Detection of autoantibodies against myeloid lysosomal enzymes: a useful adjunct to classification of patients with biopsy-proven necrotizing arteritis
Article Abstract:
Vasculitis, or inflammation of lymph and blood vessels, may be a major or minor symptom of disease. Various forms of vasculitis are classified by clinical syndromes or the size of the affected blood vessels. However, some forms of vasculitis that are idiopathic (have no known cause) and involve medium- and/or small-sized arteries are difficult to classify. They include polyarteritis nodosa (PAN), Churg-Strauss syndrome, and Wegener's granulomatosis (WG). Autoantibodies, abnormal immune proteins directed against the body's own tissues, have been detected in idiopathic forms of vasculitis. These autoantibodies are directed against the lysosomal enzymes of certain bone marrow cells, such as neutrophils. The lysosomal enzymes are contained within a storage compartment of the cell and function to degrade proteins and carbohydrates. The autoantibodies are called antineutrophil cytoplasmic antibodies (ANCA) and are directed against the lysosomal enzymes protease and myeloperoxidase (MPO) of the neutrophil, a type of white blood cell. Twenty-eight patients with idiopathic vasculitis involving medium- and small-sized arteries were assessed for the presence of ANCA. In general, ANCA directed against protease were detected in patients with WG, and ANCA directed against MPO were present in patients with Churg-Strauss syndrome and systemic PAN. No ANCA were detected in patients with PAN limited to the skin and skeletal muscle. These findings show that identification of ANCA may help to classify idiopathic forms of vasculitis affecting small- to medium-sized blood vessels. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: American Journal of Medicine
Subject: Health care industry
ISSN: 0002-9343
Year: 1991
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Hematologic manifestations of systemic mast cell disease: a prospective study of laboratory and morphologic features and their relation to prognosis
Article Abstract:
Systemic mast cell disease (SMCD) is a disorder in which mast cells (connective tissue cells containing histamine, glycosaminoglycans, and other factors) accumulate in organs. In most cases, the disease causes little alteration in patients' lives, but some patients decline rapidly and die. This report presents data from a prospective study conducted during the past 13 years at the National Institutes of Health. Forty-six patients (average age at diagnosis, 39 years) were involved in the study. Thirty-two patients with SMCD and 14 patients with cutaneous mast cell disease (CMCD) had bone marrow examinations, and all but one had bone marrow biopsies. (CMCD involves mast cell infiltration into the skin and supporting tissues.) An extensive description is presented of the histopathological findings. SMCD, diagnosed when mast cells have infiltrated the bone marrow, was associated with a poorer prognosis than CMCD; 10 of the SMCD patients died between one and 22 years after diagnosis, compared with no deaths in the CMCD group. Factors associated with a poor prognosis are described, including low levels of platelets, anemia, abnormalities in peripheral blood smears, and elevated alkaline phosphatase. The criteria that can be used to differentiate patients with SMCD and related blood disorders are also described. Patients who were older when symptoms first appeared and who had elevated levels of lactate dehydrogenase in their blood were at greater risk of death. Other factors associated with increased risk of illness and death are presented. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: American Journal of Medicine
Subject: Health care industry
ISSN: 0002-9343
Year: 1991
User Contributions:
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