Vasculitis: Vessel Distribution
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,…
Joint and Central Nervous System Findings in Autoinflammatory Syndromes
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…
Cutaneous Findings in Autoinflammatory Syndromes
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…
Pathophysiology of Autoinflammatory Syndromes
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…
Imaging of Polymyalgia Rheumatica and Giant Cell Arteritis
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…
Fibromyalgia
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…
Clinical Manifestations of Polymyalgia Rheumatica and Giant Cell Arteritis
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…
Articular Chondrocalcinosis (Pseudogout)
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…
Tophaceous Gout
SECONDARY GOUT Hyperuricemia and gout may be a consequence of the overproduction of uric acid caused by an increased turnover of nucleic acids in myeloproliferative disorders, sickle cell anemia and…
Nonarticular Rheumatism
The nonarticular pain syndromes have been demonstrated to have definite associations with a group of conditions including nonrestorative sleep, irritable bowel syndrome, chronic fatigue, various mood disorders, chronic and migrainous…
