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Module 13: SLE Systemic Lupus Erythematosus

Terms in this set (10)

ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Multisystem inflammatory disease of the joints, serosal linings, skin, kidneys, blood, and central nervous system.
Autoantibodies such as ANA, double-stranded DNA, and anti-Smith antibodies are present and related to the pathogenesis of disease.
Systemic lupus erythematosus (SLE) is the prototype of immune complex diseases; its pathogenesis is related to the formation of antibody-antigen complexes that exist in the circulation and deposit in the involved tissues.
The spectrum of symptoms is due to tissue-specific autoantibodies as well as damage to the tissue by lymphocytes, neutrophils, and complement evoked by the deposition of immune complexes
Who is most likely to be affected by pediatric SLE?
most common in girls between the ages of 9 and 15 years.
The American College of Rheumatology has established criteria to aid in the diagnosis of SLE; four of the following 11 criteria are necessary to establish the diagnosis:
Malar rash—photosensitive, so-called butterfly rash on the cheeks and nasal bridge

Discoid rash—annular, scaly rash on the scalp, face, and extremities that can lead to scarring

Photosensitivity

Mucous membrane ulcers

Arthritis—nonerosive arthritis of large and small joints, typically in a symmetrical distribution

Serositis—pericarditis and/or pleuritis, often associated with chest pain and difficulty breathing

Renal abnormalities—proteinuria (> 0.5 g/d) and/or cellular casts

Neurologic abnormalities—seizures and/or psychosis

Blood count abnormalities—low white blood cell count (< 4000/mm3), Coombs test-positive anemia, and/or thrombocytopenia (< 100,000/mm3)

Positive ANA—seen in almost 100% of patients with SLE

Autoantibodies—positive double-stranded DNA antibody, anti-Smith antibody, anticardiolipin antibodies, lupus anticoagulant, and/or false-positive blood test for syphilis
Common S&S of SLE are:
fever, fatigue, weight loss, anorexia, Raynaud phenomenon, myositis, vasculitis, chorea, neuropathies, depression, and cognitive changes.
What important laboratory finding would you expect to find in SLE?
The ANA test is positive in almost 100% of patients, usually at titers of 1:320 or above. In patients with suspected SLE, it is important to obtain a full ANA profile—including antibodies directed against double-stranded DNA, Smith, ribonucleic protein, and Sjogren's specific antibody A and B to better characterize their serologic markers of disease.
What other labs would you order for a patient with suspected SLE?
Complete blood count abnormalities are common, including leukopenia, anemia, and thrombocytopenia. Approximately 15% of patients are Coombs test-positive, but many patients develop anemia due to other causes, including chronic disease and blood loss. Patients with significant renal involvement may have electrolyte disturbances, elevated kidney function tests, and hypoalbuminemia. The ESR is frequently elevated during active disease. In contrast, many patients with active SLE have a normal CRP. When the CRP is elevated, it is important to investigate possible infectious causes, particularly bacterial infections. It is critical to monitor the urinalysis in patients with SLE for proteinuria and hematuria, as the renal disease may be otherwise clinically silent.
Patients who have evidence of antiphospholipid antibodies should be treated with what?
HINT: Thrombotic events due to these antibodies require long-term anticoagulation.
Aspirin
The treatment of SLE should be tailored to the organ system involved so that toxicities may be minimized.
Prednisone is the mainstay of treatment and has significantly lowered the mortality rate in SLE. Patients with severe, life-threatening, or organ-threatening disease are typically treated with intravenous pulse methylprednisolone.
The prognosis in SLE relates to the presence of renal involvement or infectious complications of treatment.
The survival rate has improved to 90%. The disease has a natural waxing and waning cycle; the disease may flare at any time and spontaneous remission may rarely occur.
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