Sjögren's syndrome: review with recent insights into immunopathogenesis.
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Biomedical subjects
Publications and source records attributed to A Montanaro.
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A prospective study was performed in 40 chronic uremics which included: (1) the intramuscular administration to all patients of 40 micrograms of a DNA-recombinant vaccine (Engerix-B) at 0, 1, 2, 6 months; (2) an intramuscular booster dose of 40 micrograms at 18 months in patients having an anti-HBs titer greater than 100 mIU/ml at the 7th month (group A); (3) a further intramuscular supplementary dose of 40 micrograms at 12 months (besides that at 18 months) in patients developing an antibody titer less than 100 mIU/ml at the 7th months (group B); (4) an intradermal course of 5 micrograms of vaccine every 2 weeks until the protective titer (greater than or equal to 10 mIU/ml) was achieved, and then every month for a total of 6 months in patients who did not develop a protective titer even after 19 months (group C). At the end of the study, all patients had developed a protective titer: 77.5% after the 4th intramuscular dose, 12.5% after the 5th and 10% after 3.5 +/- 0.5 (mean +/- SEM) intradermal inoculations. The mean antibody titers were 1,461 +/- 98 mIU/ml in group A, 594 +/- 684 in group B and 131 +/- 133 in group C. In conclusion, our two-step integrated protocol gives an anti-HBs protective titer in all our patients.
The aim of this study was to examine the distribution and types of adhesion molecules expressed over endothelial cells and the ligands present on lymphocytes which infiltrate exocrine glands in patients with Sjogren's syndrome. Minor salivary gland biopsies were examined from twelve patients with Sjogren's syndrome and eight normal subjects for the presence of adhesion molecules using monoclonal antibodies and an Indirect Immunoperoxidase technique. There was an increased expression of intercellular adhesion molecule-1 (ICAM-1, CD54) on endothelial cells, lymphocytes, fibroblasts and salivary gland epithelial cells. In addition we documented the expression of endothelial leukocyte adhesion molecule-1 (ELAM-1) on endothelial cells in salivary glands from patients but not the controls. Many of the endothelial cells expressing these adhesion molecules in patients with Sjogren's syndrome had the morphological appearance of high endothelial venules. V-CAM-1 was shown to be present in some of the salivary biopsies from patients with Sjogren's syndrome. Lymphocytes infiltrating salivary glands strongly express LFA-1 (CD11a/CD18) molecules. Some infiltrating lymphocytes, and most monocytes, expressed C3bi-R (CD11b/CD18) and the p150.95 (CD11c/CD18) antigens on their cell surface. The results of this study reveal the enhanced expression of vascular endothelial and lymphocyte adhesion molecules on the minor salivary glands of patients with Sjogren's syndrome. The presence of such receptors and their putative ligands indicate an important role for these molecules in the pathogenesis of Sjogren's syndrome.
The effects of dietary manipulations on autoimmune disease are understood poorly. In this article, we detail our experience with a human subject who developed autoimmune hemolytic anemia while participating in a research study that required the ingestion of alfalfa seeds. Subsequent experimental studies in primates ingesting alfalfa sprout seeds and L-canavanine (a prominent amino acid constituent of alfalfa) is presented. The results of these studies indicate a potential toxic and immunoregulatory role of L-canavanine in the induction of a systemic lupus-like disease in primates.
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Formaldehyde is truly ubiquitous in our ecology and continuing important commercial applications. Most of us have daily contact with this chemical. The most significant outdoor source of this chemical is gasoline and diesel fuel combustion. The primary indoor source is combustion of tobacco products. Formaldehyde is associated with a disagreeable odor that can produce "annoyance" symptoms and at higher concentrations can be a transient and completely reversible irritant to the eyes and mucous membranes of the respiratory tract. It is so soluble and rapidly metabolized that it rarely reaches the lower respiratory tract to inflict damage. The exception is in cigarette smokers who actively inhale. Formaldehyde may on rare occasions induce bronchial asthma at relatively high exposure doses. There are no conclusive studies that prove the development of de novo IgE-mediated respiratory tract symptoms secondary to inhalation of formaldehyde vapors. The approach to formaldehyde-induced symptoms should be one of careful documentation of objective physiologic changes.
