SYSTEMIC LUPUS

ERITEMATUS

Yuliasih
Sub departemen rematologi – penyakit dalam
RSUD dr Soetomo Universitas Airlangga

Definisi

• Lupus :
o penyakit autoimun
o akut dan kronik
o multisystem
o klinis ada beberapa bentuk
 systemic (SLE),
 discoid (DLE, scarring rash only),
 drug-induced (DILE),
 neonatal (NLE).

Systemic Lupus Erythematosus
• General
– autoimmune multisystem disease
– prevalence 1 in 2,000
– 9 to 1; female to male (1 in 700)
– peak age 15-25
– immune complex deposition
– photosensitive skin eruptions, serositis,
pneumonitis, myocarditis, nephritis, CNS
involvement

45 tahun • kulit hitam > putih • Surve DI USA  1. ultraviolet light. antibiotics. usia 15 .000 penderita • trigger factors chronic inflammatory responses belum dikeyahui • SLE : hasil interkasi yang komplek antara genetik dan faktor lingkungan • Faktor lingkungan : infeksi. hormones • Obat obat tertentu diduga meningkarkan aktivitas respon imun atau menurunkan respon imun procainamide. . obat tertentu . Angka kejadian • ♀ > ♂ perbandingan 9:1.500. hydrazaline. extreme stress.

Pathogenesis kegagalan memproses imun respon Genetik faktor Lingkungan : infeksi . 3. 2. stress sel T& B autoreaktif pembentukan autoantibodi yang berlebihan – 1. sitokine pembentukan imun komplek Kerusakan organ . aktivasi komplemen. sistem koagulasi. Sinar matahari.

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anti La. AUTOIMUNE ?  antibodi gagal mengenali sel tubuh sendiri  Akibatnya?  timbulkan keradangan didalam tubuh  Pada lupus keradangannya timbul bersifat sistemik  Antibodi yang ditemukan: ANA. ds DNA. & anti RNP. anti Ro. .

Systemic Lupus Erythematosus .

Mechanisms of autoantibody production in SLE and APS Mechanisms in Rheumatology ©2001 .

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Mechanisms of renal damage by anti-DNA antibodies Mechanisms in Rheumatology ©2001 .

Tidak semua penderita buterfly rash. kadang hanya 1 atau dua simtom lymphadenopaty . baru kemudian timbul gejala lain  nyeri sendi. efusi pleura. Yang lebih jelas  alopecia. Gambaran klinis Gambaran Klasik :  wanita muda . berbulan bulan.  demam . edema +protein uria . memberi gambaran klinis jelas .

3 turun oMuntah Cepat lelah 84. Gejala non spesifik Pada awalnya bisanya Hasil penelitian : penderita SLE didahului dengan gejala yg dirawat inap di dr Soetomo april –sep 2003 (98 penderita) yg non spesifik odemam omual Berat badan 67.7 oNafsu makan turun demam 53 oBerat badan turun oCepat lelah .

Manifestasi Sistem Muskuloskeletal • Artralgia. Myositis • Tenosinovitis  Jaccoud Arthritis • Osteonekrosis . Artritis • Myalgia.

SLE: Jacoud’s arthropathy .

SLE can cause skin problems…… .

– bila membaik menimbulkan bercak hiperpigmentasi . Manifesatasi sistem mukokutaneus • Manifestasi yang sering dijumpai • Gejala klasik berbentuk malar rash • Gambaran kliniknya beragam • Dikelompokkan : – Kurataneus lupus akut: – sangat fotosensitive. – berlangsung beberapa/ hari minggu.

papulosqumosa 2.ruam anular eritema /eritrema sentrifugum/psoriasis • Distribubusinya pada daerah yg terpapar sinar matahari fotosensitive • Dihubungkan antibodi Ro • Ciri khasnya central area hiperpigmentasi • Bila sembuh tidak menimbulkan jaringan parut . Kutaneus lupus sub akut • ada dua bentuk 1.

