Although many providers will obtain an antinuclear antibody (ANA) to rule out systemic Lupus erythematosus(SLE), the ANA is an extremely sensitive test, but not very specific, and should be followed up by more definitive tests such as the anti-DS-DNA or anti-Smith antibody
Although many providers will obtain an antinuclear antibody (ANA) to rule out systemic Lupus erythematosus(SLE), the ANA is an extremely sensitive test, but not very specific, and should be followed up by more definitive tests such as the anti-DS-DNA or anti-Smith antibody: Excerpt from Avoiding Common Pediatric Errors
Author:
Sonya Burroughs, MD
What to Do - Gather Appropriate Data
SLE is an autoimmune disorder that affects multiple organ systems. The
causeisunknown,butthehallmarkofthediseaseistheproductionofautoantibodies to the cell nucleus. The incidence of SLE varies significantly among
various ethnic groups. Incidence also varies based on sex. Data suggests
that the incidence rates of SLE with onset <19 years is 6 to 18.9/100,000
in white females, with higher rates in African American (20–30/100,000)
and Puerto Rican females (16–36.7/100,000). The male:female ratio
is 1:4.4.
Although the cause is unknown, there is a strong genetic component
to SLE, with a 25% to 50% concordance rate in monozygotic twins. Approximately 10% to 16% of patients have a first-or second-degree relative
with the disorder. An association with human leukocyte antigen has been
found. The high female-to-male ratio, coupled with the fact that pregnancy
frequently causes flares and worsens nephritis, strongly suggests that female
hormones may play a role in development of the disease. Several drugs are
known to induce lupuslike disease including, but not limited to, hydralazine,
procainamide, isoniazid, minocycline, and D-penicillamine.
TheclinicalfindingsofSLEarearesultofinflammationandbloodvessel
abnormalities. Bland vasculopathy, vasculitis, and immune-complex deposition have all been described. Patients frequently present with complaints of
fever, diffuse hair loss, weight loss, fatigue, and arthralgias or arthritis (especiallyofsmalljointsandwrists).Lymphadenopathyandhepatosplenomegaly
may also be present. The differential diagnosis should include malignancy,
infection, or other autoimmune disorders (i.e., rheumatoid arthritis, and
Sjögren syndrome).
ANAs are seen in up to 95% of patients with SLE. ANA testing is
sensitive, but not very specific for SLE. ANA also occurs in up to 5% of
the normal population. Antibodies to double-stranded DNA (anti-ds DNA)
and to the Sm nuclear antigen (anti-Sm) are found only in patients in SLE
.
The diagnosis is based on history, physical exam, and laboratory findings. Diagnostic criteria have been established by the American Academy of
Rheumatology. These criteria are sensitive (78% to 96%) and specific (89%
to 96%) for SLE. See Table 114.1.
Table 114.1 1997 Revised/Revised Classification
Criteria for Systemic Lupus
Erythematosus (SLE)
Before a patient can be classified as having definite SLE, at least four of the following 11
disorders must be present at some time in the disease course:
• Malar rash: Fixed erythema, flat or raised, over the malar eminences, tending to spare
the nasolabial folds
• Discoidrash:Erythematousraisedpatcheswithadherentkeratoticscalingandfollicular
plugging
• Photosensitivity: Unusual skin rash due to reaction to sunlight, by patient history or
physician observation
• Oral ulcers: Oral or nasopharyngeal ulceration, usually painless, observed by physician
• Arthritis: Nonerosive arthritis involving two or more peripheral joints, characterized
by swelling, tenderness, or effusion
• Serositis: Pleuritis by convincing history or pleuritic pain or rub heard by a physician
or evidence of pleural effusion
OR pericarditis documented by ECG, rub, or evidence of pericardial effusion
• Renal disorder: Persistent proteinuria >0.5 g/day or >3+ or cellular casts, which may
be red cell, hemoglobin, granular, tubular, or mixed
• Neurologic disorder: Seizures or psychosis in the absence of offending drugs or known
metabolic derangements (uremia, ketoacidosis, or electrolyte imbalance)
• Hematologic disorder: Hemolytic anemia with reticulocytosis,
OR leukopenia <4000/mm3 on two or more occasions,
OR lymphopenia <1500/mm3 on two or more occasions
OR thrombocytopenia <100,000/mm3 in the absence of offending drugs
• Immunologic disorder: Positive antiphospholipid antibody or anti-dsDNA antibody
or anti-Sm antibody or false-positive serologic syphilis test known to be positive for
at least 6 months and confirmed by negative Treponema pallidum immobilization or
fluorescent treponemal antibody absorption test
• Antinuclear antibodies: An elevated titer of antinuclear antibody by
immunofluorescence or an equivalent assay at any point in time and in the absence of
drugs known to be associated with "drug-induced lupus" syndrome
ECG, electrocardiogram
Adapted with permission from Tan EM, Cohen AD, Fries JF, et al. The 1982 revised criteria for
the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1274, and Hochberg
MC. Updating the American College of Rheumatology revised criteria for the classification of
systemic lupus erythematosus. Arthritis Rheum. 1997;40:1725, with permission.
The management of SLE is based on symptoms and organ involvement. The treatment can be extremely challenging due to treatment side effects. Corticosteroids are a mainstay of SLE therapy. Symptoms treated with
this modality include constitutional symptoms, arthritis, serositis, nephritis,
cerebritis, vasculitis, and hematologic abnormalities. Despite the fact that
corticosteroids are useful anti-inflammatory agents, their adverse effects and
withdrawal must not be forgotten. SLE is one of many conditions in which
high dose (1–2 mg/kg/day) corticosteroid treatment is necessary. Toxicity
often accompanies this therapy. Cushingoid appearance, obesity, and hypertension are commonly seen as a result of moderate-to-high-dose steroid
therapy. Growth suppression may also occur in children, as does steroid
myopathy. The risk of developing avascular necrosis associated with SLE
is increased further by long-term steroid therapy (4–6 months of continuous use). Other side effects include osteopenia, increased risk of infection,
peptic ulceration, posterior subcapsular cataracts, mood swings, depression, and emotional lability. Remember, sudden withdrawal of prolonged
and high-dose corticosteroid therapy may result in life-threatening adrenal
insufficiency. Patients may exhibit high fever in the absence of infection, hypotension, nausea, vomiting, confusion, hyponatremia, hyperkalemia, and
hypoglycemia. Steroidsupplementationmay also be needed inthesepatients
to prevent addisonian crisis.
Suggested Readings
Gedalia A, Shetty AK. Chronic steroid and immunosuppressant therapy in children. Pediatr
Rev. 2004;25:425–434.
Gottlieb BS, Ilowite NT. Systemic lupus erythematosus in children and adolescents. Pediatr
Rev. 2006;27:323–330.
Taylor ML, Gill JM. Lupus and related connective tissue diseases. Clin Fam Prac. 2005;7:209–
224.
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Book Source Details
- Book Title: Avoiding Common Pediatric Errors
- Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
- Year of Publication: 2008
- Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Avoiding Common Pediatric Errors
Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7489-6
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