Systemic Lupus

Erythematosus
Justin A. Crocker
AM Report 12/2/09

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.
2.
3.
4.
5.
6.

Butterfly rash
Discoid lupus
Photosensitivity
Oral ulcers
Arthritis
Serositis

7. Neurologic d/o
8. Hematologic d/o
9. Renal d/o
10.Immunologic: antiDNA, anti-Sm, false
pos STS
11.Anti-nuclear antibody

Cutaneous

Most common rash is photosensitive, raised

erythematous malar rash. 55-85% develop at
some point in disease
Discoid Lupus Erythematosus (DLE): 15-30%
circular, scaly hyperpimented lesions with
erythematous rim, atrophic centercan be
disfiguring
Mouth/vaginal/nasal ulcers
Alopecia: may be diffuse or patchy. Occurs 50%

Malar Rash

Discoid Rash

Oral Ulcers

MSK

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
If single joint has persistent pain, consider
osteonecrosis (prevalence increased in SLE over
general population, especially if on steroids.)
Myositis with elevated CK and weakness rarely
occurs

Arthritis

Serositis - Pulmonary

Pleuritis with or without effusion

- if case is mild, tx: NSAIDS
- if case is severe, tx: steroids
Life-threatening manifestations: interstitial
inflammation which can lead to fibrosis and
intra-alveolar hemorrhage.
Also pneumothorax and pulmonary HTN can
occur

Serositis Cardiac

Pericarditis: most common cardiac manifestation

and usually responds to NSAIDs.
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

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: if excruciating, often indicate acute flare
Seizures of any type
Psychosis: must distinguish from steroid-induced
psychosis (occurs in 1st weeks of tx at doses 40mg
prednisone and resolves after several days of reducing
or stopping tx)

Cont.
TIA, Stroke: mostly increased among
patients that are APLA positive
50-fold increase in risk of vascular events
in women under 45 compared to healthy
women
Treatment for clotting event is long-term
anticoagulation

Heme
Anemia: usually Normochromic,
normocytic
Leukopenia: almost always consists of
lymphopenia, not granulocytopenia
Thrombocytopenia

Renal
Nephritis: usually asymptomatic, so
always check UA if patient has known or
suspected SLE
Occurs early in course of disease-if not
present w/in 1 yr, probably will not occur.
Histologic classification by renal biopsy is
useful to plan therapy

Histologic Classifications

Class I is minimal mesangial glomerulonephritis which is

histologically normal on light microscopy but with mesangial
deposits on electron microscopy.
Class II is based on a finding of mesangial proliferative lupus
nephritis. This form typically responds completely to treatment
with corticosteroids.
Class III is focal proliferative nephritis and often successfully
responds to treatment with high doses of corticosteroids.
Class IV is diffuse proliferative nephritis. This form is mainly
treated with corticosteroids and immunosuppressant drugs.
Class V is membranous nephritis and is characterized by
extreme edema and protein loss.
Class VI Glomerulosclerosis

Immunoglobulins
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
APLA: not specific. Used to identify
patients at increased risk for clots,
thrombocytopenia and fetal loss

ANA

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
U/A
ESR
Complement levels
Renal bx 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

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

Cont.

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 lifethreatening disease
Cyclosporine: used in steroid-resistant SLE, risk of
nephrotoxicity
Cyclophosphamide (cytoxan) Almost all trials performed
on patients with nephritis