Cases reported "Pulmonary Fibrosis"

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1/33. Detection of anti-ADAM 10 antibody in serum of a patient with pulmonary fibrosis associated with dermatomyositis.

    OBJECTIVES: It has been suggested that the humoral immune system plays a part in the pathogenesis of pulmonary fibrosis. Although circulating autoantibodies to lung protein(s) have been suggested, few lung proteins have been characterised. The purpose of this study is to determine the antigen recognised by serum of a patient with pulmonary fibrosis associated with dermatomyositis. methods: To accomplish this, anti-small airway epithelial cell (SAEC) antibody in a patient's serum was evaluated using a western immunoblot. RESULTS: An autoantibody against SAEC was found, and the antigen had a molecular weight of 62 kDa. Using the patient's serum, clones from the normal lung cDNA library were screened and demonstrated that anti-SAEC antibody in the patient's serum was against ADAM (A disintegrin and metalloprotease) 10. CONCLUSION: This is the first report that demonstrates the existence of anti-ADAM 10 antibody in a patient with pulmonary fibrosis associated with dermatomyositis.
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2/33. Interstital lung disease due to contamination of forced air systems.

    Eight patients had hypersensitivity pneumonitis due to contaminated home or office forced-air heating or air-conditioning systems. We studied their clinical and laboratory features, and the results indicated that this disease may occur as an acute or insidious form differing in type and intensity of respiratory and systemic symptoms. Thermophilic actinomycetes contaminatinf the forced air systems were identified as the sensitizing agents in most cases. Precipitating antibodies to the organisms could be shown in the serums of the patients and the antigen identified by immunofluorescent studies in the three lung biopsies examined by this method.inhalation challenge studies with the cultured organism or other materials obtained from the forced air systems reproduced the clinical syndrome in the four patients tested. Avoidance of the contaminated system, and the use of corticosteroids in more severe cases,seems to be appropriate therapy for patients with this disease.
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3/33. hypersensitivity pneumonia-nonspecific interstitial pneumonia/fibrosis histopathologic presentation: a study in diagnosis and long-term management.

    BACKGROUND: Nonspecific interstitial pneumonia/fibrosis (NSIP) has been classified a form of idiopathic interstitial pneumonia/fibrosis. We have shown that cases of NSIP without demonstrable serum precipitins may be caused by inhalation of high levels of mold and/or bacteria in closed environments. OBJECTIVE: We report a patient with a clinical and histopathologic diagnosis of NSIP without serum precipitins caused by a microbial contamination in her home. Her case was converted from an acute to an insidious clinical presentation by inadequate remediation. A prolonged avoidance-challenge technique demonstrated that this case of NSIP was a form of hypersensitivity pneumonia that was reversible by effective remediation. methods: The patient was identified by compatible signs and symptoms, roentgenographic studies, pulmonary function tests, and a transbronchial lung biopsy. She was further evaluated with a detailed environmental history, serologic tests, and investigation of the home environment. An environmental avoidance and challenge technique was performed to confirm cause and effect and to determine that remediation had been effective. RESULTS: review of the biopsy showed NSIP and failed to reveal any non-caseating granuloma formation. Investigation of the home revealed a cladosporium species contamination of the air conditioning system and penicillium species beneath an entryway carpet. serum precipitins to commercial antigens of common mold to the south texas area were negative. Avoidance and challenge techniques confirmed the home as the causative environment in this case of NSIP. The patient has been free of signs and symptoms and has taken no medication for interstitial lung disease over the past 30 months. CONCLUSIONS: Some cases of NSIP may be caused by inhalation of microbial antigen(s) in a closed environment. An environmental challenge technique was an effective method to determine the causative environment and confirm that remediation had been effective. Inadequate remediation may lead to symptomatic improvement, but may convert a patient from an acute to an insidious presenter. The environmental challenge obviates a need for specific challenges to determine specific causation. Remediation of or moving from an environmental contamination to achieve reversibility or prevent progression was the treatment of choice to avoid use of long-term immunosuppressive agents.
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4/33. Lymphoid interstitial pneumonia.

    A 19-year-old man presented with dyspnea, cough and chest pains; he also complained of nausea, anorexia and postprandial vomiting and reported a 10-kg weight loss. Generalized lymphadenopathy and some rales over both lung bases were noted and a chest radiograph showed bilateral nodular lesions. Persistent leukocytosis, thrombocytosis, proteinuria and anergy to a series of natural antigens were found. The diagnosis of lymphoid interstitial pneumonia was made from material obtained at open lung biopsy. Rapid but incomplete clearing of the lung lesions resulted from steroid therapy; the other abnormalities were corrected gradually, except for the proteinuria, which persisted. The clinical improvement and the ability to work and play have been maintained for the past 20 months.
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5/33. Monitoring of serum KL-6 antigen in a patient with radiation pneumonia.

    serum marker KL-6 antigen has been reported to be a valuable indicator of the disease activity of interstitial pneumonia. It is not clear how sensitive the serum KL-6 antigen level is in reflecting histologic changes in lung tissues. We report here the results of serial measurements of serum KL-6 antigen in a 76-year-old male patient with radiation pneumonia. serum KL-6 antigen levels were more sensitive than lactate dehydrogenase and procollagen type III N-terminal peptide. The level of serum KL-6 antigen appears to reflect the histologic changes of the lung more sensitively than does c-reactive protein.
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6/33. autoantibodies and the spectrum of sjogren's syndrome.

