Systemic Lupus Er
ythematosus (SLE)
What is lupus ?
● Systemic lupus erythematosus (SLE/‘lupus’) is a multisystem
autoimmune disorder with a broad spectrum of clinical features
involving almost all organs and tissues
● Chronic inflammatory disease with genetic, environmental, and
hormonal factors
● The pathophysiology multifactorial and incompletely understood
but involves autoantibody production, deposition of immune
complexes, complement activation, and accompanying tissue
destruction/vasculitis
● SLE is 10–20× more common in women than men, and most likely to
develop between the ages of 15 and 40 years
● It is more common and often more severe in certain ethnic groups
such as those of African-Caribbean, India, Hispanic, and Chinese
origin living in the USA and Europe than in white Caucasians
● Women of childbearing age account for 90% of cases, but men
have more severe disease.
● Milder in elderly patients; more severe in children usually appears in
late childhood or adolescence
Signes Clinique
● Signes generaux : Anorexie,asthenie,fievre
● Signes cutanes :
1. Lesions cutanees specifiques
• Lupus cutane aigu: Éruption érythémateuse et squameuse malaireen
vespertilio (nez et joues) , Lésions erythémato-squameuses du décolleté
et face dorsale des doigts
• Lupus cutane chronique : discoide plaques bien limitees laissant une
cicatrice atrophique,autes aspects: lupus tumidus ,panniculite,lupus
engelure
• Photosensibilite: atteinte des zones photo-exposees
2. Lesions non specifiques :
• Lesions des muqueuses : rosions muqueses buccales :
gencives,lalais ,levres
• Lesions de phaneres : alopecie diffuse lors des poussees
• Lesions de vascularite lupique : purpura
vasculare,necrose,ulceration,livedo racemosa
Lesions vasculaire de lupique Lesions de muqueuse de buccale
Lesions rash de lupus
butterfly rash Discoide lesion in SLE Non-scaring alopecia
● Signes osteo-articulaires :
1. Atteinte articulaire :
• Polyarthrite bilaterale symetrique non erosive
• Arthralgies inflammatoires
• Rhumatisme de jaccoud ( deformant )
2. Atteinte osseuse :
• Osteonecrose aseptique
• Osteoporose
3. Atteinte musculo tendineuse : myalgies , rarement myosite ( anti-
RNP) ,Tenosynovites
● Atteinte renale :
1. Glomerulonephrite lupique :
• Facteur pronostique majeur du lupus
• Souvent asymptomatique : depistage par bandelette urinaire
reguliere
• Biopsie renale +++
• ACR OU protein dans urine 24h > 500mg
2. Autres atteintes renales :
• Atteinte tubule-interstitielle ( plus rare )
• Atteinte vasculaire ( rechercher SAPL ++)
● Atteinte cardio-vasculaire :
1. Atteinte cardiaque :
• Pericardite ( dyspnea,douleur thoracique ) ( plus courant )
• Myocardite
• Endocardite de liebmann-sacks
2. Atteinte vasculaire :
• Atherosclerose ( insuffisance coronaienne )
• Syndrome de taynaud
• Hypertension arterielle
• Thrombose veineuse ou arterielle ( SAPL) ,Vascularite lupique
( rare )
● Atteinte respiratoire :
• Pleuresie exudative lymphocytaire
• Pneumopathie interstitielle ( rare )
• HTAP ( pri ou post embolique )
● Atteinte neurologique
1. Systeme nerveux central :
• Cephalees ,convulsions
• Atteinte demyelinisante,myelopathie
• Menigite aseptique
• Troubles cognitifs,movemnets anormaux
2. Systeme nervex pheripherique
• Guilain-barre
• Polyneuropathi,myasthenia secondaire
• Atteinte hematologique
1. Clinique : adenopathies peripheriques,splenomegalie
2. Biologique : Leucopenie(< 4000/mm3 ) ,thrombopenie
(<100,000/mm3 )
3. ,anemie par hemolyse
● Atteintes plus rares :
1. Atteinte digestive et hepatique
• Ascite,hepatite auro-immune
• Vascularite mesenterique
2. Atteinte ophtalmo:
• Retinite
• Syndrome sec ( sjogren associe)
• Associations pathologique : autes maladies auto-immunes associees :
• Sjogren,connectivite mixte,thyroiditis
Diagnosis
● Diagnosis is based on a combination of clinical and laboratory
findings. Several diagnostic criteria have been used, including the
1997 ACR criteria, the 2012 SLICC criteria, and the 2019
EULAR/ACR classification criteria. In real world practice, the
2019 EULAR/ACR criteria may be less sensitive than the 1997
ACR or 2012 SLICC criteria.
