CLASSIFICATION CRITERIA The initial classification criteria for APS (Sapporo, 1998) were revised in 2006. These include clinical (vascular thrombosis and pregnancy-related morbidity) and laboratory (presence and measurement of antibody titers…
Causes of anemia in SLE patients include chronic inflammation, hemolysis, blood loss, renal insufficiency, myelodysplasia, hypersplenism, or marrow aplasia. A white blood cell count lower than 4500/µL has been reported…
Renal Manifestations. Renal involvement eventually will affect up to 70% to 80% of all patients, but the clinical presentation varies and includes an abnormal urine sediment; nephritis, nephrotic syndrome; or…
A positive ANA test alone is not sufficient to establish the diagnosis. Survival rates in SLE are much better today than 50 years ago. Morbidity comes from disease itself, as…
CHURG-STRAUSS SYNDROME Epidemiology/Clinical Manifestations. Churg-Strauss syndrome (CSS) is a rare disease observed in all age groups and occurring equally between sexes with an incidence of about 3 per million people….
Antineutrophil cytoplasmic antibodies (ANCA) have had important clinical applications in GPA, although their role in pathogenesis remains uncertain. Two types of ANCA have been identified in patients with vasculitis: ANCA…
POLYARTERITIS NODOSA Epidemiology/Clinical Manifestations. Polyarteritis nodosa (PAN) was reported by Kussmaul and Maier in 1866 and was the first vasculitis to be described. The definition of PAN has evolved and,…
PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, ADENOPATHY SYNDROME Periodic fever, aphthous stomatitis, pharyngitis, adenopathy (PFAPA) syndrome is a common cause of recurrent fever in children but has also been reported in…
TUMOR NECROSIS FACTOR RECEPTOR–ASSOCIATED PERIODIC SYNDROME Tumor necrosis factor receptor–associated periodic syndrome (TRAPS) results from alterations in the gene encoding the TNF cell surface receptor. TRAPS has a widely variable…
PRIMER ON THE INNATE IMMUNE SYSTEM The innate immune system represents the first-line response to immunologic challenges and is composed of cellular defenses (neutrophils, dendritic cells, macrophages, and natural killer…
