Diffuse interstitial lung disease due to AA amyloidosis.
A man developed interstitial lung disease and nephrotic syndrome due to AA amyloidosis. There was no evidence of an underlying disease predisposing to amyloidosis.
Aged↗
Biomedical subjects
D Valeyre
Publications and source records attributed to D Valeyre.
Pulmonary Langerhans' cell granulomatosis (histiocytosis X).
Pulmonary Langerhans' cell granulomatosis is usually classified among the lung diseases of unknown origin. Better appreciation of the characteristic clinical features of this disease, more detailed description of the pathologic lesions, and recent advances in the basic biology of Langerhans' cells have provided new insights into the pathogenesis of this process.
Cell Transformation, Neoplastic↗
Pulmonary sarcoidosis: CT assessment of lesion reversibility.
By comparing serial computed tomographic (CT) scans obtained when sarcoidosis was clinically active and after the onset of remission, an attempt was made to differentiate inflammatory from fibrotic lesions in the lungs of patients with sarcoidosis. Twenty patients with pulmonary infiltration seen on their chest radiographs were studied. For each patient, lesions found on the first CT scan were assessed by two observers as being decreased or increased on the second CT scan. Nodules (n = 8), irregularly marginated nodules (n = 5), and alveolar or pseudoalveolar consolidation (n = 5) always disappeared or clearly decreased. Septal lines (n = 10), nonseptal lines (n = 9), and lung distortion (n = 7) remained unchanged or increased. Some findings varied among patients: Micronodules (n = 9) and subpleural thickening (n = 5) disappeared or decreased in sarcoidosis of recent origin. Many findings of pulmonary infiltration seen on the first CT scan can be considered expressions of either inflammatory (reversible CT findings) or fibrotic (irreversible CT findings) lesions.
Adolescent↗
A search for mycobacterial DNA in granulomatous tissues from patients with sarcoidosis using the polymerase chain reaction.
We have used the polymerase chain reaction as a tool to detect the presence of mycobacterial DNA from organisms of the Mycobacterium tuberculosis complex and other species of mycobacteria in samples from patients with sarcoidosis. Using systems based on the amplification of a fragment of the gene coding for the 65-kD mycobacterial antigen, which were demonstrated to detect approximately 20 mycobacterial genomes/microgram total DNA, DNA from M. tuberculosis was reproducibly identified in DNA extracted from granulomatous tissues from two patients with sarcoidosis, but could not be detected in DNA extracted from tissue biopsies (n = 16) or cells recovered by lavage (n = 6) from most sarcoid patients or from control subjects (n = 22). Using a system based on the amplification of a fragment of the IS6110 insertion element, which could reliably detect two genomes of mycobacterial DNA/microgram total DNA, no additional positive results were observed. In an effort to identify another species of Mycobacterium present in granulomatous tissues from sarcoid patients but not control tissues, a fragment of the 65-kD mycobacterial antigen was amplified and then reamplified using "nested" primers recognizing sequences that are highly conserved among mycobacteria and closely related species, and the amplified DNA products were cloned and sequenced. Amplified DNA was observed in a minority of samples from patients and control subjects (32/84 and 34/77 attempts, respectively, p greater than 0.2), resulting from amplification of DNA from at least 17 different organisms.(ABSTRACT TRUNCATED AT 250 WORDS)
Base Sequence↗
Characterization of gamma/delta T-lymphocytes in the peripheral blood of patients with active tuberculosis. A comparison with normal subjects and patients with sarcoidosis.
