Scleroderma—Clinical Findings
Pulmonary hypertension, characterized by rapidly progressive dyspnea, occurs in 7% to 12% of patients typically 10 to 15 years after onset of Raynaud phenomenon. The diffusing capacity is disproportionally reduced…
Scleroderma—Clinical Manifestations
Scleroderma is categorized into localized and systemic varieties. Localized scleroderma consists of morphea and linear scleroderma, which is manifest as sclerotic lesions of the skin without visceral involvement. Systemic sclerosis,…
Antiphospholipid Syndrome
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…
Lupus Erythematosus of the Heart
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…
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…
