Portofolio SH
Portofolio SH
Portofolio SH
Erythematosus
Yudith Annisa Ayu Rezkitha, dr., Sp.PD
Fakultas Kedokteran
Universitas Muhammadiyah Surabaya
SLE
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,
but
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.
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
(Musculosceletal)
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
difficulty
Headache
Seizures of any type
Psychosis
8. Heme
Anemia: usually Normochromic,
normocytic
Leukopenia: almost always consists of
lymphopenia, not granulocytopenia
Thrombocytopenia
9. Renal
Nephritis
Histologic classification by renal biopsy is
useful to plan therapy
10.Immunologic
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
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
complications
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
Immunosuppressives
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
nephrotoxicity
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
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