Studies in experimental animals have suggested that gamma/delta T-cells play an important role in the immune response against mycobacteria, but evidence for the participation of these cells in the course of human tuberculosis remains fragmentary. We have evaluated the number and state of activation of gamma/delta T-cells in the peripheral blood of patients with active tuberculosis using two-color cytofluorometry, and we have sought evidence that these cells might play a role in the impaired responses to recall antigens seen in some patients by comparing the proliferation of blood T-lymphocytes before and after removing gamma/delta T-cells by panning. Results were compared with those obtained for cells from normal subjects and from patients with sarcoidosis. The proportion and absolute number of circulating CD3+ gamma/delta T-cells were not significantly different comparing blood from patients with tuberculosis and that from control subjects [54.6 +/- 39.9 (n = 17) and 59.1 +/- 30.2 cells/microliters (n = 10), respectively, p > 0.2], and the proportion of cells expressing receptors using the V delta 1 variable region remained unchanged in patients with tuberculosis. Few gamma/delta T-cells from patients with tuberculosis expressed surface antigens associated with activation (IL-2R, < 1%; HLA-DR, 2.6 +/- 3.4%). Four of 15 patients with sarcoidosis had a proportion of gamma/delta T-cells that was outside the range observed in normal subjects, but the absolute number of CD3+ gamma/delta T-lymphocytes was not different comparing the two groups (p > 0.2).(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Bronchogenic carcinoma in patients with pulmonary histiocytosis X.
Five cases of bronchogenic carcinoma were observed among 93 patients with pulmonary histiocytosis X (Hx). Mean age at the time of diagnosis of Hx was 42 years; on the average, cancer occurred 10.5 years later. All patients were smokers and continued to smoke heavily at the time of diagnosis of cancer. Comparison of the five cases associating Hx and lung carcinoma with a group of 88 control patients suffering from Hx alone suggested that smoking played the predominant role in the pathogenesis of cancer. In fact, among the four patients with Hx and carcinoma older than 45 years, tobacco consumption was significantly greater (64.7 +/- 37 pack-year, mean +/- SD) than that of the 15 control patients of the same age with only Hx (40.8 +/- 11.6, p less than 0.01). In light of this good correlation, the diagnosis of Hx strongly advocates stopping tobacco smoking and long-term medical follow-up.
Adolescent↗
[Diagnosis of diffused interstitial fibrosing lung diseases].
Diagnosing chronic diffuse infiltrative lung diseases is often difficult and requires a strict diagnostic approach. The patient's history and in some cases the presence of extrathoracic signs make it possible to distinguish between primary and secondary lung disease (notably pneumopathy). Radiological imaging, functional exploration, bronchoalveolar lavage and histopathology must be performed in a rational order, taking into account their degree of invasiveness and the diagnostic criteria they may provide. When as a result of these investigations the disease has been classified in its aetiological category, its anatomical repercussions must evaluated by non-invasive explorations yielding a true "lesional diagnosis".
Humans↗
[CD8+T-cell lymphocytic alveolitis in sarcoidosis. 4 cases].
We report 4 cases of histologically proven sarcoidosis with lymphocytic alveolitis. The T CD4+/T CD8+ ratio was below 1 in the bronchoalveolar lavage fluid. Since there was no clinical and paraclinical evidence of extrinsic allergic alveolitis or HIV infection, and since 3 of the patients were nonsmokers and the 4th one smoked moderately, we considered that the T CD8+ lymphocytic alveolitis was related to the sarcoidosis. In 3 patients the presence of mediastinal adenopathy suggested that sarcoidosis was of recent onset. The fact that T CD4+/T CD8+ ratio was normal in blood indicated that T CD8+ lymphocytes were predominant only in the lung. Thus, the finding of T CD8+ lymphocytic alveolitis should not exclude a diagnosis of sarcoidosis.
Adult↗
Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high-resolution CT.
