Clinical value of mediastinoscopy in the diagnosis of sarcoidosis: an analysis of 68 cases.

Mediastinoscopy was performed for confirmation of the diagnosis in 68 patients who were suspected clinically and radiologically of having sarcoidosis. In 66 of 68 cases in which mediastioscopy was performed a diagnosis was attained. In 35 cases, endobronchial biopsy was performed by bronchoscopy. In only 5 of these (14.2 %) was the diagnosis of sarcoidosis confirmed. The sensitivity of mediastinoscopy was remarkably superior compared with that of endobronchial biopsy. No complication developed with either mediastinoscopy or endobronchial biopsy. In Turkey, mediastinoscopy without any complication costs about 650 USD while bronchoscopy and endobronchial biopsy cost about 150 USD. In our study in which we looked for a histological confirmation -- in the cases suspected of sarcoidosis -- mainly through mediastinoscopy and rarely through other methods (i.e., endobronchial biopsy in one case, skin biopsy in another), we did not come up with a different diagnosis. Therefore, patients suspected of having sarcoidosis should undergo a careful clinical, laboratory, and radiologic examination; they should be under continuous close observation; when necessary (e.g., skin and lip biopsy), the tissue diagnosis should be made by other methods, but if there is the possibility of a disease such as tuberculosis and lymphoma, mediastinoscopy should be performed. The diagnosis of stage 3 sarcoidosis is difficult. For diagnosis, sometimes videothoracoscopy or explorative thoracotomy may be necessary. However, in all our 3 cases with stage 3, we reached the diagnosis of sarcoidosis by the less invasive and less expensive method of mediastinoscopy. Despite our small number of cases, we believe that mediastinoscopy is a very important instrument for diagnosis of stage 3 sarcoidosis.

Löfgren syndrome in Turkey.

In the 36-year period between 1966 and 2002, 514 patients were diagnosed with sarcoidosis at Cerrahpasa Medical Faculty, Istanbul, Turkey, and of these 98 (19.1%) had Löfgren syndrome. The frequency of female patients with Löfgren was higher than the frequency among other sarcoidosis patients (female:male ratio 4.8 vs. 1.64; P < 0.001). Erythema nodosum was diagnosed in 72.4% of the subjects and arthritis or arthralgia was diagnosed in 51%. Erythema nodosum and arthritis or arthralgia were more frequent in Löfgren; however, pulmonary parenchymal involvement was more frequent in other sarcoidosis patients (all P-values < 0.001).

Miliary tuberculosis: clinical manifestations, diagnosis and outcome in 38 adults.

OBJECTIVE: The aim of the study was to determine the clinical, radiographic and laboratory characteristics, diagnostic methods, and prognostic variables in patients with miliary tuberculosis (TB). METHODOLOGY: The records of 38 patients (15 male, 23 female; mean age 41 years, range 16-76 years) with miliary TB from 1978 to 1998 were analyzed. Patients were evaluated also as to whether they presented with a fever of unknown origin (FUO). Criteria for the diagnosis of miliary TB were (i) miliary pattern on chest X-ray or (ii) biopsy or autopsy evidence of miliary organ involvement. Paraffin-embedded tissues with granulomata (n = 15) were re-evaluated for the presence of Mycobacterium tuberculosis DNA by polymerase chain reaction (PCR). RESULTS: Predisposing conditions were present in 24% of the patients. The findings were fever, weakness, night sweats, anorexia/weight loss (100% for each), hepatomegaly (37%), splenomegaly (32%), choroidal tubercles (13%), neck stiffness (11%), altered mental status (8%), anaemia (76%), leukopenia (26%), thrombocytopenia (16%), lymphopenia (76%), pancytopenia (8%) and hypertransaminasemia (55%). Eighteen patients (47%) met the criteria for a FUO. Miliary infiltrates were found on chest X-rays of 32 of 38 cases (84%). In six cases without miliary infiltrates, the diagnosis was made by laparotomy in four cases, and autopsy in two cases. Tuberculin skin test was positive in 32% of cases. Acid-fast bacilli were demonstrated in 37% (16/43), and cultures for M. tuberculosis were positive in 90% (9/10) of tested specimens (predominantly sputum and bronchial lavage). Granulomas were found in 85% (11/13) of lung, 100% (15/15) of liver, and 56% (9/16) of bone marrow tissue specimens. Acid-fast bacilli staining was negative in all (0/21), while PCR was positive in 47% (7/15) of specimens with granulomata. Mortality was 18%. Stepwise logistic regression identified male sex (P = 0.005), non-typical miliary pattern (P = 0.015), altered mental status (P = 0.002) and failure to treat for TB (P = 0.00001) as independent predictors of mortality. CONCLUSIONS: Miliary infiltrates on chest X-ray or FUO should raise the possibility of miliary TB. Therapy should be administered urgently to prevent an otherwise fatal outcome.

Results of hepatitis B vaccination in sarcoidosis.

