Cutaneous Lupus Band Test
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…
Renal Lesions in Systemic Lupus Erythematosus
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…
Key Features of Primary Vasculitic Diseases
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….
Vasculitis: Clinical and Histologic Features of Granulomatosis with Polyangitis (Wegener)
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…
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…
