Systemic lupus erythematosus: emerging concepts. Part 2: Dermatologic and joint disease, the antiphospholipid antibody syndrome, pregnancy and hormonal therapy, morbidity and mortality, and pathogenesis.
OBJECTIVE
To review 1) advances in the pathogenesis, diagnosis, and management of dermatologic and joint disease and the antiphospholipid antibody syndrome in patients with systemic lupus erythematosus; 2) controversies related to pregnancy and hormonal therapy and to morbidity and mortality in these patients; and 3) current views on the pathogenesis of systemic lupus erythematosus.


METHODS
Review of the English-language medical literature with emphasis on articles published within the last 5 years. More than 400 articles were reviewed.


RESULTS
Despite considerable overlap, cutaneous lesions specific to lupus erythematosus may be divided into subsets with distinct clinical, histologic, and immunofluorescent features. A recent short-term, prospective, uncontrolled trial found hydroxychloroquine and retinoids to be of similar efficacy in the treatment of cutaneous lupus erythematosus. Optimal treatment for patients with lupus and the anticardiolipin antibody syndrome remains to be defined; uncontrolled, retrospective, and treatment-withdrawal studies suggest that warfarin may be more protective than aspirin. Whether pregnancy induces lupus flares has not yet been established; existing data suggest both that it does and that it does not. Oral contraceptive use and postmenopausal estrogen replacement therapy appear not to cause clinical deterioration in patients with lupus. Recent studies have documented a substantial improvement in the survival of patients with systemic lupus erythematosus; they found 5-year survival rates of 90% or more and 10-year survival rates of more than 80%. Most data suggest that systemic lupus erythematosus results from the activation of self-reactive T cells and B cells by genetic or environmental factors.


CONCLUSIONS
The optimal treatment for dermatologic disease and the antiphospholipid antibody syndrome in patients with systemic lupus erythematosus remains unknown. Although mortality has decreased substantially, the morbidity related to the disease itself and to complications of therapy is still considerable. More studies are needed to further elucidate the effects of pregnancy on this condition and the pathogenetic mechanisms responsible for the development of this disease.
Boumpas
D T
DT
National Institutes of Health, Bethesda, Maryland, USA.
Fessler
B J
BJ
Austin
H A
HA
Balow
J E
JE
Klippel
J H
JH
Lockshin
M D
MD
D016736:Antiphospholipid Syndrome / Q000209:etiology; D005260:Female; D006728:Hormones / Q000627:therapeutic use; D006801:Humans; D007592:Joint Diseases / Q000209:etiology; D008178:Lupus Erythematosus, Cutaneous / Q000150:complications / Q000209:etiology / Q000401:mortality; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000209:etiology / Q000401:mortality; D011247:Pregnancy; D011248:Pregnancy Complications
D006728:Hormones