Complex autoimmune inflammatory disease that can affect virtually any organ system in the body, and the presentation can vary considerably from patient to patient.

A patient is said to have a high likelihood of having systemic lupus erythematosus (SLE) if that person exhibits 4 of the following 11 features:

Coronary artery disease is a leading cause of premature death in SLE. In young women with SLE, the risk of myocardial infarction is increased 50-fold.

Drug-induced lupus is a syndrome of lupus-like illness associated with the ingestion of certain medications (procainamide, hydralazine, á-methyldopa, isoniazid, quinidine).

Syndrome characterized by recurrent arterial and/or venous thromboses, recurrent fetal loss, and thrombocytopenia in association with sustained elevated titers of antiphospholipid antibodies.

The initial testing consists of a lupus anticoagulant assay (usually the aPTT) plus an anticardiolipin ELISA. If negative or equivocal, further testing with other coagulation tests (i.e., DRVVT, β₂GP-I ELISA, or assays for other phospholipids) can be pursued.

The onset is typically heralded by Raynaud’s phenomenon, with the later development of sclerodactyly and skin thickening and fibrosis over the hands, arms, legs, face, and trunk.

Late complications of the musculoskeletal disease include joint contractures, muscle atrophy, and skin ulceration over the contracted joints.

Internal organ involvement includes interstitial lung disease, cardiac arrhythmias, rapidly progressive renal failure, esophageal dysmotility, and gut hypomotility.

Antibodies to topoisomerase I (anti-Scl-70)—70% of patients with diffuse scleroderma.

Limited scleroderma, including CREST (Calcinosis cutis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia) syndrome, is characterized by a more restricted cutaneous disease.

Anticentromere antibodies—95% of patients with CREST.

Scleroderma renal crisis is a form of rapidly progressive renal insufficiency that can affect patients with diffuse scleroderma.