    In studies reported recently, the sera of patients with sjogren's syndrome were found to contain precipitating antibodies to nuclear antigens that can be identified by immunodiffusion analysis. These precipitating autoantibodies have been termed SS-A and SS-B antibodies. We show that identification of these autoantibodies helped in establishing the diagnosis of sjogren's syndrome in 12 of 30 patients in whom the diagnosis had not been considered at the time of the physician's initial examination. The reasons for this were related to lack of spontaneous complaints of keratoconjunctivitis sicca and xerostomia and prominence of symptoms associated with arthritis, myalgia, pulmonary fibrosis, and cardiac disease. This study re-emphasizes the importance of multisystem disease in sjogren's syndrome and shows that specific serologic assays for autoantibodies aided in diagnosis.
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7/33. Antibodies to glycyl-transfer rna synthetase in patients with myositis and interstitial lung disease.

    OBJECTIVE. We have previously described anti-EJ antibodies, and provided evidence that these antibodies react with glycyl-transfer rna (gly-tRNA) synthetase. The aim of the present study was to identify patients with anti-EJ antibodies and describe the clinical associations of the antibody, in particular, whether it is associated with the syndrome of myositis and interstitial lung disease (ILD) that has been previously associated with autoantibodies to the aminoacyl-tRNA synthetases for histidine, threonine, and alanine. methods. Sera from patients with suspected or proven polymyositis or dermatomyositis (DM), sera with anticytoplasmic patterns, and control sera were tested for anti-EJ antibodies by immunoprecipitation (IPP). Positive sera and controls were tested for the ability to inhibit gly-tRNA synthetase by preincubation of the enzyme source with the serum. RESULTS. Anti-EJ antibodies were demonstrated in the sera of 5 patients, by IPP of characteristic tRNAs and protein. Original serum EJ and each of the new sera significantly inhibited the enzymatic activity of gly-tRNA synthetase but not histidyl-tRNA synthetase. All 5 of the new patients had inflammatory myopathy, a typical DM rash, and ILD. One, who had an overlap syndrome with systemic lupus erythematosus, had anti-EJ at least 4 months before the development of clinical myositis. arthritis and Raynaud's phenomenon, other features associated with antisynthetases, were also seen. CONCLUSION. Anti-EJ is associated with the syndrome of myositis and lung disease that is seen in association with other antisynthetases. The finding of specific inhibition of gly-tRNA synthetase by all anti-EJ-positive sera strongly supports the identification of EJ antigen as gly-tRNA synthetase.
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8/33. Increased expression of growth factor genes for macrophages and fibroblasts in bronchoalveolar lavage cells of a patient with pulmonary histiocytosis X.

    Pulmonary histiocytosis X is the local manifestation of a systemic disorder of unknown cause characterised by infiltration of Langerhans cell like histiocytes and parenchymal fibrosis. In a male smoker with histologically proved histiocytosis X and functional impairment bronchoalveolar lavage showed an increase in CD-1/OKT-6 antigen positive histiocytes to 8%. Northern blot analysis of rna from bronchoalveolar lavage cells showed an exaggerated expression of the M-CSF gene and of the c-fms gene encoding for the corresponding receptor. An increased level of c-sis rna, which encodes the B chain of platelet derived growth factor, was also found. Diffuse reticulonodular infiltrates on the chest radiograph resolved with glucocorticoid treatment and CD-1/OKT-6 antigen positive histiocytes fell to 3%. Macrophage colony stimulating factor, c-fms and c-sis gene expression were reduced almost to normal after treatment. The results suggest that macrophage colony stimulating factor and platelet derived growth factor may have a role in the initiation or maintenance of pathological reactions in pulmonary histiocytosis X.
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9/33. Lymphocytic interstitial pneumonia associated with a marked increase in monoclonal IgM-kappa-type rheumatoid factor and serum CA19-9.

    A 62-year-old Japanese female was admitted due to dyspnea. She showed a marked increase in CA19-9 (maximum, 192,000 U/ml) and monoclonal IgM-kappa type rheumatoid factor (RF) activity. The patient died of respiratory failure 3 months later. autopsy findings revealed an infiltration of IgM-kappa-positive plasma cells in the pulmonary interstitium, and therefore a diagnosis of lymphoid interstitial pneumonia (lip) was made. The bronchoepithelial cells were stained with monoclonal antibody-reactive with CA 19-9 antigen. This is the first documented lip, associated with a marked increase in monoclonal IgM-kappa type RF and CA19-9 in the serum.
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10/33. Disseminated adenovirus (type 19) infection in a neonate. Rapid detection of the infection by immunofluorescence.

    A case of fatal disseminated adenovirus infection in a neonate who suffered from severe keratoconjunctivitis and pneumonitis is reported. The diagnosis was made seven days after the onset of illness based on the detection of adenovirus antigen in the smears of the tracheal suction and conjunctival swab by immunofluorescence. Viral antigen was detected in the frozen or formalin-fixed autopsy specimens of the lungs, kidneys, spleen, liver and lymph nodes. Typical crystal arrangement of adenovirus virions was observed in the alveolar epithelial cells by electron microscopy. The isolated virus was identified to be of type 19 by a neutralization test. The IF examination using adenovirus group specific immune reagents on the smears of clinical specimens appears to be useful for rapid diagnosis of viral infections.
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