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…
Pathophysiology. The cause of giant cell arteritis is unknown. The dominant force in vascular injury is cellmediated immunity. Resident “sentinel” vascular dendritic cells are thought to present antigen. Migrating dendritic…
Pathoetiology. The prevalence and severity of fibromyalgia are determined by impaired slow-wave sleep, the magnitude of mood disturbance, and gene polymorphisms. These factors influence the brain and dorsal horns of…
POLYMYALGIA RHEUMATICA Polymyalgia rheumatica is characterized by proximal symmetric limb girdle pain and stiffness that may be accompanied by systemic inflammatory features. The disease responds in dramatic fashion to low-dose…
The mechanism of CPPD deposition in articular cartilage is not yet fully understood but may, in part, relate to genetically determined activity of phosphate transporters. Serum calcium, phosphate, and alkaline…