– The forehead and V-area of the neck can be similarly involved. (malar rash ). Acute Coetaneous Lupus Erythematosus • ACLE : Localized – characterized by confluent symmetric erythematic and edema centered over the malar eminences The nasolabial folds are typically spared. • generalized ( photosensitive lupus dermatitis” ) .

discrete macular. or papular lesions. a less common variety. or both. that later become confluent and hyperkeratosis . presents as a more widespread morbilliform or exanthemata eruption • ACLE that can stimulate toxic epidermal necrolysis • strong association between ACLE and systemic LE activity. .• ACLE begins on the face as small.Generalized ACLE.

• the pathology is usually nonspecific. transient. buccal. • Superficial ulceration of the oral or nasal mucosa is a frequent accompaniment of ACLE • The posterior areas of the hard palate are most commonly affected.• ACLE :photosensitive . gingival. . however. In the early stages of such lesions. the labial. • Post inflammatory pigmentary change is most prominent in patients with LE with darkly pigmented skin • ACLE lesions do not result in scarring. lasting only several days or weeks. and lingual mucosa may also be involved.

– superficial disseminated LE. pityriasiform LE. and – maculopapular photosensitive LE • SCLE consists of non scarring papulosquamous or annular skin lesions • LE-specific histopathology and occur in a characteristic photo-distribution • a higher frequency of anti-Ro (SS-a) antibody positivity .SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS • SCLE skin lesions including – symmetric erythema centrifugum. – autoimmune annular erythema. – psoriasiform LE. – subacute disseminated in LE. – disseminated DLE.

Bentuk ptiriasis Vesikulo anular bulosa papulosqumosa .

shoulders. V-area of the neck. on the face • Infrequently. and less commonly. SCLE lesions present initially with any appearance of erythema multiforme • Lesions typically heal without scarring .• SCLE lesions : • sun-exposed areas : upper back. extensor aspects of the arms.

adherent scale that extends into dilated hair follicles . • Early DLE lesions most commonly evolve into larger. red-purple macules or papules with a scaly surface. well- demarcated. CHRONIC CUTANEOUS LUPUS ERYTHEMATOSUS Classic Discoid Lupus Erythematosus • DLE lesion of this type begin as flat or slightly elevated. coin-shaped (“discoid”) erythematous plaques covered by a prominent.

• These discoid plaques can enlarge and merge to form even larger. confluent. disfiguring plaques • Involvement of hair follicles is a prominent clinical feature of DLE lesions .

• reversible. eyelids. and extensor aspects of the arms. including the eyebrows. and lips. V- area of the neck. ears. nonscarring alopecia that patients with SLE often develop during periods of s stemic diasease activity. • Irreversible scarring alopecia resulting from permanent follicullar destruction occurred in 34% of patients in one recent series. scalp. Any area of the face can be affected.• Erythema and hyperpigmentation are present during the initial phase of DLE lesions • Typical DLE lesions occur most often on the face. . nose.

• DLE lesions that occur only on the head or neck ( “localized DLE. .” • Painful erosive palmar-plantar DLE involvement can predominate in some cases producing significant disability and presenting an especially difficult management problem • DLE lesions can be precipitated by sunlight exposure. however. this occurs less frequently than with ACLE and SCLE.”) • DLE lesions occuring both above and below the neck are referred to as “generalized DLE.

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Dermatomyositis: Gottron’s Papules SLE: Interarticular Dermatitis .

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buttocks. 1 to 3 cm in diameter. often without overlying clinical change at the surface of the skin • The head. • approximately 1% to 3% of patients with SLE have this complication • Lupus panniculitis/profundus may occur in the absence of systemic disease • The lesions present as deep. PANNICULITIS • characterized by inflammatory lesions in the lower dermis and subcutaneous tissue. and thighs are the sites of predominant involvement . firm nodules. chest. proximal upper arms.

