Lymphangioleiomyomatosis: a study of 72 patients from the Spanish registry.

BACKGROUND: Pulmonary lymphangioleiomyomatosis (LAM) is a rare lung disease that almost exclusively affects young women of childbearing age. The true incidence and prevalence of LAM are unknown. This study was conducted to evaluate the characteristics of lymphangioleiomyomatosis in Spain. METHODS: Over a 2-year period, a questionnaire designed for this study was collected. This questionnaire included sociodemograhic, clinical, radiological and functional data. Information about the study and this questionnaire were both sent by e-mail to all the participants of the interstitial disease registry of 2004. RESULTS: Seventy-two patients, all of whom were women, were included in the registry, with a mean age of 44.56 +/- 11.1 yr. Sixty-three patients (87.5%) presented the sporadic LAM and 9 (12.5%) presented LAM associated with tuberous sclerosis (LAM-TS). LAM diagnosis was confirmed with an open lung biopsy in 57 patients (79.2%) and was performed with thoracic HRCT compatible with LAM diagnosis in the other 15 cases. The most frequent symptom was dyspnoea (90%) followed by cough (44.4%). Almost 40% of patients presented renal angiomyolipomas in the study and the most frequent spirometric pattern was obstructive in more than half of the patients. Most patients with LAM-TS (88.8%) had renal angiomyolipomas compared with 31.7% in the sporadic LAM group. CONCLUSION: The characteristics of the Spanish population affected with LAM are similar to those of other countries. Most patients were symptomatic, had a history of previous pneumothorax and presented abnormal radiological findings and pulmonary function tests.

Are pleural fluid parameters related to the development of residual pleural thickening in tuberculosis?

STUDY OBJECTIVE: Identification of predictive factors for the development of residual pleural thickening (RPT). DESIGN: Retrospective study. LOCATION: A 1,500-bed tertiary hospital. PATIENTS: Patients with pleural tuberculosis diagnosed between December 1991 and February 1995 in our Respiratory Disease Service. INTERVENTIONS: The clinical and radiologic characteristics, and measurements of microbiological and biochemical parameters and markers in pleural fluid were studied. RPT was defined in a posteroanterior chest radiograph as a pleural space of >2 mm measured in the lower lateral chest at the level of an imaginary line intersecting the diaphragmatic dome. MEASUREMENTS AND RESULTS: In 56 patients studied, 11 (19.6%) had RPT 10 mm and 24 (42.8%) had RPT >2 mm. The pleural fluid of patients with RPT 10 mm had a significantly lower glucose concentration and pH and higher lysozyme and tumor necrosis factor-alpha levels than the other patients. The pleural fluid of patients with RPT >2 mm showed no significant differences. CONCLUSIONS: The development of RPT 10 mm was related to higher concentrations of lysozyme and tumor necrosis factor-alpha and lower glucose concentration and pH in pleural fluid compared with development of lower measurements of RPT.

Determination of tissue polypeptide-specific antigen in pleural fluid and serum from patients with pleural effusion.

As a tool for differentiating malignant and benign pleural effusions, we evaluated the diagnostic value of the assay of tissue polypeptide-specific antigen (TPS) in pleural fluid and serum, and of the pleural fluid TPS/serum TPS ratio in patients with pleural effusion. We studied prospectively 147 consecutive patients who had pleural effusions: 43 malignant pleural effusions and 104 benign pleural effusions. TPS levels were measured by RIA. The sensitivity and specificity of these measurements were: TPS in pleural fluid (cutoff 20,000 U/L): 0.21 and 0.98; TPS in serum (cutoff 300 U/L); 0.31 and 0.96; pleural fluid TPS/serum TPS ratio (cutoff 1200): 0.07 and 0.99. All these values enhanced the sensitivity of cytologic analysis of pleural fluid. However, we conclude that TPS assay in pleural fluid and serum, and the pleural fluid TPS/serum TPS ratio have limited diagnostic value in patients with pleural effusion.

