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Biomedical subjects

M B Rubens

Publications and source records attributed to M B Rubens.

At least 37 records · Page 2Linked to original sources
PubMed1999

Acute respiratory distress syndrome: CT abnormalities at long-term follow-up.

PURPOSE: To document abnormalities at computed tomography (CT) in adult survivors of acute respiratory distress syndrome (ARDS), to determine the relationships between CT patients during the acute phase and at follow-up, and to assess the effects of mechanical ventilation on the development of CT abnormalities. MATERIALS AND METHODS: Thin-section CT scans were obtained during the acute illness and at follow-up in 27 patients with ARDS. The extent and distribution of individual CT patterns were independently analyzed. RESULTS: At follow-up CT, a reticular pattern was the most prevalent (23 patients [85%]) and extensive CT abnormality, with a striking anterior distribution (more anterior distribution than posterior distribution, P < .001). A reticular pattern at follow-up was inversely correlated with the extent of intense parenchymal opacification on scans obtained during the acute illness (Spearman r = -0.26; P < .001). The extent of a reticular pattern at follow-up CT was independently related to the total duration of mechanical ventilation (P = .02) but was most strongly related to the duration of pressure-controlled inverse-ratio ventilation (P < .001). CONCLUSION: A reticular pattern, with a striking anterior distribution, is a frequent finding of follow-up CT in ARDS survivors and is most strongly related to the duration of pressure-controlled inverse-ratio ventilation.

Acute Disease↗
PubMed1998

Bronchoalveolar lavage cellularity: lone cryptogenic fibrosing alveolitis compared with the fibrosing alveolitis of systemic sclerosis.

Lone cryptogenic fibrosing alveolitis (CFA) is histologically identical to fibrosing alveolitis associated with systemic sclerosis (FASSc), but it has a much worse prognosis after matching for disease severity at presentation. The aims of this study were to gain insights into possible pathogenetic mechanisms contributing to this prognostic difference, by comparing bronchoalveolar lavage (BAL) cellularity in the two diseases, and to evaluate the relationships between BAL findings and the regional and global extent of disease, quantified by thin-section computed tomography (CT) and lung function indices. Patients with CFA were distinguished by more extensive fibrosing alveolitis on CT (p < 0.02) and by higher counts of neutrophils (total per ml, p < 0.02; percentage p < 0.03) and eosinophils (total per ml, p < 0.002; percentages, p < 0.02) in BAL fluid. After adjustment for functional and morphologic measures of disease extent, eosinophil percentages and total counts were increased in CFA (p < 0.05 in all 12 multivariate models), but they were not independently related to regional or global disease severity. Neutrophil percentages and total counts were virtually identical in CFA and FASSc in disease of comparable severity, and they increased with increasingly extensive lobar disease and global disease, as judged by CT, p < 0.0005 in all analyses. Neutrophil levels were more closely linked to the extent of disease on CT than to the severity of functional impairment, on univariate and multivariate analysis. The higher BAL eosinophil levels seen in CFA, compared with those seen in FASSc, after adjustment for disease extent, indicate that an eosinophilic influx may be linked to the pathogenesis of fibrosing alveolitis. By contrast, BAL neutrophil levels increase with increasingly extensive disease on CT, but they do not differ independently between CFA and FASSc, suggesting that neutrophil degradation products are unlikely to account for the excess mortality in CFA, compared with that in FASSc.

Bronchoalveolar Lavage Fluid↗
PubMed1997

Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography.

OBJECTIVE: Thin-section computed tomography (CT) provides a sensitive and reproducible method of quantifying the morphologic extent of disease in the clinical management of fibrosing alveolitis associated with systemic sclerosis (FASSc). The aim of this study was to determine which indices of lung function best reflect the extent of disease on CT in FASSc, and to determine the independent influences of smoking history, extent of fibrosing alveolitis, demographic features, and concurrent treatment upon functional impairment in FASSc. METHODS: Sixty-four patients with FASSc were studied using CT and static and exercise lung function testing. Statistical relationships were determined by multiple regression analyses. RESULTS: Five patients with overt pulmonary hypertension were characterized by severe impairment in 3 indices of lung function: diffusing capacity for carbon monoxide (DLCO), DLCO adjusted for alveolar volume (KCO), and arterial partial pressure of oxygen. On multiple regression analysis, the major determinant of functional impairment was the extent of fibrosing alveolitis on CT. A history of smoking was independently associated with preservation of total lung capacity and depression of KCO, but did not otherwise influence functional-morphologic correlations. The percent predicted DLCO correlated better with extent of disease on CT (r = -0.70) than did oxygen desaturation on exercise (r = 0.55), the physiologic component of the clinical-radiographic-physiologic score (CRP index) (r = 0.52), or other indices of lung function. Lung volume measures correlated poorly with disease extent on CT. CONCLUSION: The percent predicted DLCO best reflects the extent of fibrosing alveolitis in FASSc, and therefore should be measured in routine evaluations. Exercise testing may also have a useful role in staging the severity of pulmonary fibrosis, but the CRP index offers no additional advantage over the DLCO and exercise testing.