Table Nonspecific Cutaneous Manifestations of Systemic Lupus Erythematosus (SLE) Vasculitis Sclerodactyly Antiphospholipid syndrome Rheumatoid nodules Raynaud phenomenon Erythema multiforme- Alopecia like lesions DLE lesions in the scalp Telogen effluvium Cutaneous mucinosis “Lupus hair” Bullous lesions Pigmentary changes Extension of liquefaction Miscellaneous degeneration of acute Ro (SS-A) Acnetoderma antibody-positive SLE lesions Mid-dermal elastolysis Anti-type VII collagen antibodies Acanthosis nigricans Bullae as the result of putative immune complex vasculitis .

Cutaneous Vasculitis • Reportedly. vasculitis lesions involving arterioles and veins high in the papillary portion of the dermis produce erythema and petechiae . vasculitic lesions occur in 20% to 70% of patients with SLE • The level and intensity of the inflammatory insult of blood vessels in the skin determine the morphologic features of cutaneous vasculitis. • For example.

telangiectasia. as well as in the reticular portion of the dermis. and small infarcts can also result from vasculitic lesions involving small arterioles and venules in the hands • Nailfold erythema. nontender lesions involving the thenar and hyperthenar eminence (Janeway spots) can also occur . may produce palpable and nonpalpable purpuric lesions or urticaria-like lesions. or both. • Intense vasculitic lesions in this area can result in bullous formation or ulcerations • Splinter hemorrhages. cuticle nailfold injection (periungual telangiectasia). • erythematous. have been reported to occur in 10% to 15% of patients with SLE.• Blood vessel involvement deep in the papillary dermis.

Non specific manifestation Urticaria vasculitis Plantar erythema Digital vasculitis Periungual erythema Periungual erythem .

Livido retikularis
Angioedema Angioedema
& ulcer vaskulitis

Digital vasculitis Atrophic of nail Nailfold capillary

RAYNAUD’S PHENOMENON

• Raynaud’s phenomenon is characterized by triphasic
color changes involving the fingers, toes, or both.
a vasospastic component characterized by blanching,
then by a purplish, cyanotic discoloration, followed by
a reperfusion erythema at rewarming associated
commonly with pain

• The pathogenesis of Raynaud’s phenomenon is due to
severe vasospasm involving abnormal digital arteries
having intimal hyperplasia.
• There is no correlation between the severity of the
Raynaud’s phenomenon and SLE disease activity

ALOPECIA
• A third form of alopecia,
also transient, is
probably closely related
in causation to the
telogen effluvium, and is
termed “lupus hair” or
“wooly hair”

SCLERODACTYLY

• Sclerodermatous changes are uncommon in SLE. However, in
recent years patients with U1RNP-positive lupus have been
detected to have sclerodermatous changes characterized
by sclerotic lesions and swollen puffy hands in addition to
Raynaud’s phenomenon

and beard. eyebrows. eyelashes.and hyperpigmentation at sites of previous cutaneous lesions is a common finding in patients with LE • It should be noted that antimalarial therapy has long been recognized as a cause of altered pigmentation. PIGMENTARY CHANGES • Postinflammatory hypo. • These pigmentary changes are reversible if the antimalarial is stopped . This therapy can result in grayness of the scalp hair.

hiperpigmentasi Oral ulcer .

wajah . sembuh 4. menimbulkan jaringan parut. kulit kepala .distribusi : telinga leher.permukaan ada kerak. Kutaneus Lupus Kronik Discoid lesion Discoid lesion Bentuk klasik : DLE awalnya ruam berbentuk bulat sebesar mata uang koin. Manifestasi Sistem Mukokutaneus 3.

• Arm And forearms – Violaceous eryhema ofent extends to extensor aspect of deltoid area. fisure. upper arms. cuticulre dystrophic apprearnce – Closed inspection with in light skinned – reveal dilated cappilary loops or cuticular haemorrhage – Non pruritic hypperkeratotic eruption accopanied by scaling. hiperpigmentation mechanic’s hand – The finger especially at tip may display the physical changes of Raynaud’s phenomenon . forearms – Hand & wirst : periungual erytema.

Nailfold Capillary Exam Normal Systemic Sclerosis Juvenile Adult Dermatomyositis Dermatomyositis .

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Digital vasculitis .

Digital vaskulitis .

Manifesatsi kulit lainnya Non spesifik manifestation .

