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Systemic Lupus

Yudith Annisa Ayu Rezkitha, dr., Sp.PD
Fakultas Kedokteran
Universitas Muhammadiyah Surabaya
 Autoimmune disease that affects multisystems
 1.5 million cases of lupus
 Prevalence of 17 to 48 per 100,000 population
 Women > Men - 9:1 ratio
 90% cases are women
 African Americans > Whites
 Onset usually between ages of 15 and 45 years,
 Can occur in childhood or later in life
Clinical Manifestations
 For the purpose of identifying patients in
clinical studies, a person has SLE if 4 or
more of the 11 criteria are present,
serially or simultaneously, during any
interval of observation. (specificity 95%,
sensitivity 75%)
 It is important to remember that a patient
may have SLE and not have 4 criteria.
1. Butterfly rash 7. Neurologic d/o
2. Discoid lupus 8. Hematologic d/o
3. Photosensitivity 9. Renal d/o
4. Oral ulcers 10.Immunologic: anti-
5. Arthritis DNA, anti-Sm, false
(musculosceletal) pos STS
6. Serositis 11.Anti-nuclear antibody
1. Malar Rash
2. Discoid Rash
4. Oral Ulcers
5. Arthritis
 Polyarthritis, mild to disabling, occurs most
frequently in hands, wrists, knees. Occurs 90%
 Joint deformities occur in only 10%
 Arthritis of SLE tends to be transitory
 Myositis
6.Serositis - Pulmonary
 Pleuritis with or without effusion
Life-threatening manifestations: interstitial
inflammation which can lead to fibrosis and
intra-alveolar hemorrhage.
 Also pneumothorax and pulmonary
Hypertension can occur
6.Serositis – Cardiac
 Pericarditis: most common cardiac manifestation
 Myocarditis (rare) and fibrinous endocarditis
(Libman-Sacks) may occur. Steroids plus
treatment for CHF/arrhythmia or embolic events.
 MI due to atherosclerosis can occur in <35 y/o
7. Neuro
 Cranial or peripheral neuropathy occurs in 10-15%, it is
probably secondary to vasculitis in small arteries
supplying nerves.
 Diffuse CNS dysfunction: memory and reasoning
 Headache
 Seizures of any type
 Psychosis
8. Heme
 Anemia: usually Normochromic,
 Leukopenia: almost always consists of
lymphopenia, not granulocytopenia
 Thrombocytopenia
9. Renal
 Nephritis
 Histologic classification by renal biopsy is
useful to plan therapy
 Anti-dsDNA IgG: very specific, may
correlate with disease activity
 Anti-Sm: specific, but only present in 25%
of cases, does not correlate with activity
11. ANA (Anti nuclear Antibody)
 ANA: positive in 95% of cases. Pretest
probability affects interpretation. In PCP setting,
2% for SLE. In rheum: 30%
 Low Positive (1:160 or lower): SLE likelihood
<2% (<26% for rheumatologists)
 High Positive (1:320 or higher): SLE likelihood:
2-17% (32-81% for rheumatologists)
 SLE specific patterns: Rim and Homogenous
Additional work-up
- Serum cr. and albumin
- CBC w/ diff
- ESR (Laju endap Darah)
- Complement levels
- Renal biopsy if warranted
 Treatment plans are based on patient age,
sex, health, symptoms, and lifestyle
 Goals of treatment are to:
-prevent flares
-treat flares when they occur
-minimize organ damage and
High Activity Disease level Tx
 Corticosteroids (Mainstay of SLE treatment)
 To rapidly suppress inflammation
 Usually start with high-dose IV pulse and convert
to PO steroids with goal of tapering and
converting to something else.
 Commonly used: prednisone, hydrocortisone,
methylprednisolone, and dexamethasone
 Primarily for CNS/renal involvement
 Mycophenolate mofetil (cellcept)
 Azathioprine (imuran): requires several months to be
effective, effective in smaller percentage of patients
 MTX: for treatment of dermatitis and arthritis, not life-
threatening disease
 Cyclosporine: used in steroid-resistant SLE, risk of
 Cyclophosphamide (cytoxan) Almost all trials performed
on patients with nephritis
Conservative management
 For those w/out major organ involvement.
 NSAIDs: to control pain, swelling, and fever
 Caution w/ NSAIDS though. SLE pts are at
increased risk for aseptic meningitis
 Antimalarials: Generally to treat fatigue joint
pain, skin rashes, and inflammation of the lungs
 Commonly used: Hydroxycholorquine
 Used alone or in combination with other drugs