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Comprehensive Review and Assessment for
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Issue: September 1, 2006
Case: Connective Tissue Disorders
Taken from
Med-Challenger EM Career
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Question:
Systemic lupus erythematosus (SLE) is a multiorgan
autoimmune disease hallmarked by the nearly universal presence
of antinuclear antibodies. While the predominant disease pattern
is a vasculitis resulting from deposition of antibody-antigen
complexes, the wide range of autoantibodies produced may also
result in specific antibody-mediated injury as well.
It affects up to 0.05 percent of the population,
predominantly young females (90 percent), and is more common in
African-Americans. Family and genetic studies reveal a genetic
predisposition and correlation with major histocompatibility
complex (MHC) class II and III and other genes, including
homozygous defects of early complement genes. With regard to the
history and physical findings of SLE, all of the following are
true EXCEPT:
Answer Options:
- Fever, fatigue, anorexia, malaise, and weight loss are usually mild and
occur only in a minority.
- Joint symptoms (symmetric arthralgias and polyarthritis; tenosynovitis
and edema favoring the hands, wrists and knees) occur in over 90%.
- Rash occurs in the majority, often a "butterfly" rash of the face or
other sun-exposed areas. Alopecia, conjunctivitis, and oral lesions may
occur.
- Periungual erythema and hemorrhages, splinter hemorrhages, subcutaneous
nodules, skin ulcers, and telangiectasias may occur.
- Vasculitis may cause findings of cerebritis (psychosis,
convulsions, cognitive dysfunction, headache), neuritis,
pneumonitis and restrictive lung disease, myocarditis,
pericarditis, nephritis, diarrhea, hepatitis, and peritonitis.
Answer:
A
Remediation:
Fever, fatigue, anorexia, malaise, and weight loss are
usually prominent. Most patients experience exacerbations alternating with
quiescent periods or true remissions (in up to 20%). Up to 70% of patients
are photosensitive, with disease flare-ups triggered by ultraviolet light.
Foods and chemicals have also been implicated as triggers. Lupus or
lupus-like syndromes can also occur as a reaction to several drugs
(including, most commonly, procainamide and hydralazine). The presence of
anti-histone antibodies is suggestive of drug-induced SLE.Malar rash is
classically associated with SLE. However, a maculopapular eruption on
sun-exposed areas can also be seen.
Discoid lupus erythematosus (DLE) occurs in about 20% of patients with
SLE, and causes focal scarring and permanent alopecia, though only about 5%
of DLE patients will progress to SLE. DLE is characterized by
hypopigmentation with a surrounding rim of hyperpigmentation and follicular
plugging. DLE lesions scar and can result in irreversible alopecia.
About 50% of SLE patients will develop clinical nephritis and risk of
renal insufficiency. Additionally, neurologic manifestations occur in about
50%, cardiopulmonary manifestations in 60%, and ocular manifestations (e.g.,
sicca syndrome, conjunctivitis, episcleritis, retinal vasculitis) are
present in about
15% of SLE patients.
About the Image(s):
Image 1: Systemic Lupus Erythematosus - Butterfly Face
Rash
Systemic lupus erythematosus is generally characterized by
erythema that is sometimes violaceous, occurring especially over the nose
and cheeks. In contrast to discoid lupus erythematosus (DLE), scarring does
not occur. In addition to the face, the rash may be seen in other
sun-exposed areas, such as the V of the neck and the sun-exposed portions of
the forearms and hands.
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