[Prospective study of 1,000 consecutive patients with pleural effusion. Etiology of the effusion and characteristics of the patients]. / Estudio prospectivo de 1.000 pacientes consecutivos con derrame pleural. Etiología del derrame y características de los pacientes.

OBJECTIVE: To describe the characteristics of patients with pleural effusion (PE) and the causes of PE in a prospective, consecutive series of patients. SETTING: A tertiary care hospital associated with the Universidad Complutense de Madrid (Spain). PATIENTS: One thousand consecutive patients with PE for whom clinical signs indicated the need for diagnostic thoracocentesis were studied prospectively in our service from December 1991 to July 2000. RESULTS: The most common cause of PE was neoplasm (n = 364 patients). The most common place of origin of the tumor was the lung (n = 125), followed by the pleura (mesothelioma, n = 48). The most common histologic type was adenocarcinoma (n = 128). Tuberculosis was the second most common cause of PE (n = 155). PE was transudate in 118 patients, mainly secondary to heart failure. Among the 42 patients who were positive for human immunodeficiency virus (HIV), the most common cause of PE was tuberculosis. Tuberculosis was also the most likely cause of PE in patients under 40 years of age. CONCLUSIONS: The most common causes of PE were neoplasm and tuberculosis. Tuberculosis was the most common cause in patients under 40 years of age and in those infected by HIV.

[Tracheobronchial granular-cell tumors. A report of 8 cases]. / Tumores de células granulares traqueobronquiales. Presentación de 8 casos.

To describe the clinical and radiologic signs, evolution and response to treatment of patients diagnosed of tracheobronchial granular cell tumors (GCT) in our practice. Retrospective computerized review of all bronchoscopic procedures performed in our hospital from January 1974 through November 1996. Patients with GCT were identified and their case histories reviewed. Eight male patients with 9 GCT were identified. Mean age was 55 years. Only one was symptomatic (hemoptysis) and only one had radiologic signs (solitary pulmonary node). Six patients were diagnosed of tumors in other organs. With the exception of one GCT located in the trachea, all were found in the right bronchial system. Endoscopy revealed mucosal abnormalities in six patients and nodes in three. Treatment was conservative in four patients, endoscopic in three (2 mechanical endoscopic resections and one laser Nd-YAG resection in a patient with two tumors), and surgical in one. Disease evolution as treated was favorable during the observation period. Tracheobronchial GCT are rare tumors. Most patients were between 50 and 70 years old, were often asymptomatic and had few radiologic manifestations. They suffered accompanying neoplasia in other organs. GCT were located in central and segmentary bronchial and nodes or abnormal mucosa could be seen endoscopically. Treatment was conservative, endoscopic or surgical, depending on tumor size and patient characteristics.

[Ambulatory diagnosis of patients requiring a pleural biopsy. Study of 100 consecutive cases]. / Diagnóstico ambulatorio de los pacientes que precisan biopsia pleural. Estudio de 100 casos consecutivos.

Our aim was to describe our experience with outpatients requiring pleural biopsy. The first 100 patients with pleural exudate from whom at least one pleural biopsy specimen was taken on an outpatient basis at our hospital since January 1993. Clinical and radiological signs were recorded for all patients. We also analyzed complications, frequency of use of other diagnostic test and time required for diagnosis. The mean age of the 100 patients (64 men) was 56 years. Outpatient study of these patients was not prevented by the advanced age on the sample (with 35 patients older than 79), the size of the pleural effusions (23 of which were large), or the diagnosis of diseases with poor prognoses (with 43 effusions diagnosed as neoplastic). Complications encountered in taking the biopsy specimens were similar to those reported for other series. The mean time until diagnosis was 7.4 days. Biopsies can often be obtained from patients with pleural effusion on an outpatient basis, even when clinical symptoms vary widely, without increasing the number of complications and within a reasonable period of time.

Chylothorax and chylous ascites due to heart failure.

Chylous ascites and chylothorax have rarely been reported as a consequence of severe right heart failure. To our knowledge, this is the first case report of both disorders occurring as a result of ischaemic cardiomyopathy. The autopsy findings and possible mechanisms of production are discussed.

