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systemic lupus erythematosus, animal models 677 systemic type III hypersensitivity<br />

involvement that may take several forms; diffuse proliferative<br />

glomerulonephritis is the most serious. Subendothelial<br />

immune deposits in the kidneys are typical and may exhibit<br />

a “wire-loop” appearance to a thickened basement membrane.<br />

In the skin, immunofluorescence can demonstrate<br />

deposition of immune complexes and complement at the<br />

dermal–epidermal junction. Immune deposits in the skin<br />

are especially prominent in sun-exposed areas. Joints may<br />

be involved, but the synovitis is nonerosive. Typical female<br />

patients have butterfly rashes over the bridges of their noses<br />

along with fever and pain in peripheral joints. However,<br />

the presenting complaints vary widely. Patients may have<br />

central nervous system involvement, pericarditis, or other<br />

serosal cavity inflammation. Pericarditis may be present<br />

along with involvement of the myocardium or cardiac<br />

valves to produce Libman–Sacks endocarditis. Splenic<br />

enlargement, pleuritis, and pleural effusion or interstitial<br />

pneumonitis, and other organ or system involvement may be<br />

observed. Patients may also develop antiphospholipid antibodies<br />

called lupus anticoagulants. They may be associated<br />

with a false-positive VDRL (Venereal Disease Research<br />

Laboratory) test for syphilis. A drug such as hydrazaline<br />

may induce a lupus-like syndrome; however, the antinuclear<br />

antibodies produced in drug-induced lupus are often specific<br />

for histones, a finding not commonly found in classic<br />

SLE. Lupus erythematosus induced by drugs remits when<br />

the drug is removed. Discoid lupus is a form limited to the<br />

skin. Corticosteroids have proven very effective in suppressing<br />

immune reactivity in SLE. In more severe cases,<br />

cytotoxic agents such as cyclophosphamide, chlorambucil,<br />

and azathioprine have been used. Refer to LE cell.<br />

systemic lupus erythematosus, animal models<br />

The main lupus models include inbred murine strains. They<br />

are best suited for investigation because their serologic, cellular,<br />

and histopathologic characteristics closely resemble<br />

those of the corresponding human disease. The three<br />

principal types of lupus-prone mice include New Zealand<br />

(NZB, NZW, and their F1 hybrid), BXSB, and MRL mice.<br />

In addition, single autosomal-recessive mutations termed<br />

gld (generalized lymphoproliferative disease) and lprcg<br />

(lpr-complementing gene) have been described.<br />

Systemic sclerosis.<br />

systemic sclerosis<br />

Refer to progressive systemic sclerosis.<br />

systemic type III hypersensitivity<br />

Refer to type III hypersensitivity.<br />

S

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