Lupus Erythematosus
Lupus Erythematosus: Excerpt from The 5-Minute Pediatric Consult
Elizabeth Candell Chalom, MD
Lupus Erythematosus - BASICS
Lupus Erythematosus - description
Multisystem, autoimmune disease characterized by production of antibodies to various components of cell nucleus, in conjunction with variety of clinical manifestations
Lupus Erythematosus - epidemiology
- Age:
- Onset: 20% in childhood, but very rare under 5 years old
- Female:Male ratio: Between 3–5:1 (prepubertal) and 9–10:1 (postpubertal)
Lupus Erythematosus - incidence
Peak incidence: Between ages 15 and 40 years
Lupus Erythematosus - prevalence
US estimate: 5,000–10,000 children
Lupus Erythematosus - risk factors
Lupus Erythematosus - genetics
- Increased frequency in 1st-degree family members of patients with systemic lupus erythematosus (SLE)
- 10% of patients have ≥1 affected relative.
- Concordance rate of 25–50% in monozygotic twins and 5% in dizygotic twins
- Some major histocompatibility antigens associated with increased incidences of lupus, such as HLA-DR2 and DR3 in whites and DR2 and DR7 in blacks
Lupus Erythematosus - etiology
Although exact etiology is unknown, lupus is an autoimmune disease, with genetic, environmental, and hormonal factors playing a role.
Lupus Erythematosus - DIAGNOSIS
Diagnostic criteria: 4 of following 11 criteria, developed by the American College of Rheumatology, must be met to classify a patient as having SLE:
- Malar (butterfly) rash
- Discoid rash
- Photosensitivity
- Oral or nasal ulcers
- Arthritis
- Cytopenia: Anemia, leukopenia (<4,000/mm3), lymphopenia (<1,500/mm3), or thrombocytopenia (<100,000/mm3)
- Neurologic disease: Seizures or psychosis
- Nephritis: >0.5 g/d proteinuria or cellular casts
- Serositis: Pleuritis or pericarditis
- Immunologic disorder: Antibodies to double-stranded DNA, Smith nuclear antigen, LE-cell preparation, false-positive serologic test for syphilis, or antiphospholipid antibodies (anticardiolipin antibodies or positive lupus anticoagulation test)
- Positive ANA
Lupus Erythematosus - signs & symptoms
- Immune complex–mediated vasculitis, which can occur in almost any organ system
- Cutaneous lesions: Very variable. Include:
- Erythematous malar or “butterfly” rash
- Maculopapular rashes (which can occur anywhere on body)
- Periungual erythema
- Mucosal membrane vasculitis
- Arthritis: Can affect large and small joints; usually symmetric and nonerosive
- Hematologic pathology: Includes:
- Hemolytic anemia
- Anemia of chronic disease
- Leukopenia
- Lymphopenia
- Thrombocytopenia
- Neurologic symptoms include:
- Headaches
- Psychosis
- Depression
- Seizures
- Organic brain syndromes
- Peripheral neuropathies
- Renal pathology (present in up to 75% children with SLE)
- Includes mesangial changes and glomerulonephritis (focal, diffuse, proliferative, or membranous)
- 1st signs of renal disease in lupus patient are often proteinuria and active urinary sediment.
- Hypertension, nephrotic syndrome, and renal failure can also occur.
- Serositis: Usually seen as pericarditis or pleuritis, but peritonitis can also occur.
Lupus Erythematosus - history
- History of photosensitivity or malar rash common but not necessary
- Many patients have systemic complaints, such as fevers, fatigue, and malaise.
- Many patients complain of joint pain, Raynaud phenomenon, or alopecia.
- Chest pain from pericarditis or pleural effusions may be present.
- Raynaud phenomenon can be associated with lupus.
Lupus Erythematosus - physical exam
- Rash: May be malar, discoid, or vasculitic. Periungual erythema may also be seen.
- Oral or nasal ulcers (usually on hard or soft palate) that are painless and often go unnoticed by patients
- Arthritis of large and small joints
- Pericardial friction rub if patient has pericarditis
- Edema may be present secondary to renal disease.
- CNS changes such as personality changes, psychosis, or seizures
Lupus Erythematosus - tests
Lupus Erythematosus - lab
- ANA:
- Found in >95% patients with SLE, but positive ANA can occur in many diseases (and in 10–20% of normal population)
- Anti–double-stranded DNA and anti–Smith nuclear antigen:
- Very specific to lupus, but not all patients with lupus have these autoantibodies. In many patients, anti-DNA levels vary with activity of disease.
- CBC:
- Anemia, leukopenia, lymphopenia, and/or thrombocytopenia may be seen.