Measurement of pleural pressure during therapeutic thoracentesis.

The aim of therapeutic thoracentesis (TT) is to aspirate as much pleural fluid as possible. Monitoring pleural pressure (PlP) during TT has been proposed to avoid the adverse effects due to an unintended sharp drop in PlP. The objectives of this study are to ascertain the diagnostic value of the PlP measurement, to find a predictive variable of the amount of fluid that can be removed, to obtain insight into the characteristics of the PlP curve and pleural elastance (PE) during TT, and to describe the complications of TT. Sixty-one unselected patients were studied. Only the four patients with suspected trapped lung had an initial PlP lower than -4 cm H(2)O and a PE higher than 33 cm H(2)O/L. There was a weak correlation (r = 0.52) between PE during the first 0.5 L aspirated and the total amount of fluid aspirated. Partial PE values were 10, 7.5, and 14 cm H(2)O/L at the early, intermediate, and late phases of TT. No complications were found except for nine pneumothoraces. In conclusion, the technique was clinically helpful because large amounts of pleural fluid could be aspirated with few and mild complications, and because it allows clinicians to support the preliminary diagnosis of trapped lung. None of the studied variables was found to predict the suitability of aspirating more than 1.5 L. Rather than being monotonically descendent, the PlP curve shows a three-part line with the deepest slopes at the first and last phases of the thoracentesis.

Diagnostic value of CA 72-4, carcinoembryonic antigen, CA 15-3, and CA 19-9 assay in pleural fluid. A study of 207 patients.

BACKGROUND: The differential diagnosis of pleural effusion is a frequent clinical problem. Several tumor markers have been evaluated in pleural fluid, but the value of CA 72-4 assay and of combinations of tumor marker assays has not been firmly established. To find a minimally invasive tool for differentiating between pleural effusions of malignant or benign origin, the authors assessed the diagnostic value of CA 72-4, carcinoembryonic antigen (CEA), CA 15-3, and CA 19-9 assays in pleural fluid individually and in combination. METHODS: The authors prospectively studied 207 patients with pleural effusion (65 malignant, 48 tuberculous, 24 parapneumonic, 26 transudates, 14 miscellaneous, and 30 of unknown nonneoplastic origin). The levels of CA 72-4, CEA, CA 15-3, and CA 19-9 were measured in pleural fluid by radioimmunoassay. RESULTS: CA 72-4 assay in pleural fluid had an acceptable sensitivity and very good specificity for diagnosing malignant pleural effusion. The combination of CA 72-4 plus CEA plus CA 15-3 yielded the best accuracy, 0.90 (95% confidence interval [CI] 0.85-0.94), with a sensitivity of 0.78 (95% CI, 0.67-0.88), specificity of 0.95 (95% CI, 0.90-0.98), positive predictive value of 0.88 (95% CI, 0.77-0.95), and negative predictive value of 0.91 (range, 0.85-0.94). A good clinical strategy may be to begin with a CEA assay (specificity of I) and then, if it is negative, to add CA 15-3 or even CA 72-4 assays to improve sensitivity. The diagnosis of mesothelioma is more likely with a high CA 15-3 level and normal CEA and CA 19-9 levels. CONCLUSIONS: Assays of CEA, CA 72-4, and CA 15-3 in pleural fluid, or the combination of CEA with CA 15-3 and CA 72-4, was useful in differentiating between pleural effusion of malignant and benign origin.

Combined use of novel epithelial (MOC-31) and mesothelial (HBME-1) immunohistochemical markers for optimal first line diagnostic distinction between mesothelioma and metastatic carcinoma in pleura.

