Munther A. Khamashta

Antiendothelial cell antibodies (AECA) in systemic lupus erythematosus (SLE)

ConclusionThere is now overwhelming evidence that AECAs exist and are commonly found in the sera of patients with SLE and other autoimmune connective tissue dieases. In many of these diseases, the antibodies mirror disease activity; in SLE, they are associated with clinical manifestations such as nephritis, vasculitis, and thrombosis. The main challenges for the future include the practical aspects of standardizing a technique for measuring AECAs and assessing the potential role for these antibodies in pathogenicity.

40 – THE CLINICAL ASPECTS OF THE ANTIPHOSPHOLIPID SYNDROME

The antiphospholipid syndrome (APS) is a noninflammatory autoimmune disease. The most critical pathologic process is thrombosis, which results in most of the clinical features suffered by these patients. Recurrent thrombosis together with an adverse pregnancy history and the presence of persistently elevated levels of anti-phospholipid antibodies defines the syndrome. A patient with APS must manifest at least one of two clinical criteria (vascular thrombosis or pregnancy morbidity) and at least one of two laboratory criteria (positive lupus anticoagulant or medium-to-high titer β 2 -glycoprotein I-dependent IgG or IgM anti-cardiolipin antibody, confirmed on two separate occasions, at least 6 weeks apart). Clinicians should recognize that the International Consensus Criteria were developed primarily for research purposes to ensure more uniform characterization, as well as subcategorization,of patients included in the studies. This discussion views this objective as crucial for credible investigative efforts and for appreciation of subtleties of treatment. As with other autoimmune conditions, such as systemic lupus erythematosus, there are individuals who present with one or more clinical or laboratory features suggestive of APS, but in whom the diagnosis cannot be made by the relatively strict International Consensus Criteria. In such cases, experienced clinical judgement is required for best care.

Systemic Lupus Erythematosus, Antiphospholid Syndrome, Scleroderma, and Inflammatory Myopathies

Systemic lupus erythematosus (lupus, LE, SLE) is a genetically determined disease characterized by diverse clinical features [1].