Anincreasingnumberofpatientsareaffectedbycoronary artery disease manifestingasanginaormyocardialinfarction.Thiscomplicationmaybeseeninyoungpatientswithlongstandingdisease,andisespeciallyprevalentinthosewhohavebeentreatedwithcorticosteroids.Thepathogenesisofacceleratedcoronaryatherosclerosisisunclearbutisprobablymulti-factorial.Riskfactorsforatherosclerosis,includinghypertension,obesity,andhyperlipidemia,aremorecommonlypresentinSLEpatientsthaninthepopulationatlarge.Inaddition,immunecomplexesandanti-phospholipidantibodiesmaycauseendothelialdamageandpromoteatherosclerosis.
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Fig. 5.26 Libman-Sacksendocarditisofthemitralvalveinlupuserythematosus.Thevegetationsattachedtothemarginofthethickenedvalveleafletareindicatedbyarrows. (Courtesy of Dr. Fred Schoen, Department of Pathology, Brigham and Women’s Hospital, Boston, Massachusetts.)
Spleen. Splenomegaly,capsularthickening,andfollicularhyperplasiaarecommonfeatures.Centralpenicilliaryarteriesmayshowconcentricintimalandsmoothmusclecellhyperplasia,producingso-calledonion-skin lesions.
Lungs. Inadditiontopleuritisandaccompanyingpleural effusions,somecasesarecomplicatedbychronicinterstitial fibrosisandsecondarypulmonaryhypertension.
Other Organs and Tissues. LE,orhematoxylin,bodiesinthebonemarroworotherorgansarestronglyindicativeofSLE.LymphnodesmaybeenlargedduetohyperplasiaofBcellfolliclesorevendemonstratenecrotizinglymphadenitisduetovasculitis.
SLE is a highly variable multisystem disease, and its diagnosis relies on a constellation of clinical, serologic, and morphologic findings (see
Table 5.10 ). It may be acute or insidious in its onset. Often, the patient is a young woman with some or all of the following features: a butterfly rash on the face; fever; pain without deformity in one or more joints; pleuritic chest pain; and photosensitivity. In many patients, however, the presentation is subtle and puzzling, taking forms such as fever of unknown origin, abnormal urinary findings, or joint disease masquerading as rheumatoid arthritis or rheumatic fever. Generic ANAs, detected by immunofluorescence assays, are found in virtually 100% of patients, but these are not specific for SLE. Renal involvement may produce a variety of findings, including hematuria, red blood cell casts, proteinuria, and nephrotic syndrome (Chapter 14). Anemia or thrombocytopenia are presenting manifestations in some patients and may be dominant clinical problems. In others, neuropsychiatric manifestations including psychosis or convulsions, or coronary artery disease may be prominent. Infections also are common, presumably because of the immune dysfunction that underlies SLE as well as treatment with immunosuppressive drugs.