PULMONARY MANIFESTATIONS OF SJGREN'S SYNDROME
Identifieur interne : 002739 ( Main/Curation ); précédent : 002738; suivant : 002740PULMONARY MANIFESTATIONS OF SJGREN'S SYNDROME
Auteurs : Hilary C. Cain [États-Unis] ; Paul W. Noble [États-Unis] ; Richard A. Matthay [États-Unis]Source :
- Clinics in Chest Medicine [ 0272-5231 ] ; 1998.
English descriptors
- Teeft :
- Abnormal, Abnormal findings, Abnormal hrct, Abnormal test, Abnormality, Airway, Airway disease, Airways disease, Alveolitis, American review, Antinuclear antibody, Arthritis, Arthritis rheum, Bengal staining, Biopsy, Bronchial hyperreactivity, Bronchiolitis, Bronchiolitis obliterans, Bronchoalveolar lavage, Chest radiograph, Chest radiographic evidence, Clin, Clinical significance, Connective tissue disease, Consecutive patients, Cystic lung disease, Dessication, Diagnostic criteria, Diego criteria, Disease progression, Extraglandular, Extraglandular disease, Fluid lymphocytosis, Fluid neutrophilia, Follicular bronchiolitis, Function tests, Greater decline, High prevalence, High rates, Hrct, Immune complexes, Immune dysregulation, Immunodeficiency syndrome, Interstitial, Interstitial disease, Interstitial lung disease, Keratoconjunctivitis sicca, Lacrimal glands, Longitudinal study, Lung biopsy, Lung disease, Lung function, Lupus, Lymph nodes, Lymphocyte, Lymphocyte infiltration, Lymphocytic, Lymphocytic alveolitis, Lymphoid, Lymphoma, Malignancy, Malignant, Malignant lymphoma, Malignant transformation, Manifestation, Monoclonal antibodies, Moutsopoulos, Mucociliary clearance, Multiple bullae, Objective signs, Obstruction, Obstructive, Obstructive airways disease, Obstructive lung disease, Ocular symptoms, Other causes, Physiologic evidence, Pleural effusion, Primary disease, Primary syndrome, Proinflammatory cytokines, Pseudolymphoma, Pulmonary disease, Pulmonary evaluation, Pulmonary fibrosis, Pulmonary function, Pulmonary function testing, Pulmonary lymphoma, Pulmonary manifestations, Recent study, Respiratory diseases, Respiratory involvement, Respiratory manifestations, Respiratory symptoms, Rheum, Rheumatoid, Rheumatoid arthritis, Rheumatoid factor, Rheumatol, Salivary, Salivary flow rate, Salivary gland biopsy, Salivary glands, Secondary disease, Secondary syndrome, Serologic evidence, Sicca, Sicca symptoms, Sicca syndrome, Small airways, Subclinical, Symptom, Syndrome, Systemic, Systemic lupus erythematosus, Tracheobronchial dessication, Vital capacity.
Abstract
Sjgren's syndrome, a chronic autoimmune inflammatory disease, is one of the most common systemic rheumatic diseases, affecting 0.1 of the general population and 3 of older adults32; women are more commonly affected than men (9:1, female:male). Affected patients typically present in the fourth or fifth decade of life, the common presenting symptoms being the classic triad of: dry mouth (xerostomia), dry eyes (keratoconjunctivitis sicca or xerophthalmia), and arthritis.72 Heinrich Sjgren, whose name the syndrome bears, was not the first to describe this condition, but he recognized that the disease was a systemic disorder and described the pathology of the xerophthalmia, thereby introducing the term keratoconjunctivitis sicca. Sjgren's syndrome is now known to be an autoimmune exocrinopathy67 and autoimmune epithelitis.50 The syndrome is characterized pathologically by lymphoproliferation and lymphocyte infiltration of glandular and nonglandular tissue. In most patients, lymphocyte infiltration is confined to the salivary and lacrimal glands, but extraglandular infiltration occurs in 5 to 10 of affected patients.72 Lymphocyte infiltration has been identified in the lungs, pancreas, gastrointestinal tract, hepatobiliary system, kidneys, skin, and bone marrow.44 Lymphoproliferation in Sjgren's syndrome is usually benign, but malignant transformation occurs in some cases. Typically, the cellular infiltrate is predominantly CD4-positive T cells1 and also may contain B lymphocytes, plasma cells, and large reticulum cells.72 The lymphocytes may form nonmalignant, tumor-like aggregates, a condition termed pseudolymphoma. If the infiltrative process is frankly malignant it is typically a non-Hodgkin's lymphoma or less frequently Waldenstrm's macroglobulinemia. Sjgren's-associated lymphoma may arise years after the diagnosis of Sjgren's syndrome. Therefore, a pathologic diagnosis of the cellular infiltrate should be obtained at the time of disease onset and if there is evidence of disease progression.
