[Diagnosis of sarcoidosis].
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Biomedical subjects
Publications and source records attributed to R Loddenkemper.
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AIM: To determine the value of high-resolution MRI in pleural and chest wall diseases, the normal and pathologic costal pleura and adjacent chest wall between paravertebral and the axillary region were examined with contrast enhanced high-resolution T1-weighted MRI images using a surface coil. MATERIAL AND METHODS: Normal anatomy was evaluated in 5 healthy volunteers and a normal specimen of the thoracic wall, and correlation was made with corresponding HR-CT and histologic sections. CT-proved focal and diffuse changes of the pleura and the chest wall in 36 patients underwent HR-MRI, and visual comparison of MRI and CT was done retrospectively. RESULTS: Especially sagittal T1-weighted HR-MRI images allowed accurate delineation of the peripleural fat layer (PFL) and the innermost intercostal muscle (IIM), which served as landmarks of the intact inner chest wall. PFL and IIM were well delineated in 3/4 patients with tuberculous pleuritis, and in all 7 patients with non-specific pleuritis, as opposed to impairment of the PFL and/or the IIM, which was detected in 15/18 malignancies as a pattern of malignant chest wall involvement. In one case of tuberculous pleural empyema with edema of the inner chest wall HR-MRI produced false positive diagnosis of malignant disease. CONCLUSION: HR-MRI images improved non-invasive evaluation of pleural and chest wall diseases, and allowed for differentiation of benign and malignant changes.
This overview of the indications for interventional thoracoscopy is far from exhaustive and new applications will surely be proposed. In conclusion, thoracoscopy provides diagnosis of pleural-based malignancy or tuberculosis with a high degree of accuracy when routine cytology and closed-needle pleural biopsies have failed. In patients in whom adequate visualization can be accomplished, an unequivocal pathologic diagnosis of benign disease can be made with a specificity approaching 100%. If transbronchial biopsy and bronchoalveolar lavage are inconclusive, VATS lung biopsy appears to be a safe alternative to open lung biopsy by thoracotomy for diagnosis of diffuse interstial or infectious lung disease. Thoracoscopy is often effective in the management of malignant pleural effusion and spontaneous pneumothorax. A close working relationship between pulmonary physicians and thoracic surgeons will assure that patients undergoing diagnostic thoracoscopy, under local anesthesia with intravenous sedation in the pulmonary endoscopy suite, are appropriate candidates for this procedure. It is absolutely mandatory that physicians intent on performing this procedure be adequately trained. We believe that collaboration between thoracic surgeons and pulmonologists not only facilitate training in thoracoscopy, but also insure that patients undergoing thoracoscopy will be carefully assessed from both perspectives.
Previous studies on the relationship between activated BAL T lymphocytes and clinical features in sarcoidosis revealed controversial results. We determined lavage lymphocytes, T lymphocytes, activated T lymphocytes, helper, suppressor and natural killer cells in 50 patients with pulmonary sarcoidosis and compared the results with clinical findings, serum angiotensin converting enzyme (ACE), the radiological pattern and lung function data (vital capacity [VC], total lung capacity [TLC], FEV1, transfer coefficient KCO and arterial-alveolar oxygen difference [AaDO2] during exercise). Patients with erythema nodosum (n = 7) showed a lower proportion of activated T lymphocytes (p < 0.05) and lymphocytes (p < 0.01) than the other patients. There was a significant correlation between activated T lymphocytes and AaDO2 during exercise (r = 0.49, p < 0.001), and an inverse correlation was seen between activated T lymphocytes and VC or TLC (r = -0.35 and r = -0.36, p < 0.01). We conclude that the percentage of activated BAL T cells may be related to the degree of parenchymal involvement as expressed by functional disturbance.