A young, previously healthy woman presented with increasing muscle pain, lower limb swelling, fatigue and eosinophilia. She had consumed L-tryptophan tablets (one to two at night) over the preceding five months for management of her insomnia. Her condition slowly deteriorated and she developed generalised oedema and severe lethargy. A white blood cell count was 21.3 x 10(9)/L with 43% eosinophils (Normal range: 4.0-11.0 x 10(9)/L with 1-6% eosinophils. A biopsy specimen of the deep fascia and gastrocnemius muscle demonstrated fasciitis and myositis. The patient failed to recover after cessation of L-tryptophan use but her condition improved rapidly without significant sequelae after systemic treatment with corticosteroids.
Sjogren's syndrome is an autoimmune condition with extraordinary and unique involvement in the eyes and respiratory tract. These patients frequently present or are referred to an allergist for evaluation. Recognition of the syndrome is critical for effective management.
Enteric protein loss resulting in profound hypoalbuminemia and anasarca is an uncommon manifestation of systemic lupus erythematosus and only rarely is the initial presentation of disease. A few patients with SLE and protein-losing enteropathy in the absence of increased central venous pressure or intestinal lymphangiectasia have been reported. We describe the utility alpha-1-antitrypsin clearance in stool for diagnosing and monitoring enteric protein loss during successful immunosuppressive drug therapy in a patient who presented with massive enteric protein loss as the initial manifestation of systemic lupus erythematosus.
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Kaposi's sarcoma and disseminated sporotrichosis of the skin and joints developed simultaneously in a homosexual man with antibodies to human immunodeficiency virus. There was no identified source of exposure to Sporothrix organisms. Sporotrichosis may be a presenting opportunistic infection associated with acquired immunodeficiency syndrome and tends to be disseminated at the time of diagnosis.
Vasculitis is commonly considered in the differential diagnosis of systemic disease in the elderly. Because the spectrum of vasculitis is so variable, it is useful to utilize a classification system that limits the diagnostic options. Classification systems based on potential pathologic mechanisms or specific histologic findings are of limited usefulness in the initial assessment of an elderly patient. A classification system based on the clinical features exhibited will be discussed in the context of vasculitic syndromes which may present in the elderly. While this approach is by no means comprehensive, it should allow the correct diagnosis of vasculitis in the majority of cases. Common and unique presentations of vasculitis in the elderly will also be discussed.
The allergist-immunologist is ideally trained to play a critical role in the recognition and evaluation of patients with building-related illness. The practitioners of this subspecialty should also take an active role in critical studies that will expand the knowledge base in this whole area, and collaborate as well with colleagues in toxicology and industrial hygiene to develop accurate and sensitive measures of implicated pollutants, irritants, and allergens. The role of multiple low level pollutants on healthy workers must be assessed in an objective fashion. Methods to evaluate and enforce regulations on building ventilation should be developed and widely applied. It is only in this fashion that an expanding population of office workers can be assured a suitable work environment as we emerge from the age of the industrial blue collar worker to the technical, office-based white collar worker.
Intravenous immunoglobulin can be a very effective form of treatment for patients with primary immunodeficiency states. Recommendations for intravenous dosing previously have been empirically derived. In order to determine the potential prolongation of intervals between infusions following the administration of 500 mg/kg of intravenous immunoglobulin, 11 patients were studied. This high-dose therapy was well tolerated and resulted in a modest prolongation of therapeutic IgG levels when compared with lower-dose 150 mg/kg regimens. Significant variability among individual patients was observed. Implications of this high-dose therapy are discussed.
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After operations for prolapsed lumbar disc, the authors recommend early resumption of the erect posture combined with a programme of re-education. This is started during the first days after the operation and continued for three months. In most cases it assists the rapid recovery of spinal function. The best results are obtained in patients in whom the operation completely relieves the nerve root symptoms. The programme of re-education, begun early, presents no problems if excessive strain on the lumbar spine is avoided during the first seven to nine days after operation.
A 61-year-old woman with malignant lymphoma, histiocytic type, with slerosis (sclerosing reticulum cell sarcoma) primary in the spleen is described. She has survived clinically free of malignant lymphoma for 11 years after splenectomy therapy alone. This may be the first case of primary splenic malignant lymphoma, histiocytic type, with sclerosis, ever reported in the medical literature.