- Urinalysis:
- May show proteinuria or active urinary sediment if there is renal dysfunction
- Complement levels:
- Can fall very low during a lupus flare (C3 and C4)
- PTT:
- Patients may also have prolonged PTT, as result of antiphospholipid (APL) antibodies, often seen in SLE. Patients with APL antibodies at increased risk for thrombotic events, such as deep venous thromboses, strokes, and fetal losses during pregnancies.
Lupus Erythematosus - differencial diagnosis
- Systemic juvenile-onset rheumatoid arthritis
- Oncologic disease (leukemia, lymphoma)
- Other vasculitic disorders
- Dermatomyositis
- Fibromyalgia
- Drug-induced lupus
- Pitfalls:
- Avoid overdiagnosis; positive ANA in the absence of clinical signs or symptoms of SLE is not lupus.
Lupus Erythematosus - TREATMENT
Lupus Erythematosus - general measures
Avoid excessive sun exposure and use sunscreen liberally.
Lupus Erythematosus - special therapy
For very severe lupus, bone marrow immunoablation or transplantation are options.
Lupus Erythematosus - medication
- NSAIDs may be used for musculoskeletal and mild systemic complaints, although ibuprofen has been noted to cause an aseptic meningitis in a small number of patients with SLE.
- Hydroxychloroquine often used to help control cutaneous manifestations
- Steroids often necessary to control systemic and renal manifestations
- Patients with renal disease often need immunosuppressive agents, such as cyclophosphamide (usually given as monthly IV boluses). Mycophenolate mofetil, cyclosporin, or azathioprine may also be used.
- Patients with mainly arthritic symptoms may be treated with weekly methotrexate, PO or SC
- Patients with antiphospholipid antibodies are often treated with a baby aspirin daily. If they have already had a significant clotting event, they need stronger anticoagulation.
- Angiotensin converting enzyme inhibitors are often used to help prevent renal damage from proteinuria.
- Patients with abnormal lipid profiles that does not respond to diet may need statins
- Rituximab (anti-CD20 antibody) causes B-cell depletion and is now being used in SLE, especially for thrombocytopenia.
- Other biologic agents also being tested in lupus (e.g., antibodies to CD40 and C5)
Lupus Erythematosus - FOLLOW UP
Lupus Erythematosus - prognosis
- Extremely variable. Renal disease and CNS involvement are poor prognostic signs, whereas systemic complaints and joint findings are not.
- 10-year survival in child presenting with SLE is >90%
Lupus Erythematosus - complications
- End-stage renal disease
- Infections secondary to treatments used to control disease
- Atherosclerosis and myocardial infarctions at a young age
- Libman-Sacks endocarditis which increases risk of subacute bacterial endocarditic.
- Neonatal lupus:
- Neonatal lupus (NLE) is due to maternal autoantibodies (usually SS-A or SS-B antibodies) that cross the placenta and can cause rashes, congenital heart block, cytopenias, and/or hepatitis in the newborn baby.
- Most symptoms of NLE resolve by 6 months of age, but the heart block, if it occurs, is permanent.
- Many mothers of babies with NLE are asymptomatic and unaware that they have these autoantibodies.
- The rash, erythema annulare, can begin a few days after delivery or within the 1st few weeks of life.
- Topical steroids can minimize the lesions. Congenital heart block is due to damage of the conducting system of the developing fetal heart.
- Bradycardia may be noted by 22 weeks’ gestation, and CHF with nonimmune hydrops fetalis may ensue.
Lupus Erythematosus - bibliography
- Godfrey T, Khamashta MA, Hughes GR. Therapeutic advances in systemic lupus erythematosus. Curr Opin Rheumatol. 1998;10:435–441.
- Gottlieb BS, Ilowite NT. Systemic lupus erythematosus in children and adolescents. Pediatrics in Review. 2006;27(9):323–330.
- Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. [Letter] Arthritis Rheum. 1997;40(9):1725.
Schumacher HR, ed. Primer on the Rheumatic Diseases. 10th ed. Atlanta, GA: Arthritis Foundation; 1993.
Lupus Erythematosus - CODES
Lupus Erythematosus - icd9
710.0 Systemic lupus erythematosus
Lupus Erythematosus - FAQ
- Q: If a patient has a positive ANA but no clinical signs of SLE, how often should the ANA be followed?
- A: A positive ANA will usually remain positive indefinitely, but it has no real significance in the absence of clinical or other laboratory disturbances. Up to 20% of the normal population may have a positive ANA, so there is no need to repeat the test.
- Q: Can SLE patients with end-stage renal disease obtain renal transplants?
- A: Yes, and SLE usually does not recur in the new kidney.
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, 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: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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