Carbon Monoxide↗
PubMed1997

Effects of airway infection by Pseudomonas aeruginosa: a computed tomographic study.

BACKGROUND: Pseudomonas aeruginosa commonly infects the airways of patients with bronchiectasis. A study was undertaken to examine the relationship between infection of the airways with this pathogen, the morphological pattern of bronchiectasis on thin section computed tomographic (CT) scanning, symptom duration, smoking habits of the patients, and the presence of airflow obstruction. METHODS: Thin section CT scans of 22 adult patients with bronchiectasis and concurrent sputum infected by P aeruginosa (Pa +ve) and those of 45 randomly selected patients not infected by P aeruginosa (Pa -ve) were analysed independently by two thoracic radiologists. Patients with cystic fibrosis were excluded. Each scan was scored at a lobar level for extent of bronchiectasis, severity of bronchial wall thickening and dilatation, predominant pattern of bronchiectasis, presence of mucus plugging, and degree of decreased attenuation of the lung parenchyma. RESULTS: The Pa +ve group had more extensive bronchiectasis and a greater degree of bronchial wall thickening and dilatation on the CT scan than the Pa -ve group; more extensive decreased attenuation was seen in the Pa +ve group. These findings were robust on multivariate analysis; decreased attenuation was also independently related to the duration of sputum production. CONCLUSION: Patients with bronchiectasis infected by P aeruginosa have more extensive and severe bronchiectasis on thin section CT scanning than those without P aeruginosa infection. The bronchi and small airways are both involved, reflecting the end result of complex interactions between host airways and the numerous virulence factors produced by P aeruginosa.

Adult↗
PubMed1997

Cryptogenic fibrosing alveolitis and the fibrosing alveolitis of systemic sclerosis: morphological differences on computed tomographic scans.

BACKGROUND: The purpose of this study was to identify morphological differences on the computed tomographic (CT) scan between cryptogenic fibrosing alveolitis (CFA) and the fibrosing alveolitis associated with systemic sclerosis (FASSc), and to examine their biological relevance. METHODS: One hundred and seven patients with CFA (n = 55) or FASSc (n = 52) who had undergone thin section CT scanning were included. Multivariate analysis was used to identify morphological differences on the CT scans between lone CFA and FASSc, and to determine whether the pattern and distribution of disease on the CT scans were functionally significant (as judged by the lung transfer factor (TLCO), forced vital capacity (FVC), and arterial oxygen tension (PaO2)) or predictive of survival (independent of the type and extent of fibrosing alveolitis, age, sex, and smoking history). RESULTS: Increasingly extensive disease on CT scans was associated with a coarser reticular pattern (increase in reticular score per percentage increase in disease extent = 0.06, 95% confidence interval (CI) 0.03 to 0.09, p < 0.0005) and increasing upper zone involvement (increase in ratio of upper zone to total disease per percentage increase in disease extent = 0.002, 95% CI 0.000 to 0.003, p < 0.04). Patients with CFA were characterised by a higher upper zone ratio (difference = 0.08, 95% CI 0.02 to 0.13, p < 0.004) and a weak trend towards a coarser reticular pattern (p = 0.09), independent of disease extent. Smokers with CFA had more upper zone involvement (difference = 0.11, 95% CI 0.05 to 0.16, p < 0.0005) and a coarser reticular pattern (difference in reticular score = 1.92, 95% CI 0.27 to 3.55, p < 0.02) than smokers with FASSc. The extent of disease on the CT scan was predictive of lung function impairment and survival but the pattern and distribution of disease were not. CONCLUSIONS: Patients with CFA have relatively more upper zone involvement than those with FASSc independent of the extent of disease on the CT scan. This finding may result from smoking related damage but is not functionally significant.

Diagnosis, Differential↗
PubMed1997

Obliterative bronchiolitis: individual CT signs of small airways disease and functional correlation.

