Widespread Vasculitis Research Articles

Kawasaki syndrome (the mucocutaneous lymph node syndrome).

Kawasaki syndrome is a newly-recognized clinical entity characterized by multisystem involvement. It has an acute onset and triphasic clinical course. Although essentially a self-limiting disease, permanent vascular damage, especially involving the coronary arteries, may result. Pathologically, the disease is characterized by widespread vasculitis. There is a monomodal age distribution, with peak occurrence during the first two years of life and few affected over the age of 8 years. Males outnumber females 1.5:1; persons of Japanese extraction are overrepresented compared with other races, caucasians underrepresented. Community-wide epidemics have been reported from diverse locations but there is no evidence for direct person-to-person transmission. Etiology remains unknown. Therapy is supportive and should be directed at careful clinical evaluation for cardiovascular abnormalities and antiplatelet aggregation therapy.

Kawasaki syndrome (the mucocutaneous lymph node syndrome).

Kawasaki Syndrome is a newly recognized clinical entity characterized by multisystem involvement. It has an acute onset and a triphasic clinical course. Although essentially a self-limited disease, permanent vascular damage, especially involving the coronary arteries, may result. Pathologically the disease is characterized by widespread vasculitis. There is a monomodal age distribution with peak occurrence during the first 2 years of life; few affected over the age of 8 years. Males outnumber females 1.5:1, persons of Japanese extraction are overrepresented compared with other races, and Caucasians are underrepresented. Community-wide epidemics occur in diverse locations but there is no evidence for direct person-to-person transmission. Etiology remains unknown. Therapy remains supportive and should be directed at careful clinical evaluation for cardiovascular abnormalities and antiplatelet aggregation therapy.

Roentgenographic abnormalities in Rocky Mountain Spotted Fever.

Rock Mountain spotted fever (RMSF) is a tick-borne rickettsial disease which produces a widespread vasculitis. A mortality of 7% to 13% has been reported in the United States which is due at least in part to delay in diagnosis and appropriate treatment. The classic features of this disease include a history of tick bite with the clinical presentation of skin rash and fever in association with thrombocytopenia. Few reports have emphasized the radiologic chest abnormalities in this disease or their relationship to thrombocytopenia. We review 70 cases of RMSF with abnormal roentgenographic features and their pathologic correlation.

Return of Renal Function After Renal Insufficiency With Cyclophosphamide Therapy in Wegener's Granulomatosis

To the Editor. —Wegener's granulomatosis, first described in 1931 by Klinger 1 and further defined by Wegener in 1936, 2 is a well-delineated clinicopathologic entity involving necrotizing granulomatous vasculitis of the upper and lower respiratory system, glomerulonephritis, and variable degrees of widespread small-vessel vasculitis. Despite the impressive therapeutic results achieved with cyclophosphamide in this syndrome by Wolff et al, 3 to our knowledge only one patient has been described to recover substantial long-term renal function after renal failure has supervened. 4 We describe a patient with such a diagnosis initially seen with rapidly progressive renal insufficiency necessitating dialysis, treated early and aggressively, resulting in remarkable recovery of renal function. Such an outcome illustrates the importance of early intervention. Report of a Case. —A 70-year-old woman had normal findings from physical examination, normal chest roentgenogram, and a creatinine value of 1.0 mg/dL in September 1978. In January 1979, malaise, fatigue, decreased

Lymphosarcoma of the mandible associated with macroglobulinaemia of Waldenström : SHTEYER A. and MARKITGIU A. Int J. Oral Surg.7 (1978) 585–589

<dm:abstracts xmlns:dm="http://www.elsevier.com/xml/dm/dtd"><ce:abstract xmlns:ce="http://www.elsevier.com/xml/common/dtd" view="all" class="author" id="aep-abstract-id7"><ce:section-title>Publisher Summary</ce:section-title><ce:abstract-sec view="all" id="aep-abstract-sec-id8"><ce:simple-para id="fsabs002" view="all">Cryoglobulinemia is defined as the presence of circulating immunoglobulins (I<ce:inf loc="post">g</ce:inf>) that precipitate at temperatures &lt; 37°C and re-dissolve on re-worming. Such an in vitro phenomenon is detectable in a wide number of chronic infectious and immunological disorders, as well as in some hematological malignancies. This chapter discusses Mixed Cryoglobulinemia (MC) that refers to a distinct clinical syndrome in the absence of other well known systemic or neoplastic disorders. Clinically, MC is characterized by a typical triad—purpura, weakness, arthralgias—and by multisystem organ involvement including chronic hepatitis, membranoproliferative glomeruonephritis (MPGN), peripheral neuropathy, skin ulcers, widespread vasculitis, and less frequently lymphatic and hepatic malignancies. The treatment of MC syndrome is particularly challenging because of its complex etiopathogenesis. For a correct therapeutic approach to hepatitis C virus (HCV)-related MC, one must deal with the concomitance of conflicting conditions: HCV infection, autoimmune, and lymphoproliferative alterations. While asymptomatic patients usually do not need any treatment even in the presence of high levels of cryocrit, patients with mildmoderate symptoms—such as palpable purpura—are particularly sensitive to the smallest variations of daily steroid dosage (1-2 mg).</ce:simple-para></ce:abstract-sec></ce:abstract></dm:abstracts>

Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Thirteen cases of acute posterior multifocal placoid pigment epitheliopathy occurred in young adults or older children. In ten there was a characteristic, possibly infectious syndrome that preceded the onset of ocular manifestations by a few weeks. One patient developed cerebrospinal fluid lymphocytosis and a cerebral vasculitis coincident with the ocular changes. Extensive evaluations for viral and other infectious etiologies in this and other cases were unrevealing. An inflammatory reaction was observed in the anterior chambers of most patients, and cells were noted in the anterior vitreous bodies of a few patients. In one instance a serous detachment of the retina occurred adjacent to the optic disk. Our findings and others support the conclusion that the fundus lesions resulted from a multifocal choroiditis primarily involving the choriocapillaris and affecting the pigment epithelium and retina secondarily. The data also suggested that the choroiditis may be associated with a more widespread vasculitis occurring as an immune reaction to an earlier systemic infection.

Lymphocytic choriomeningitis virus infection in fetal, newborn, and young adult Syrian hamsters (Mesocricetus auratus).

The pathogenesis of lymphocytic choriomeningitis virus infection in fetal, newborn, and young adult hamsters was studied. Infected newborn hamsters initially developed a persistent viremia and viruria with titers often in excess of 10(4.0) mean infectious doses/0.03 ml of blood or urine. After week 12 two different patterns of infection became evident. Approximately one-half of the hamsters eventually cleared the infection, whereas the others developed a chronic progressive and ultimalely fatal disease characterized by continuous high-titered viremia and viruria and high titers of virus in their tissues. Complement-fixing antibody and, to a lesser degree, virus-neutralizing antibody coexisted with the viremia. Hamsters with persistently high levels of viremia and viruria developed chronic glomerulonephritis and widespread vasculitis, whereas hamsters that cleared their infections did not develop these lesions. Litters of hamsters born to viremic mothers were invariably infected. Litter sizes were small and breeding effectiveness was reduce; however, vertical, congenital infection was successfully passed through three generations. The course of infection in the congenitally infected hamsters was similar to that in newborn infected hamsters, with all animals producing complement-fixing antibody, some animals being capable of clearing the viremia and remaining healthy, and other animals having persistent viremia and fatal disease. Inoculated young adult hamsters did not become diseased, developed viremia and viruria which persisted up to 3 and 6 months, respectively, and developed complement-fixing antibody by 10 days after infection. The prolonged urinary excretion of large amounts of lymphocytic choriomeningitis virus by asymptomatic, chronically infected hamsters is an important public health consideration when dealing with potential human infection.

Neuropathological effects of persistent infection of mice by mouse hepatitis virus

Mouse hepatitis virus (MHV3) can persist for months in strains of mice with genetically controlled "semisusceptibility" to this virus. The pathology of the chronic neurological disease induced in these animals has been investigated by conventional histology and immunofluorescence. A2G mice develop a chronic choroidoependymitis and meningitis leading to severe hydrocephalus and hydromyelia. In C3H mice a widespread vasculitis was observed, with both viral antigens and bound immunoglobulins in vessal walls. No significant glomerulonephritis was found. Systemic amyloidosis was present in the spleen, liver, and kidneys. The virus was not detected in neural tissues, but brain and spinal cord lesions were found near inflammatory areas surrounding damaged vessels. It is suggested that viral persistance in ependymal cells is directly responsible for the lesions in A2G mice, whereas an immunopathological lesion of blood vessels of the central nervous system underlines the damage to mice of the C3H strain.

Breast involvement in Wegener's granulomatosis.

Two cases of Wegener's granulomatosis are described in which the early and predominating lesions were found in the breast. The histological features of breast necrosis with granulomatous inflammation and widespread vasculitis in the breast in the absence of any demonstrable pathogens enabled a presumptive diagnosis to be made in one case before the development of the more characteristic clinical features in the respiratory system.

Arteritis, Myocardial Infarction, and Rheumatoid Arthritis

During a four-year period, diffuse vasculitis developed in six of 102 patients with rheumatoid arthritis. Two of these six patients also sustained myocardial infarctions. Coronary arteritis was found at autopsy in one patient; it was also suspected as the cause of the infarction in the patient who survived. The autopsy protocols of an additional 95 rheumatoid patients were examined. For seven patients with myocardial infarctions, no examples of coronary arteritis were found in the available histological sections. The present study does not support the hypothesis that coronary arteritis is a common cause of myocardial infarction in rheumatoid arthritics. However, occasionally, in the presence of widespread vasculitis, coronary arteritis may lead to myocardial infarction.