● 2012 SLICC criteria: The patient must have at least four criteria (at
least one clinical criteria and one immunologic criteria) OR
biopsyproven lupus nephritis with a positive ANA or anti-dsDNA.
● The Systemic Lupus Erythematosus International Collaborating
Clinics (SLICC) is an international group of rheumatologists and
immunologists with special interests in lupus who have been working
together on lupus research since 1991
2012 SLICC
● Clinical criteria :
1. Acute cutaneous lupus
2. Chronic cutaneous lupus
3. Nonscaring alopecia
4. Oral or nasopharyngeal ulcers
5. Arthritis
6. Serositis
7. Proteinuria and cellular casts
8. Seizures, psychosis, or other neurologic manifestations
9. Autoimmune hemolytic anemia
[Link] or lymphopenia (< 4000/mm3 )
[Link]
● Immunologic criteria
1. Antinuclear antibodies (ANA)
2. Anti–double-stranded DNA antibodies, anti-Smith (SM)
antibodies,or antiphospholipid antibodies
3. Anti-Sm antibody
4. Antiphospholipid antibody
5. Low complement levels
6. Positive Coombs test
֍. SLE should almost never be diagnosed in the absence of an elevated ANA titer
( Current diagnosis and treatement 2023 )
Management of SLE
● Preventive treatment: avoid sun exposure, smoking cessation,
vaccines, diet and exercise
● Treatement is depend on the severity of the SLE itself ( Mild?
Moderate ? Severe? )
● NSAIDs usually control SLE-associated arthritis, arthralgias, fever,
and mild serositis but not fatigue, malaise, or major organ system
involvement.
● Hydroxychloroquine :
○ 200 or 400 mg bid may be effective in the treatment of
rash, photosensitivity, arthralgias, arthritis, alopecia, and
malaise associated with SLE and in the treatment of
○ The drug is not effective for treating major organ
manifestations, but long-term usage reduces both disease
progression as well as number of flares.
○ Patients should be advised about the need for annual eye
testing, and after 5 years of use they should be referred to an
ophthalmologist to assess for retinal toxicity
○ For patients intolerant of HCQ, chloroquine phosphate 250
mg daily is a useful alternative.
● Glucocorticoids (GCs)
○ Prednisolone is frequently used with immunosuppressive
drugs to rapidly reduce SLE disease activity.
○ prednisone, 1–2 mg/kg PO daily, which can be given in divided
doses. After disease is controlled, prednisone should be tapered
slowly, with the dosage being reduced by no more than 10%
every 7–10 days
○ Intravenous ‘pulsed’ methylprednisolone 0.25–1 g used
daily for 3 consecutive days is preferred in serious organ or life-
threatening disease
● Immunosuppressive therapy :
○ Indications for immunosuppressive therapy in SLE include
lifethreatening manifestations of SLE such as
glomerulonephritis, CNS involvement, thrombocytopenia,
hemolytic anemia, and the inability to reduce corticosteroid
dosage or severe corticosteroid side effects.
○ Choice of an immunosuppressive therapy is individualized to the
clinical situation. Cyclophosphamide is used for life-threatening
manifestations of SLE. High-dose monthly IV pulse
cyclophosphamide (0.5–1 g/m2) may be less toxic but is also
less immunosuppressive than low-dose daily oral
cyclophosphamide (1– 1.5 mg/kg/d).
o Azathioprine (1–3 mg/kg/d) and mycophenolate mofetil
(500–1500 mg bid) are also used as steroid-sparing agents for
serious lupus manifestations
o Methotrexate (7.5–20 mg weekly) is often used for
musculoskeletal and skin manifestations
o Other: Belimumab , Rituximab
o All patients, not just those with photosensitive rashes, are
advised on use of sunscreens with sun protection factor (SPF) of
30 or greater, protective clothing, and sun avoidance. Isolated
skin lesions may respond to topical steroids.
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