The diagnostic value of chest radiography and high-resolution computed tomography (CT) in chronic diffuse interstitial lung disease (CDILD) was assessed in 140 consecutive patients with diffuse infiltration of the lung visible at radiography. Radiographs and CT scans were separately read by three independent observers without knowledge of clinical and pathologic data. The observers listed the three most likely diagnoses and recorded the degree of confidence they had in their choice on a 0%-100% probability scale. Findings at radiography and high-resolution CT were recorded by each observer and were used for a stepwise discriminant analysis between diagnoses. First-choice diagnoses of all three observers that were made with a high level of confidence (probability, greater than or equal to 75%) were more accurate with CT than with radiography (P less than .001). The superiority of high-resolution CT over radiography was most obvious for histiocytosis X and sarcoidosis; in cases of pulmonary fibrosis, CT was not significantly different from radiography. The interobserver agreement for the proposed diagnosis was significantly better with high-resolution CT (P less than .001). Twenty-one of 26 radiographic findings and 21 of 25 CT findings were discriminant. Stepwise discriminant analysis revealed the superiority of CT over radiography, since the ranking of all findings showed that the four most discriminant findings, and eight of the first 12 findings, were revealed with CT.
Adolescent↗
Glucose, K+, and albumin concentrations in the alveolar milieu of normal humans and pulmonary sarcoidosis patients.
Some properties of the alveolar epithelial barrier during transalveolar transport of water and solutes were studied in normal humans and patients with sarcoidosis by means of the transalveolar capillary concentration gradients of various solutes. A total of 9 normal control subjects (Group A) and 60 sarcoidosis patients, 52 with an evolving disease (Group B) and 8 recovered (Group C), underwent bronchoalveolar lavage (BAL). The second aliquot of fluid was used to measure urea, glucose, potassium, and albumin, which were also investigated in plasma. Urea was used to determine the volume of alveolar epithelial lining fluid (AELF volume). Results are expressed as the ratio of solute concentration in AELF over that in plasma (A/P ratio). In Group A there were clear concentration gradients of glucose, potassium, and albumin between the AELF and plasma, as the A/P ratios of glucose, potassium, and albumin were 0.02 +/- 0.006 (mean +/- SEM), 3.2 +/- 0.34, and 0.04 +/- 0.008, respectively. In Group B the A/P ratios of glucose (0.21 +/- 0.02, p less than 0.001) and albumin (0.17 +/- 0.02, p less than 0.001) were significantly increased but that of potassium remained unchanged (2.9 +/- 0.2). The A/P ratios of these various solutes were independent of chest x-ray typing. The albumin but not the glucose A/P ratio was correlated with the percentage of lymphocytes recovered from BAL (p less than 0.02); however, there was no correlation between the albumin A/P ratio and the CD4+/CD8+ T lymphocyte ratio. In group C there was a striking contrast between the albumin A/P ratio, which was normal, and the glucose A/P ratio, which was clearly elevated despite a normal lymphocyte count.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Expression of 1,25(OH)2D3 receptors on alveolar lymphocytes from patients with pulmonary granulomatous diseases.
1,25(OH)2D3 is known to be produced at sites of granulomatous reactions. In order to characterize the cell types that are targets for this immunoregulatory hormone, we have evaluated the expression of 1,25(OH)2D3 receptors on peripheral blood T-lymphocytes and those recovered from the lung by bronchoalveolar lavage from patients with pulmonary granulomatous diseases (tuberculosis and sarcoidosis) and from normal control subjects using combined autoradiographic and immunohistochemical techniques. Lavage T-lymphocytes from patients with tuberculosis or with sarcoidosis, but not those from normal control subjects, expressed 1,25(OH)2D3 receptors as demonstrated by binding of [3H]1,25(OH)2D3, which was inhibited by the presence of excess unlabeled 1,25(OH)2D3, but not by the presence of unlabeled 25(OH)D3 (receptor-positive lymphocytes: sarcoidosis, 20 +/- 12%; tuberculosis, 31 +/- 17%). In contrast, blood lymphocytes from patients with granulomatous diseases did not express detectable 1,25(OH)2D3 receptors. The percentage of lavage T-lymphocytes expressing 1,25(OH)2D3 receptors was significantly greater for patients with tuberculosis presenting with isolated hilar adenopathy than for patients with pulmonary infiltrates and/or cavities. 1,25(OH)2D3 receptors were expressed to a greater extent on CD8+ T-lymphocytes than on CD4+ T-lymphocytes in sarcoidosis, whereas a greater proportion of CD4+ than of CD8+ T-lymphocytes from patients with tuberculosis were receptor-positive. These findings support the conclusion that the interaction of 1,25(OH)2D3 with its receptor on T-lymphocytes may play an important role in the regulation of granulomatous reactions, but because these receptors are expressed on different lymphocyte populations, the net effect of this potent immunoregulatory molecule is likely different in sarcoidosis and tuberculosis.