Livido retikularis & Angioedema angioedema ulcer vaskulitis Lupus Panikulitis/Profundus .

Alopecia Lupus hair Reversible Non reversible alopecia alopecia .

6 % Fotosensitif 28.1 % .8 % Discoid Rash 57.8 % Raynaud’s Phenomenon 4.6 % Oral Ulcer 31.Hasil penilitian : pasien yg dirawat inap di RSUD dr soetomo : april –sept 2006 (98 penderita) Malar Rash 37.1 % Maculopapulo Rash 29.6 % Manifestasi Non Spesifik 62 % Alopecia 39.

Manifestasi Paru • Pleuritis • Pnemonitis • Pulmonary haemorrhage • Emboli paru • Hipertensi pulmonal • Dysfungsi diafragma Pleural efusi Pnemonitis Perdarahan paru Hipertensi pulmonal .

Manifestasi Jantung • Perikarditis • Myokarditis • Endokarditis • Penyakit koroner • Hipertensi • Gagal jantung • Kelainan konduksi .

3 • 51 .CRP ↓ LED • Lymfadenopathy • < 25 8.2 • splenomegali • 25 – 50 15. Manifestasi hematologi • Anemia : penyakit Anemia 21.75 6.3 • Leukopenia Lymfopenia 69.4 kronik.1 .4 • Lymphopenia Trombopenia 45 • Trombopenia • LED ↑.AIHA Lekopenia 17.

Manifestasi Ginjal • Lupus nepritis .

50% penderita diare. obstipasi. Vaskulitis gastrointestinal gastritis. Manifestasi sistem gastrointestinal • Gambaran klinis :( gut Lupus) nyeri perut. mual/muntah. • Bisa merupakan Gejala / Ditemukan selama perjalanan penyakit •  sering dianggap sbg akut abdomen shg dilakukan tindakan surgery/ . oral ulcer. pseudo-obst intestinal. patofisiologi disfagia.

• BOF/LLD ileus paralitik Air fluid level • CT Scan Abd: dilatasi gaster dan dilatasi traktus Dilatasi intestinal Dilatasi gaster intestinal Disertai fecal material .

Manifestasi sistem syaraf pusat Berupa gejala neurologi dan psikiatri Manifestasinya sangat luas Gejala neurologi :  non spesifik : pusing. migrain  spesifik : central dan perifer Nomenclature NPSLE ACR 1999 : 19 sindoma .

fatigue. low grade fevers (kadang high fever). : •metacarpophalangeal. •Non erosive 2. Constitutional symptoms : •malaise. nafsu makan turun •Mual. Polyarthritis. muntah . •BB turun. •lymphadenopathy. •pergelangan tangan. Keluhan yang sering dijumpai 1. •interphalangeal.

Mucocutaneous symptoms :  rash :khas malar rash ( bridge of the nose)  alopecia. .  fotosensitive rash (shawl area).  extensor surfaces of the upper arms. a  anular rash.  discoid rash. • mucosal ulcers • hairline • the finger pulps and periungual areas.3. • vasculitic lesions.

cognitive disorder. leukopenia. . • psychosis. meningitis. seizure. • mood disorder. cytopenia ) 6.4 Proteinuria 5 Hematologic abnormalities (anemia. • movement disorder . • stroke. Neuropsychiatric: • headache. Serositis: Pleuropericardial pain is more frequent Abdominal pain biasanya tidak terdiagnosis secara radiologi maupuin endoskopi 7.

. akan menimbulkan kerusakan ginjal permanen. GINJAL o Keradangan pada ginjal  menimbulkan kebocoran (keluarnya protein). tungkai. o Menyebabkan tubuh kekurangan protein tertentu (albumin) yang akhirnya menimbulkan pembengkakan tubuh yang biasanya dimulai dari muka. perut ( seluruh tubuh) o Jika terlambat pengobatan.

