article Assessment of systemic lupus erythematosus

系统性红斑狼疮的评估

Department of Medicine, Division ofRheumatology, Johns Hopkins UniversitySchool of Medicine, Baltimore, Maryland,USA.

Gordon K.W. Lam, MD; Michelle Petri,MD, MPH.

Please address correspondence to:

Michelle Petri, MD, MPH, Johns HopkinsUniversity School of Medicine, 1830 EastMonument Street, Suite 7500, BaltimoreMD 21205, USA.

E-mail: mpetri@jhmi.edu

Clin Exp Rheumatol 2005; 23 (Suppl. 39):S120-S132.

© Copyright CLINICALANDEXPERIMENTALRHEUMATOLOGY2005.

Key words:Systemic lupus erythema-tosus, lupus activity, organ damage.

ABSTRACT

Systemic lupus erythematosus (SLE) isthe archetypal autoimmune diseasegiven its complex clinical and molecu-lar manifestations. Like the other rheu-matic diseases, appropriate manage-ment is critically dependent upon theproper assessment of disease activity,o rgan damage, and quality of life.Here, we describe the components ofthe comprehensive assessment of SLE,including accurate physical and labo-ratory diagnosis, monitoring of diseaseactivity, recording of accumulated or-gan morbidity, and integration of thesewith the patient’s own perceptions ofhealth status and quality of life. In do-ing so, we will review the most appro-priate laboratory tests and indices cur-rently used in standard clinical careand in clinical research.

Introduction

SLE is an idiopathic connective tissuedisease, the spectrum of which covers awide array of clinical and laboratorymanifestations. While the etiology ofSLE is thought to be multifactorial, thedisease is characterized by the produc-tion of autoantibodies which leads toimmune complex deposition, inflam-mation, and eventually, permanent or-gan damage. SLE is considered to beone of the most common autoimmunedisorders of women of childbearingage, having an estimated prevalence of14.6 to 50.8 per 100,000 persons in thiscategory in the United States (1-3).There is a female:male ratio of approx-imately 6-10:1, with a peak incidencebetween the ages of 15 and 40. Howev-er, SLE can affect all age groups, frominfants to geriatric patients.

Accurate clinical assessment of SLE isdesirable because this disease has acomplex phenotype, a variable diseasecourse, and cumulative morbidity overtime, as new organ system involvementmay be seen over time in many patientseven 5 to 10 years after diagnosis (4).Many studies now show 5-year sur-vival rates exceeding 90% (5-7). How-S-120

ever, the survival of SLE patients hasnot improved since the 1980s, with ath-erosclerosis remaining the major causeof death. Hence, measures for diagnos-ing SLE, monitoring disease activity,assessing tissue damage, and recogniz-ing effects on individual patients are allimportant and necessary.

Assessment of SLE can be divided into4 components: 1) Accurate diagnosis;2) Monitoring disease activity; 3) As-sessment of accumulated damage mor-bidity; and 4) Determining the patient’shealth status throughout his or hercourse.

Diagnosis of SLEClinical manifestations

The complex and protean nature ofSLE demands a meticulously derivedh istory, thorough physical examina-tion, and appropriate laboratory analy-sis. Constitutional symptoms such asm alaise, fatigue, fever, and uninten-tional weight loss are common present-ing symptoms of SLE. These symp-toms are not specific to just SLE, anddiligence should be given to discerningother etiologies such as fibromyalgia,depression, infection, malignancy, endo-crinopathy, or other connective tissuediseases on initial presentation. In addi-tion, environmental triggers such as ex-posure to ultraviolet radiation, infec-tion, or the use of certain medications(such as Echinacea, sulfonamide anti-biotics, minocycline and anti-TNF bio-logics) should be identified, if possible. SLE can affect any organ system andcan present in differing combinations.The most frequent manifestations in-clude: arthritis (64-91%), skin lesions(55-86%), renal involvement (28-73%),Raynaud’s phenomenon (24-61%), cen-tral nervous system involvement (11-49%), gastrointestinal symptoms (39%),pleurisy (27-36%), pericarditis (12-20%),lymphadenopathy (10-30%), ne-phrotic syndrome (13-14%), lung in-volvement (7-14%), thrombophlebitis(5-14%), myositis (4-9%), and myo-carditis (2-3%) (8).