Adult↗
Antigen-induced proliferative response of lavage and blood T lymphocytes. Comparison of cells from normal subjects and patients with sarcoidosis.
To evaluate the mechanisms responsible for anergy in sarcoid patients, we studied the ability of highly purified lavage and blood T lymphocytes from control subjects and patients with sarcoidosis to proliferate in response to recall antigens, and compared the results of antigen-induced lymphocyte proliferation with the clinical characteristics of the patients. Both blood and lavage T lymphocytes from all control subjects proliferated in response to purified protein derivative (PPD) and candida antigens, and no significant difference was observed comparing the proliferation of lymphocytes from the two sources. The antigen-induced proliferation of blood and lavage T lymphocytes from sarcoid patients was reduced compared with that of the corresponding cell populations from normal subjects (p less than 0.01 for PPD, Candida, and tetanus), and the proliferative response of sarcoid lavage lymphocytes was significantly lower than that of blood T lymphocytes from these patients. No evidence for inhibition of T lymphocyte proliferation by accessory cells (blood monocytes) or CD8+ T lymphocytes was observed, and the refractory state could not be overcome by adding exogenous recombinant human IL-2 or IL-4. An inverse correlation was observed between the PPD-induced proliferation of sarcoid lavage T lymphocytes and criteria associated with "active" disease, including lymphocytes/ml lavage fluid (p less than 0.003), 67Ga uptake (p less than 0.005), and serum angiotensin converting enzyme activity (p less than 0.005). Lavage lymphocytes from patients studied early in the course of the disease proliferated better than those from patients with more long-standing disease.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Gamma/delta T-lymphocytes are not increased in number in granulomatous lesions of patients with tuberculosis or sarcoidosis.
gamma delta T-lymphocytes have been suggested to play a role in granuloma formation and to participate in the immune responses observed in tuberculosis and sarcoidosis. To further evaluate the role of gamma delta T-lymphocytes in these disorders, we used immunohistochemical techniques and specific monoclonal antibodies to characterize the number of distribution of T-lymphocytes expressing alpha beta and gamma delta T-cell receptors in granulomatous lesions from patients with sarcoidosis (n = 8) or tuberculosis (n = 4). The vast majority of T-lymphocytes present in lymph nodes from patients with tuberculosis or sarcoidosis expressed alpha beta TCR, whereas only rare gamma delta T-lymphocytes were observed inside or at the periphery of the granulomas. Recently formed granulomas induced by positive Kveim test reactions in patients with sarcoidosis also contained only small numbers of gamma delta T-lymphocytes, including those present in biopsies from two patients with increased numbers of circulating gamma delta T-lymphocytes. These observations demonstrate that T-cells involved in immune granulomatous reactions in sarcoidosis and tuberculosis express predominantly alpha beta TCR, and they do not support the conclusion that gamma delta T-lymphocytes necessarily play an important role in granuloma formation.
Adult↗
Autoimmune haemolytic anaemia and idiopathic pulmonary fibrosis associated with HLA-B27 antigen.
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Aged↗
Pulmonary Wegener's granulomatosis. A clinical and imaging study of 77 cases.
