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Chap. 24 - EMERGENCY MANAGEMENT OF CONNECTIVE TISSUE DISORDERS AND THEIR COMPLICATIONS

Published online by Cambridge University Press:  07 September 2011

By
Kristen Biggers  and
Noah Scheinfeld
Edited by
Ronni Wolf ,
Batya B. Davidovici ,
Jennifer L. Parish  and
Lawrence Charles Parish
Kristen Biggers
Affiliation:
West Virginia University School of Medicine
Noah Scheinfeld
Affiliation:
Columbia University College of Physicians and Surgeons
Ronni Wolf
Affiliation:
Kaplan Medical Center, Rehovot, Israel
Batya B. Davidovici
Affiliation:
Kaplan Medical Center, Rehovot, Israel
Jennifer L. Parish
Affiliation:
Jefferson Medical College of Thomas Jefferson University
Lawrence Charles Parish
Affiliation:
Jefferson Medical College of Thomas Jefferson University
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Summary

COLLAGEN VASCULAR diseases are complex multiorgan states of pathologic dysfunction. The collagen vascular diseases that most commonly result in emergency situations include systemic lupus erythematosus (SLE), dermatomyositis (DM), and scleroderma. This chapter will review emergency management of connective tissue disorders and their complications. In particular, the clinical and laboratory aids required for diagnosis, therapy, and prognosis will be reviewed. Because we assume that the reader has a basic understanding of the diseases, the chapter does not review them.

SLE

SLE is a complex state of systemic dysregulation that can affect any organ system (see Figure 24.1). The noted writer Flannery O'Connor died at the age of 39, after surgery led to a reactivation and intensification of lupus that resulted in fatal kidney failure. As lupus can be a systemic disease, the most serious emergency management pertaining to it includes cardiovascular, pulmonary, hematologic, neurological, renal, and gastrointestinal (GI) dysfunctions. The prevalence of SLE for 15- to 44-year-old white women has been estimated to be between 18.3 and 40 cases per 100,000 and twice that for 15- to 44-year-old black women. The American College of Rheumatology has established a standard for the diagnosis of SLE, based on the patient having 4 of 11 criteria, including positive titers for various antibodies (Table 24.1).

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Information
Emergency Dermatology , pp. 245 - 255
Publisher: Cambridge University Press
Print publication year: 2011

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