OTAK & SISTEM SYARAF  Pusing  Sering lupa  Sulit berkonsentrasi  Gangguan penglihatan  Kejang  Stroke  Gangguan kepribadian / jiwa .

pulmonary fibrosis. pulmonary hypertension. •lymphopenia . laboratorium yang abnormal : •leukopenia. Myelopathy.8. acute lupus pneumonitis. myocarditis. •positive antinuclear antibody. . baru diikuti gejala lainnya. lupus profundus. 9 . •thrombocytopenia. acute psychosis  jarang merupakan gejala awal SLE Gejala-gejala tersebut bisa muncul bersamaan atau muncul tunggal sampai berbulan-bulan.

lymposit) 2.trombo. serology : C3. Esbach 5.Laboratorium 1. darah lengkap + LED ( hb. kimia klinik : alb. ANA. leko. urobilin. . fs ginjal 4. anti ro/la. Ds DNA . bilirubin ) 3.C4. urine lengkap ( protein uria . transaminase. cast eritrosit. antiphospolipid. hematuria.

Systemic Lupus Erythematosus – specific labs - native(Double stranded) DNA. SM antigen – lupus like reaction – LE cells .

etc 9. Hematologic disorderHemolytic anemia or leukopenia (<4000 twice) o lymphopenia (<1500 twice) or trombocytopenia 10. or false positive test for syphilis) 11. metabolic derangement. PhotosensitivitySkin rash : after exposure to sunlight.5g protein/d or 3+ or cellular casts 8. by physical exam 5. painless. Immunologic disorderAnti-dsDNA or anti-Sm or antiphospholipid antibodies (anticardiolipin. psychosisNot due to drugs. lupus anticoagulant. effusion in 2 or more peripheral joints 6. Seizures. swelling. Nonerosive arthritisTenderness. Positive ANANot drug-induced . Oral ulcersOral or nasopharyngeal. Malar rash : Fixed erythema over malar areas. sparing nasolabial folds 2. Pleuritis or pericarditis Convincing history or physical exam or ECG or other evidence 7. Renal disorder>0. Discoid rashErythematous : raised patches with keratotic scaling and follicular plugging 3. Kriteria ACR 1997 1. history or physical exam 4.

Systemic Lupus Erythematosus: Diagnostic Criteria .

diagnosis •The American College of Rheumatology Classification Criteria for SLE ( 4 dari 11 kriteria terkelompokkan lupus ) • Kriteria ini bermanfaat untuk penelitian bukan untuk diagnosis pasien secara individu •ACR Criteria tidak digunakan untuk mengkonfirmasi atau mengeklude diagnosis lupus •Lupus manifestasinya luas maka untuk diagnosis harus tahu gambaran klinis lupus .

lelah. • azatioprine. matahari ↓ • Farmakologi 1. • cyklosposrin . Chloroquin 4Imunosupresan : • cyklopospamide. STEROID 3. NSAID 2.Pengobatan 1. Non Farmakologi • Stress.

& bercak dikulit • Cortikosteroid: hormon anti inflamasi Cara kerja: secara cepat menekan inflamasi. nyeri sendi. . OBAT YANG DIGUNAKAN • Anti nyeri & radang  NSAID • Anti malaria: untuk mengontrol kelelahan. Digunakan dosis sekecil mungkin sampai penyakit terkontrol.

jantung. .  Immuno supresive: Obat-obat yang digunakan untuk menekan aktifitas penyakit lupus yang sangat berat. HT. Osteoporosis.  Misalnya: lupus yang mengenai otak. Efek samping : DM. & katarak.  Bahaya bila dilakukan penghentian yang mendadak terutama setelah dosis tinggi. dan ginjal. infeksi Osteonecrosis.

HIV.Deferential diagnosis mononucleosis. leukemias. scleroderma. dermatomyositis. and rarely myxoma and Overlap diseases rheumatoid arthritis. post-streptococcal glomerulonephritis) Lymphomas. . endocarditis. or systemic vasculitis. streptococcal sequelae (rheumatic fever. Sjogren syndrome.

SLE and pregnancy • SLE has been stable for more than 1 year. . SLE patients can plan to have a baby. • Prednisone is no more than 10mg/d. and cytotoxic drug has been stopped for more than 6 moth.