AIMS: To determine the value of immunohistochemistry in differentiation of malignant pleural mesothelioma from carcinoma in a pleural biopsy we optimized a double panel of MOC-31 and HBME-1 and compared the results with others from the literature. METHODS AND RESULTS: A multi-antibody panel was applied to biopsy samples from 44 cases of malignant pleural mesothelioma and 23 cases of carcinoma metastatic to the pleura. We used monoclonal antibodies against keratins, epithelial membrane antigen (EMA), epithelial antigen Ber-EP4, carcinoembryonic antigen (CEA), tumour-associated glycoprotein (B72.3), LeuM1, vimentin, desmin, epithelial related antigen (MOC-31) and mesothelial cell (HBME-1). Positivity for MOC-31 and Ber-EP4 was found to have the highest nosologic sensitivity (94.1% and 84.6%, respectively) and specificity (86.3% both antibodies) for carcinoma. Positive staining for HBME-1 and vimentin had the highest sensitivity (90.9% and 100%, respectively) and specificity (91.3% and 60%, respectively) for mesothelioma. A two-marker antibody panel with HBME-1 and MOC-31 was the most efficient for the distinction between carcinoma and malignant pleural mesothelioma. CONCLUSION: A combination of MOC-31 (an anti- epithelial marker) and HBME-1 (an anti-mesothelial marker) has a diagnostic efficiency of 76.1% for the distinction between carcinoma and mesothelioma in pleura.

Interferon-gamma in 388 immunocompromised and immunocompetent patients for diagnosing pleural tuberculosis.

The level of interferon-gamma (IFN-gamma) in pleural fluid has been reported to be increased in pleural tuberculosis. Nevertheless, its diagnostic value has not yet been well-established, and immunocompromised patients have not previously been evaluated. The aim of this study was to determine the value of the IFN-gamma level in pleural fluid for diagnosing tuberculous pleurisy in immunocompetent and immunocompromised patients. Three hundred and eighty eight consecutive patients were studied prospectively (73 with tuberculous pleural effusions, including nine with concurrent human immunodeficiency virus (HIV) infection and one after liver transplantation, and 315 with nontuberculous effusions). IFN-gamma was measured by radioimmunoassay. The sensitivity of the test, using a 3.7 U.mL-1 cut-off point, was 0.99 (95% confidence interval (95% CI) 0.93-1.00) and the specificity was 0.98 (95% CI 0.96-1.00). The sensitivity of the test did not differ in HIV-positive and HIV-negative patients. Patients with lymphoma, vasculitis or vascular connective tissue disease did not have abnormal IFN-gamma values. In conclusion, the level of interferon-gamma in pleural fluid is a very good diagnostic marker of tuberculous pleural effusion, even in immunocompromised patients.

X-linked agammaglobulinaemia and squamous lung cancer.

A 32yr-old nonsmoking male, diagnosed as having X-linked agamma-globulinemia, presented with fever, cough with purulent sputum, a very intense back pain and a mass of 10 centimetres in lower left lobe. Diagnostic evaluation revealed a squamous cell carcinoma with very aggressive metastases at L3. Malignancies are the second leading cause of death in children and adults with congenital immunodeficiency disorders, mostly non-Hodgkin lymphomas and gastric and colon adenocarcinomas, but this is the first report of lung cancer in a patient with X-linked agammaglobulinemia. Lung cancer incidence has been reported to be higher in patients with other diseases of the lung, however, there is no clear evidence of the role of bronchiectasis in developing lung cancer. It is possible that a longer survival for patients with X-LA recently diagnosed, and an association of chronic bronchial infection, could favour the development of pulmonary neoplasm.

Rapid automated determination of adenosine deaminase and lysozyme for differentiating tuberculous and nontuberculous pleural effusions.

The catalytic concentration of pleural adenosine deaminase (ADA) and the ratio of pleural lysozyme (PL) to serum lysozyme (SL) were measured in consecutive patients (49 tuberculous and 179 nontuberculous) with two automated procedures in a Hitachi 717 analyzer. Using sensitivity and specificity curves, we established cutoff values at 33 U/L for ADA and 1.7 for the PL/SL ratio. The sensitivity of ADA activities for tuberculous effusion was 90%, specificity 85%. Combining ADA with the PL/SL ratio enhanced specificity to 99%. However, high values for ADA and lysozyme ratios are not, alone or in combination, sensitive or specific enough to replace pleural biopsy or culture of pleural fluid for the diagnosis of tuberculous empyema.