Url:
DOI: 10.1016/S0272-5231(05)70110-6
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<profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Abnormal</term>
<term>Abnormal findings</term>
<term>Abnormal hrct</term>
<term>Abnormal test</term>
<term>Abnormality</term>
<term>Airway</term>
<term>Airway disease</term>
<term>Airways disease</term>
<term>Alveolitis</term>
<term>American review</term>
<term>Antinuclear antibody</term>
<term>Arthritis</term>
<term>Arthritis rheum</term>
<term>Bengal staining</term>
<term>Biopsy</term>
<term>Bronchial hyperreactivity</term>
<term>Bronchiolitis</term>
<term>Bronchiolitis obliterans</term>
<term>Bronchoalveolar lavage</term>
<term>Chest radiograph</term>
<term>Chest radiographic evidence</term>
<term>Clin</term>
<term>Clinical significance</term>
<term>Connective tissue disease</term>
<term>Consecutive patients</term>
<term>Cystic lung disease</term>
<term>Dessication</term>
<term>Diagnostic criteria</term>
<term>Diego criteria</term>
<term>Disease progression</term>
<term>Extraglandular</term>
<term>Extraglandular disease</term>
<term>Fluid lymphocytosis</term>
<term>Fluid neutrophilia</term>
<term>Follicular bronchiolitis</term>
<term>Function tests</term>
<term>Greater decline</term>
<term>High prevalence</term>
<term>High rates</term>
<term>Hrct</term>
<term>Immune complexes</term>
<term>Immune dysregulation</term>
<term>Immunodeficiency syndrome</term>
<term>Interstitial</term>
<term>Interstitial disease</term>
<term>Interstitial lung disease</term>
<term>Keratoconjunctivitis sicca</term>
<term>Lacrimal glands</term>
<term>Longitudinal study</term>
<term>Lung biopsy</term>
<term>Lung disease</term>
<term>Lung function</term>
<term>Lupus</term>
<term>Lymph nodes</term>
<term>Lymphocyte</term>
<term>Lymphocyte infiltration</term>
<term>Lymphocytic</term>
<term>Lymphocytic alveolitis</term>
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<term>Lymphoma</term>
<term>Malignancy</term>
<term>Malignant</term>
<term>Malignant lymphoma</term>
<term>Malignant transformation</term>
<term>Manifestation</term>
<term>Monoclonal antibodies</term>
<term>Moutsopoulos</term>
<term>Mucociliary clearance</term>
<term>Multiple bullae</term>
<term>Objective signs</term>
<term>Obstruction</term>
<term>Obstructive</term>
<term>Obstructive airways disease</term>
<term>Obstructive lung disease</term>
<term>Ocular symptoms</term>
<term>Other causes</term>
<term>Physiologic evidence</term>
<term>Pleural effusion</term>
<term>Primary disease</term>
<term>Primary syndrome</term>
<term>Proinflammatory cytokines</term>
<term>Pseudolymphoma</term>
<term>Pulmonary disease</term>
<term>Pulmonary evaluation</term>
<term>Pulmonary fibrosis</term>
<term>Pulmonary function</term>
<term>Pulmonary function testing</term>
<term>Pulmonary lymphoma</term>
<term>Pulmonary manifestations</term>
<term>Recent study</term>
<term>Respiratory diseases</term>
<term>Respiratory involvement</term>
<term>Respiratory manifestations</term>
<term>Respiratory symptoms</term>
<term>Rheum</term>
<term>Rheumatoid</term>
<term>Rheumatoid arthritis</term>
<term>Rheumatoid factor</term>
<term>Rheumatol</term>
<term>Salivary</term>
<term>Salivary flow rate</term>
<term>Salivary gland biopsy</term>
<term>Salivary glands</term>
<term>Secondary disease</term>
<term>Secondary syndrome</term>
<term>Serologic evidence</term>
<term>Sicca</term>
<term>Sicca symptoms</term>
<term>Sicca syndrome</term>
<term>Small airways</term>
<term>Subclinical</term>
<term>Symptom</term>
<term>Syndrome</term>
<term>Systemic</term>
<term>Systemic lupus erythematosus</term>
<term>Tracheobronchial dessication</term>
<term>Vital capacity</term>
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<front><div type="abstract">Sjgren's syndrome, a chronic autoimmune inflammatory disease, is one of the most common systemic rheumatic diseases, affecting 0.1 of the general population and 3 of older adults32; women are more commonly affected than men (9:1, female:male). Affected patients typically present in the fourth or fifth decade of life, the common presenting symptoms being the classic triad of: dry mouth (xerostomia), dry eyes (keratoconjunctivitis sicca or xerophthalmia), and arthritis.72 Heinrich Sjgren, whose name the syndrome bears, was not the first to describe this condition, but he recognized that the disease was a systemic disorder and described the pathology of the xerophthalmia, thereby introducing the term keratoconjunctivitis sicca. Sjgren's syndrome is now known to be an autoimmune exocrinopathy67 and autoimmune epithelitis.50 The syndrome is characterized pathologically by lymphoproliferation and lymphocyte infiltration of glandular and nonglandular tissue. In most patients, lymphocyte infiltration is confined to the salivary and lacrimal glands, but extraglandular infiltration occurs in 5 to 10 of affected patients.72 Lymphocyte infiltration has been identified in the lungs, pancreas, gastrointestinal tract, hepatobiliary system, kidneys, skin, and bone marrow.44 Lymphoproliferation in Sjgren's syndrome is usually benign, but malignant transformation occurs in some cases. Typically, the cellular infiltrate is predominantly CD4-positive T cells1 and also may contain B lymphocytes, plasma cells, and large reticulum cells.72 The lymphocytes may form nonmalignant, tumor-like aggregates, a condition termed pseudolymphoma. If the infiltrative process is frankly malignant it is typically a non-Hodgkin's lymphoma or less frequently Waldenstrm's macroglobulinemia. Sjgren's-associated lymphoma may arise years after the diagnosis of Sjgren's syndrome. Therefore, a pathologic diagnosis of the cellular infiltrate should be obtained at the time of disease onset and if there is evidence of disease progression.</div>
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