BACKGROUND: Sarcoidosis is known to be associated with defects in cellular immunity, especially in reference to T helper lymphocytes. Anergy to a tuberculin skin test is most characteristic of this disease. OBJECTIVES: To further the data on impaired immunity, we studied the antibody response to hepatitis B vaccination in patients with sarcoidosis. METHODS: Serologic markers of hepatitis B virus (HBV) (HBsAg, anti-HBs, anti-HBc) were studied in 40 patients with sarcoidosis (32 female, 8 male; mean age: 45 +/- 11 years, range: 25-66 years) with a mean duration of disease of 6 years. While all the markers were negative in 22 patients (55%), 2 had isolated anti-HBc positivity and 16 had both anti-HBc and anti-HBs antibodies. Thirty-five age- and sex-matched healthy subjects were studied as controls. Recombinant HBV vaccines (Genhevac B Pasteur, 20 microg) were administered (at 0, 1, and 6 months) to 16 of the seronegative cases and the controls and antibody titres were measured 1 month after the last dose. The tuberculin skin test was negative in all cases. RESULTS: While none of the vaccinees in the diseased group responded, the control group yielded an antibody response rate of 85. 7% (30/35), with a mean titre of 257.9 mIU/ml. CONCLUSIONS: Patients with sarcoidosis were invariably unresponsive to standard vaccination, while some of the diseased subjects had already mounted a natural antibody response, either before or after the development of the original disease. Cellular immunodeficiency in sarcoidosis could be a suitable model for studying immunological interactions between HBV and the host.

Direct injection of anti-cancer drugs into endobronchial tumours for palliation of major airway obstruction.

Patients with tracheal or major airway obstruction due to inoperable carcinomas are at a high risk of developing respiratory failure or post-obstructive pneumonia, or both. This often leads to death in days or weeks. In such cases there is usually an urgent need to restore the airway. This report details the short-term results and techniques used for the treatment of airway obstruction by direct intratumoural injection of several anti-cancer drugs. A total of 93 patients with nearly complete extrinsic obstruction of at least one major airway were treated by injection of anti-cancer drugs directly into the endobronchial tumours or infiltrated bronchial mucosa through a flexible fiber-optic bronchoscope. At every session of treatment 1-3 ml each of 50 mg/ml 5-fluorouracil, 1 mg/ ml mitomycin, 5 mg/ml methotrexate, 10 mg/ml bleomycin and 2 mg/ml mitoxantrone were injected separately at different sites without pre-mixing. Local intratumoural chemotherapy relieved the obstruction in 81 of the 93 patients. Endoscopically visible tumours were reduced in size, and infiltrative changes were also improved. Obstruction was not relieved in 12 patients. The therapy was well tolerated and had no systemic side-effects, and no serious complications. Intratumoural chemotherapy can be considered a new life-saving palliative method in patients with life-threatening airway obstruction.

Frequency of distribution according to histological types of lung cancer in the tracheobronchial tree.

The incidence of the location within the bronchi related to the cell types was investigated with the flexible fiberoptic bronchoscope in 355 cases of lung carcinoma. In 5 patients carcinoma was situated only in the trachea. In the other 350 cases the cell types other than adenocarcinoma were found to show different locations following their cell type. Epidermoid carcinoma was found more frequently in the two upper lobes (p less than 0.001), while small cell carcinomas showed predilection for the main bronchus on the right side, and the upper lobe in the left (p less than 0.001). No difference could be found between the upper, lower lobes and main bronchi for adenocarcinoma. It was also observed that large cell carcinomas were situated more often in the right upper lobe. The most important finding in this investigation was that, apart from adenocarcinoma, the other types were located mainly in the upper lobes, and much less frequently in the lower lobes. The predilection of localization of epidermoid and small cell carcinomas in the upper lobes suggests a possible relationship to tobacco smoke inhalation as these regions have been shown to be more affected by the smoke.

Renal amyloidosis: immunofluorescence and electron microscopy studies.

Renal biopsy specimens of 15 patients with renal amyloidosis were studied by immunofluorescence microscopy. The amyloidosis was associated with chronic pulmonary disease in five, rheumatoid arthritis in one, chronic lymphocytic leukemia in one, and familial Mediterranean fever in five patients. In three patients no associated condition could be determined although the pattern of organ involvement resembled that of secondary amyloidosis. IgG and complement (C3) were demonstrated in the glomerular capillary walls and in the mesangium in all patients. The pattern of the deposits was neither granular nor linear. Ig and C3 appeared as large confluent masses or broad ribbon-like segments. In the six patients studied by electron microscopy the fibrillary formation of amyloid was seen in the mesangium and the glomerular capillary walls corresponding to the Ig deposits. No immunofluorescence or ultrastructural differences were observed among the patients with secondary, inherited and leukemia-associated amyloidosis included in this study.

Acute glomerulonephritis. Immunofluorescent and electron-microscopic observations in sporadic cases.

The authors studied by immunofluorescent and electron microscopy renal biopsy specimens from 29 patients with various glomerular diseases. Poststreptococcal glomerulonephritis was characterized by the presence of complement (beta1C) in the mesangium and/or on the basement membrane in all cases. Immunoglobulin G (IgG) was also present in less than half of the cases in the same distribution. Electron microscopy, carried out in six cases, revealed no uniform ultrastructural change: minimal subepi helial deposits were observed in three cases, intramembranous deposits were seen in two cases, and the basement membrane was normal in one case. Glomerular abnormalities during the courses of some systemic diseases were similar. Mild renal involvement was characterized by only beta1C deposition. This finding raises the question whether a mechanism other than or in addition to that involving immune complexes is operative in the pathogenesis of acute glomerulonephritis. There is circumstantial experimental evidence that an alternate pathway of complement activation and deposition may be operative in acute glomerulonephritis.