Searching for early predictive markers of the therapeutic effects of high-dose corticosteroids ("pulse therapy") on patients with rapidly progressing idiopathic pulmonary fibrosis (IPF), we evaluated 14 such patients, who had received weekly pulse therapy for at least 3 wk. Eight patients responded to the treatment and survived. However, six patients failed to respond, and all of them died within 3 mo after treatment. Serum levels of KL-6 (MUC1 mucin), neutrophil elastase (NE), and lactate dehydrogenase (LDH) were measured before, and at 1 wk and 3 wk after treatment. Levels of KL-6 decreased significantly in patients who lived, whereas KL-6 levels tended to increase in patients who died. The values of NE did not change significantly. LDH levels decreased significantly at 1 wk, and tended to decrease at 3 wk in patients who lived. However, in patients who died, they did not significantly change. At the first cycle of treatment when clinical effects may not be evident, the decrease in KL-6 but not LDH levels was significantly related to a favorable outcome, whereas their increase was related to a poor outcome. Results suggest that monitoring with KL-6 may contribute to early clinical decisions for alternative therapy in the management of rapidly progressing IPF.

Low-dose therapy with corticosteroids does not suppress alveolitis sufficiently to prevent continued deterioration of the alveolar structures.

Abstracta better prognosis and a better response to treatment with corticosteroids than patients Background -Desquamative interstitial pneumonia (DIP) may represent the early with UIP. However, other observers 3 4 have suggested that DIP may represent an early stage stage and usual interstitial pneumonia (UIP) the late stage of the same disease. of idiopathic pulmonary fibrosis which subsequently progresses to UIP. The morphology The purpose of this study was to evaluate the computed tomographic (CT) features of both may appear in the same patient at the same time, and although in one study patients of DIP, to evaluate the changes in pattern and extent of disease over time, and to with DIP treated with corticosteroids were found ultimately to develop extensive fibrosis determine whether the appearances of DIP on the CT scan change to those of and honeycombing, 5 others 6 7 have suggested that DIP does not necessarily progress to UIP UIP during follow up. Methods -Sequential CT evaluation was and can relapse many years after the initial presentation with a similar histological picture. conducted on eight patients with DIP over a mean (SD) follow up period of 3.2 (1.3)Computed tomographic (CT) scanning has an established role in the evaluation of idioyears (range 1.6-6.5). The relative extents of ground glass and honeycombing were pathic pulmonary fibrosis.8 9 On the CT scan DIP is characterised by patchy areas of ground determined from serial CT scans. Changes in the extent and appearance of the disease glass opacification, predominantly on the periphery of the lung. 10 It has recently been sugwere examined in paired anatomically comparable CT sections.gested that the greater extent of ground glass attenuation and the paucity of cystic changes Results -Common features on the CT scans of patients with DIP were a homo-in patients with DIP should enable it to be distinguished from UIP. 11geneous increase in lung attenuation (n= 5), linear areas of attenuation (n=5), rel-UIP is easily distinguished from DIP by the presence of honeycomb cysts.10 However, cystic atively well preserved lung architecture (n=5), and the presence of small cysts (n= changes may also be seen but are less prominent in DIP. 11 DIP should change to UIP during 6). Uncommon features were architectural distortion (n=3) and traction bron-follow up if DIP represents the early stage and UIP the late stage of the same disease. chiectasis (n=1). In six patients with DIP with cystic spaces these did not changeThe purpose of this study was to assess the CT features of DIP, to evaluate the changes of with time in three cases, in two they regressed, and in one patient they increased. the pattern and extent of the disease with time, and to determine whether the CT appearances Open lung biopsy samples from patients with DIP with many cystic lesions showed of DIP change to those of UIP during follow up. dilated alveolar ducts and bronchioles and/ or pulmonary cysts, as well as numerous macrophage-filled air spaces and mild

Immunoglobulin G (IgG) antibodies against purified cord factor (trehalose-6,6'-dimycolate) prepared from Mycobacterium tuberculosis H37Rv were determined by enzyme-linked immunosorbent assay (ELISA), and its diagnostic usefulness was evaluated. Serum specimens from 65 patients with active pulmonary tuberculosis, 58 patients with inactive pulmonary tuberculosis, 36 patients with diseases other than tuberculosis, and 66 healthy adults were examined. Patients with active pulmonary tuberculosis showed significantly higher titers of IgG antibodies against cord factor than did other groups (p < 0.001). The antibody titer greater than 0.29 in absorption difference (492 to 630 nm) of 160-times diluted serum was set as positive in ELISA. For patients with active and untreated pulmonary tuberculosis, the ELISA had a sensitivity of 81% and a specificity of 96%. From these results, it was concluded that the detection of IgG antibodies against cord factor is useful for serodiagnosis of active pulmonary tuberculosis. It was also indicated that the anticord factor antibody titers decline to the normal level as a result of antituberculosis chemotherapy.