPF 15 agar melindungi dan
mencegah lesi tidak bertambah parah. Pengobatan lainya pada penyakit lupus eritromatosus
adalah simptomatik sesuai dengan gejala yang muncul.
Manifestasi Oral Pada Penyakit Lupus Eritromatosus
Lesi pada mukosa mulut merupakan yang tersering menjadi target pada lupus eritematosus,
seperti pada diskoid lupus eritematosus dan lupus eritematosus sistemik. Lesi terlihat sebagai
daerah eritematous yang berpusat dan dikelilingi oleh tepi putih yang meninggi. Lesi sering
ditemukan pada palatum, mukosa bukal, dan palatum, dapat tidak spesifik dan terlihat seperti
ulser tanpa rasa sakit. Ulserasi yang terdapat pada rongga mulut pada penyakit lupus menjadi
tanda akibat vaskulitis.Sekitar 75% penderita lupus mengeluhkan gejala pada rongga mulut
seperti rasa kering, rasa sakit, dan rasa terbakar terutama ketika makan makanan panas dan
pedas. Infiltrasi limfosit kelenjar saliva minor ditemukan pada 50-75% pasien, baik mereka
mengeluhkan adanya rasa kering pada mulut ataupun tidak.Salivary flow rate yang tidak
terstimulasi menurun pada banyak penderita lupus eritematosus sistemik. Lupus eritematosus
sistemik juga menjadi komponendiferensial diagnosis dari Sjogrens Syndrome
Lesi spesifik pada rongga mulut penderita lupus eritematosus dapat berupa aphtae ( canker
sores). Pada literatur, aphtae sering disebut juga sebagai stomatitis aphtous rekuren. Lesi ini
mengenai 15% pada populasi normal. Lesi aphtae seringnya berukuran kecil ( kurang dari 1
cm), terasa sakit, dapat ditemukan pada mukosa bukal. Lesi pada lupus eritematosus
cenderung lebih lama, lebih besar, dan terlihat pada palatum. Lesi oral pada penderita lupus
diskoid menyerupai plak berwarna merah yang dikelilingi oleh daerah putih. Lesi ini mirip
dengan lichen planus.

Video latihan SLE

Facts About Systemic Lupus

John Mallozzi, Yahoo! Contributor Network
Jun 4, 2010 "Share your voice on Yahoo! websites. Start Here."

Systemic Lupus
Lupus Treatment



Systemic lupus is an auto-immune disease with no known cause or cure. A person is

naturally equipped with anti-bodies that attack harmful intrusions within the body.
Within the body of a person who has Systemic lupus the anti-bodies for unknown
reasons also attack and damage healthy productive cells and tissue. More women are
diagnosed with Systemic lupus than men with symptoms that affect a wide range of a
person's body including organs, joints, and skin.
Symptoms often come and go with no predictable pattern, which are referred to as a
'flare-up'. A 'flare-up' may be ignited by numerous factors but a common factor among
many people includes direct sun light exposure.
Systemic lupus is considered one of the most difficult disorders to diagnose, and people
with Systemic lupus often suffer for several years experiencing numerous misdiagnoses
including severe fatigue or stress.
Skin rash

A skin rash often appears on a person's face consisting of tiny painless red dots that
spread along the nose, cheeks, and around the eyes forming the pattern of a butterfly or a
wolf's face.
Joint pain
A person with Systemic lupus experiences arthritis type pain in their joints, fingers,
knees, feet, arms, wrists, and hands which often become completely stiff and swollen.
Fingertips often lack cold temperature sensitivity and may even appear pale or purple.
Organ damage
Several organs are often attacked during a 'flare-up' increasing the possibility of causing
irreversible damage even death. The kidneys become damaged in a way that they are
unable to process and remove excess waste. Damage to the lungs lead to severe chest
pain while breathing. People with Systemic lupus are also at a high risk of developing
heart conditions that may lead to a heart attack.
Once a person is diagnosed with Systemic lupus treatment is designed to reduce or
prevent 'flare-up's' and organ and tissue damage.