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BACKGROUND: Circulating immune complexes can be elevated in serum samples of patients with sarcoidosis and are associated with disease activity, but their diagnostic significance is not understood. METHODS: The different classes of circulating immune complexes containing immunoglobulin A, G, or M, and the content of complement in circulating immune complexes (polyethylene glycol precipitation) as well as levels of complement binding circulating immune complexes (complement binding assay) were determined in 19 patients with active, untreated pulmonary sarcoidosis. The results were compared with other parameters in the serum (soluble interleukin 2 receptor, angiotensin converting enzyme, immunoglobulin A, G, and M) and the bronchoalveolar lavage fluid (lymphocytes, helper cells, suppressor cells, activated T cells), and with radiological stage and functional parameters (FEV1, vital capacity, total lung capacity, transfer coefficient (KCO), and the alveolar-arterial oxygen difference during exercise). RESULTS: In all patients circulating immune complexes could be detected by polyethylene glycol precipitation and were similar to control subjects. The content of C1q in circulating immune complexes was higher than in controls, yet in all but one of the cases was still within normal limits. In contrast, elevated levels of complement binding circulating immune complexes were found in 67% of the patients. No correlation was seen between circulating immune complexes and any of the other parameters in the serum, bronchoalveolar lavage fluid, or lung function values. No differences were found between radiological type I and II presentations of sarcoidosis. CONCLUSIONS: The complement binding assay showed a much higher sensitivity for the detection of circulating immune complexes in active pulmonary sarcoidosis than the polyethylene glycol precipitation method. As there was no correlation between levels of circulating immune complexes and other parameters of the disease they are probably not useful for the assessment of disease activity.
The decision for surgical measures in bronchial carcinoma is mainly based on the preoperative assessment of the presumed tumour extent and cell type, as well as cardiopulmonary function. Bronchoscopy, as an obligatory procedure, allows a definite diagnosis in most of the cases, definition of the most proximal tumour extension and exclusion of bilateral endobronchial disease. Transbronchial needle aspiration can provide important information on lymph node involvement, although mediastinoscopy has the highest diagnostic yield and remains the standard procedure for lymph node staging. Thoracoscopy, providing a much higher sensitivity than thoracentesis, enables differentiation between malignant or para-malignant pleural effusion in almost all of the cases. Thus, endoscopy alone often demonstrates unresectability.
A 50 year old male smoker was admitted with nonproductive cough, weight loss and bilateral infiltrates predominantly in the upper lung zones on chest X-ray. Before admission he had been treated for suspected tuberculosis without improvement. The definite diagnosis of pulmonary histiocytosis X was made by open lung biopsy. High resolution computed tomography revealed the typical "ring fixtures" distributed in the parenchyma. Diffusing capacity for carbon monoxide was markedly reduced, besides mild obstruction. After six months of observation, there was seen a radiological improvement with unchanged lung function parameters.
Recently thoracoscopy has been used with increasing frequency for the diagnosis and treatment of pleuropulmonary diseases. METHODS: The main requirements for thoracoscopy are rigid telescopes, forceps, scissors, stapler and a video recorder. The procedure can be performed either under general anaesthesia with or without double lumen intubation or under neuroleptanalgesia after inducing an artificial pneumothorax. At the end of the procedure a chest tube should always be inserted even if it is only for a few minutes until the lung re-expands after diagnostic thoracoscopy. Complications are exceptional and mortality is less than 0.017%. INDICATIONS AND RESULTS: Thoracoscopy is useful for diagnosis of a number of lung diseases. For pleural effusion, the sensitivity of thoracoscopy is 92-97% and its specificity is 99%. This is much better than needle pleural biopsy and/or fluid cytology. In malignant mesothelioma, thoracoscopy allows accurate staging. Similarly in spontaneous pneumothorax, classification based on the endoscopic aspects of the lung according to the classification of Vanderschueren allows a better selection of therapeutic alternatives. For diffuse pulmonary diseases, thoracoscopic lung biopsy has a sensitivity ranging from 60-98% depending on whether the underlying disease is sarcoidosis, idiopathic fibrosis, collagenous diseases or other rare diseases. Interventional thoracoscopy is a rapidly expanding domain. In this review the most widespread techniques are summarized. Thoracoscopic pleurodesis is performed for pleural effusion. It can be achieved by talc poudrage but other methods are available. For spontaneous pneumothorax, pleurodesis must be associated with treatment of the causal lesions. The other therapeutic procedures described here are sympathectomy for palmar hyperhidrosis, pulmonary biopsy using an endo-GIA stapler and pericardial biopsy.
Clinical appearance, radiologic findings, lung function and results of corticosteroid therapy were analyzed in 42 adult cases of biopsy-proven pulmonary histiocytosis X. Symptoms were present in only 64%. Using the ILO classification 1980, the evaluation of chest radiographs revealed all categories of profusion, size and shape of nodules. The characteristic 'ring figures', i.e. thin-walled cysts, in the parenchyma were detected in 78% of conventional tomographies (n = 27) and in all patients examined by CT (n = 5). In patients with early disease, lung function tests (n = 26) including body plethysmography, pulmonary diffusing capacity and ergospirometry revealed that parameters of gas exchange are most sensitive (TL,CO 84%, KCO 72%). Bronchial reactivity to carbachol was significantly higher than in controls (n = 12). Lung perfusion scintigram showed an abnormal, but uncharacteristic pattern in 81% (n = 26). During corticosteroid therapy, no progression was observed (n = 36). 85% of patients with radiographic evidence of progressive disease improved after administration of corticosteroids (n = 14).