We studied 77 patients with biopsy-proven WG and pulmonary manifestations, to characterize the nature and frequency of the clinical, imaging and endoscopic features of this condition. Pulmonary symptoms were cough, mild dyspnea, hemoptysis and chest pain. Five patients had no pulmonary symptoms. Imaging features consisted of nodules, infiltrates and pleural opacities. A CT scan proved useful by disclosing cavities in opacities or opacities which were not seen on an x-ray film. Fiberoptic bronchoscopy was performed in 74 patients, and it was macroscopically abnormal in 55 percent (showing bronchial inflammation or stenosis or both or isolated hemorrhage). Six patients presented with alveolar hemorrhagic syndrome. Four patients had a pleural exudate rich in polymorphonuclear leukocytes. The WG was limited to the lung in seven patients. Sixteen patients died because of active disease or iatrogenic complications (two). An improved knowledge of clinical and imaging features of WG could help the clinician reach an earlier diagnosis.
Adolescent↗
[Sarcoidosis associated with hemolytic anemia and autoimmune thrombocytopenia].
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Anemia, Hemolytic↗
[Chronic idiopathic eosinophilic pneumopathy. Carrington's disease].
Eleven cases of idiopathic chronic eosinophilic pneumonia were studied. Ten of the patients were followed up for more than 3 years and the last one for 15 years. The female predominance observed, the mean age of 51 years, the late manifestations of asthma-like symptoms preceding the systemic signs by 8 1/2 months on average and the scarcity of extra-pulmonary manifestations characterized the clinical data. The most frequent radiological signs were non-systematized, bilateral alveolar opacities in the axillary and apical regions, sometimes migratory and giving in 9 cases a mirror image of pulmonary oedema. Beside this major inflammatory syndrome, blood eosinophilia was present in 10 patients with 4,500 eosinophils on average per cubic millimetre. Eosinophils were also present in the bronchoalveolar lavage fluid of those who underwent this examination. Both clinical and radiological signs responded to corticosteroids in all cases, with return to normal chest radiography within less than 10 days. Subsequently, the possibility of radiological relapse in the same or other territories, together with the development of a steroid-dependent intrinsic asthma accounted for the fact that after a follow-up of about 3 years corticosteroid therapy could not be discontinued in any of our patients.
Adult↗
Urinary neopterin in pulmonary sarcoidosis. Relationship to clinical and biologic assessment of the disease.
Neopterin is a metabolite of guanosine-triphosphate, released in vitro by macrophages under the control of gamma-interferon and described as a marker of T cell activation in vivo. We have compared the urinary neopterin/creatinine ratio (mumol/mol) in patients with pulmonary sarcoidosis (n = 66), interstitial lung diseases other than sarcoidosis (nonsarcoid ILD, n = 35), and 45 normal control subjects. For the sarcoid population as a whole, urinary neopterin was higher (496 +/- 52 mumol/mol [mean +/- SEM]) than in control subjects (126 +/- 5 mumol/mol) (p less than 0.001). In patients with nonsarcoid ILD, urinary neopterin was frequently higher in granulomatous and/or lymphoproliferative diseases (hypersensitivity pneumonitis, tuberculosis, primitive Sjögren's syndrome, and malignant lymphomas) (781 +/- 193 mumol/mol, n = 10) but remained normal in other types of nonsarcoid ILD [( 163 +/- 14 mumol/mol, n = 25]: histiocytosis X, idiopathic pulmonary fibrosis, lung collagen-vascular diseases, diffuse neoplasms, pneumoconiosis; p less than 0.001 compared with sarcoidosis). We have also evaluated the relationship between urinary neopterin and the clinical or biologic markers currently used to assess sarcoidosis: alveolar lymphocytosis in lavage fluid (ALY), 67-gallium scan semiquantitative index (67Ga), or serum angiotensin-converting enzyme (SACE). Sarcoid patients with the highest urinary neopterin were those in whom mean values of these markers were the highest (p less than 0.05, all comparisons). Patients with positive markers (i.e., either clinical expression of sarcoidosis-ALY greater than 30%-67Ga greater than 20-SACE greater than 60 U/ml) had significantly higher urinary neopterin levels than did other sarcoid patients (p less than 0.05, all comparisons).
Adult↗