Thoracoscopy is increasingly being used for diagnosis and treatment of pleuropulmonary disease. The recent revival was made possible by the tremendous advances in endoscopic technology. The main requirements for diagnostic purposes are rigid telescopes and forceps, and for interventional thoracoscopy scissors, staplers and a video recorder. The procedure can be performed either under local or general anaesthesia, with or without double lumen intubation, after inducing an artificial pneumothorax. At the end of the procedure, a chest tube should always be inserted, even if only for a few minutes until the lung re-expands. Main diagnostic indications are pleural effusions, pneumothorax and diffuse lung disease. Main therapeutic indications are pleurodesis by talcage in effusion and pneumothorax and a variety of diseases of the lung, the pleura and the mediastinum, where thoracotomy may be replaced by video-assisted thoracoscopy. The well-known indications of the past remain a domain of pneumologists, whereas minimal invasive thoracotomy is the task of thoracic surgeons. For some indications no sharp line has to be drawn, provided the facilities and skills are present, including those for the management of complications.
Alpha 1-proteinase inhibitor (alpha 1-PI) has been demonstrated to suppress mitogen-induced lymphocyte response in vitro. To evaluate the effect of intravenous application of human alpha 1-PI (Prolastin HS) on cellular immunity, we determined total lymphocyte count, lymphocyte subsets and lymphocyte response to concanavalin A, before and 24 h after infusion of 60 mg.kg-1 body weight alpha 1-PI in eight patients with homozygous alpha 1-PI deficiency (PiZ phenotype). The results were compared with two blood samples from seven healthy controls. After infusion, serum alpha 1-PI levels were increased from 0.98 +/- 0.24 to 2.68 +/- 0.51 g.l-1. No significant differences were found for total lymphocyte count, lymphocyte subsets and lymphocyte response between both groups in both samples. Maximum 3H-thymidine incorporation before and after infusion showed no significant difference; the same was true for the two control samples. However, additional incubation in vitro with alpha 1-PI 5 g.l-1 led to a significant (p < 0.03) decrease of lymphocyte proliferation in samples after infusion. Our data indicate that alpha 1-PI substitution therapy does not lead to a major suppression of lymphocyte response to concanavalin A in PiZ individuals in vivo, although a suppressive effect was found after additional in vitro incubation with alpha 1-PI.
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The MRI and CT appearances in 48 patients with histologically confirmed benign and malignant pleural abnormalities were compared retrospectively. Abnormal pleural changes were shown in 47 out of the 48 patients by high signal intensity of the pleura in T2-weighted sequences and in contrast enhanced T1-weighted sequences on MRI. CT showed abnormalities in 45 out of 48 patients. Delineation of pleural and pulmonary changes by CT was possible in 13 out of 23 cases, and pleural disease from effusions in 15 out of 28 cases. T2-weighted MRI was successful in 14 out of 23 and 4 out of 28 cases, respectively. T1-weighted images after contrast were successful in 20 out of 23 and 22 out of 28 cases, respectively. Indications of malignant pleural disease were the presence of mediastinal or circumferential involvement or involvement of the entire pleura, thickness of more than 10 mm and nodular changes. The most reliable sign of malignancy was infiltration of the thoracic wall and the diaphragm; this was better demonstrated by MRI (18 out of 19 and 2 out of 2 cases) than by CT (14 out of 19 and 0 out of 2 cases).
In 29 patients with chronic bronchitis, 17 of whom were receiving systemic prednisolone, parameters of cellular and humoral immunity from the blood were evaluated and compared with a control group. Serum concentrations of immunoglobulins IgG, A and M, IgG subclasses, lymphocyte subsets and lymphocyte response to mitogenes (PHA, ConA, PWM), antibodies (anti-CD3) and tetanus antigen were determined. Whereas T cell subsets did not show any difference between all groups, the relative proportion of B lymphocytes was lower in the patient group without corticosteroids. Both patient groups showed a decreased lymphocyte response. As expected, serum IgG was lower in the steroid group, which could not be explained by additional cellular findings. Our data indicate that patients suffering from chronic bronchitis may show signs of a decreased cellular immunity.