PURPOSE: Individual features of small airways disease depicted at computed tomography (CT) were correlated with functional indexes in patients with obliterative bronchiolitis. MATERIALS AND METHODS: Fifteen patients (all women) who fulfilled the strict criteria for diagnosis of obliterative bronchiolitis underwent thin-section CT at full inspiration and at end expiration. The CT scans were scored by two observers for extent of decreased attenuation of the lung parenchyma; end-expiration CT signs of air trapping; and bronchial dilation, wall thickening, and mucous plugging. The functional importance of each CT sign was evaluated. RESULTS: Areas of decreased attenuation were present in all patients (median score at end expiration, 61%; range, 21%-83%). Bronchial wall thickening was identified in 13 of the 15 patients. Correlations of the extent of decreased attenuation and measures of air-flow obstruction were strongest between decreased attenuation at end expiration and air flow at low lung volumes (r(s) = -.70, P < .005). This relationship remained intact after correction for the severity of bronchial wall thickening. CONCLUSION: In patients with obliterative bronchiolitis, the extent of decreased attenuation at CT was most strongly related to depression of pulmonary function tests of the small airways. Decreased attenuation is the cardinal sign for further quantitative studies of obliterative bronchiolitis.

Adult↗
PubMed1997

Lone cryptogenic fibrosing alveolitis: a functional-morphologic correlation based on extent of disease on thin-section computed tomography.

In most clinical series of patients with cryptogenic fibrosing alveolitis (CFA), disease severity is staged using lung function indices. However, many physiologic indices are measured in routine clinical practice; the choice of variable to evaluate functional severity is contentious. Computed tomography (CT) provides a reproducible means of quantifying the morphologic extent of disease. The aim of this study was to evaluate the functional consequences of smoking-related lung damage in CFA and to identify functional measures best reflecting the extent of fibrosing alveolitis on CT. Sixty-eight patients with CFA were studied. Fourteen patients with emphysema on CT were characterized by relative preservation of FVC and TLC (p < 0.005) and relative depression of DLCO (p < 0.05) and KCO (p < 0.00005). On multivariate analysis, the extent of fibrosing alveolitis and the presence of emphysema were independent determinants of functional impairment; there was no independent relationship between smoking history and functional abnormalities. In patients without emphysema on CT, percent predicted DLCO (r = -0.68), oxygen desaturation on exercise (r = 0.64), and the physiologic component of the clinical-radiographic-physiologic (CRP) score (r = 0.62) correlated much better with the extent of disease on CT than spirometric and plethysmographic volumes. A composite functional index was generated against the extent of disease on CT, using multivariate analysis; comparison with the CRP score suggested that the relationship between morphologic disease extent and the CRP score would be improved by the inclusion of DLCO and by the use of negative weighting for depression of FEV1. These findings indicate that in CFA, the presence of concurrent emphysema on CT has a more profound influence upon functional measures than the smoking history, and underline the importance of both the measurements of DLCO and exercise testing in the assessment of the severity of CFA.

Case-Control Studies↗
PubMed1997

Functional impairment in lone cryptogenic fibrosing alveolitis and fibrosing alveolitis associated with systemic sclerosis: a comparison.

Lone cryptogenic fibrosing alveolitis (CFA) is histologically identical to fibrosing alveolitis associated with systemic sclerosis (FASSc), but it has a much worse prognosis after matching for disease severity at presentation. Thin-section CT scanning (CT) provides a reproducible method of quantifying the morphologic extent of fibrosing alveolitis. The aim of this study was to gain insights into contrasting pathophysiologic mechanisms in the two diseases by comparing patterns of functional impairment after matching for extent of disease on CT, demographic factors, smoking history, and concurrent treatment. Patients with emphysema on CT (n = 16) and patients with FASSc with overt pulmonary hypertension (n = 5) were excluded; 111 patients were studied (CFA, n = 54; FASSc, n = 57). Patients with CFA were distinguished by more severe functional impairment and more extensive disease on CT (40.1 versus 22.1%, p < 0.00005). On multivariate analysis, patients with CFA had greater reduction in arterial P(O2) (p < 0.0005), wider AaP(O2) (p < 0.0005), greater oxygen desaturation on maximal exercise (p < 0.03), and higher dyspnea scores (p < 0.02) than did patients with FASSc after controlling for extent of disease on CT and other covariates. Measures of lung volume and gas transfer did not differ independently between CFA and FASSc. These findings persisted in subanalyses of patients with limited disease, extensive disease, histologic confirmation of fibrosing alveolitis, and with the reinclusion of patients with emphysema and pulmonary hypertension. The patterns of functional impairment were indicative of more severe ventilation-perfusion mismatch or anatomic shunting in CFA after adjustment for disease extent; we speculate that perfusion of poorly ventilated lung parenchyma in CFA occurs through new vessels formed in areas of intense inflammation. This mechanism may contribute to the greater mortality of patients with CFA than of patients with FASSc because of the deleterious effects of hypoxia on concurrent cardiac disease.

Female↗
PubMed1997

Functional impairment in fibrosing alveolitis: relationship to reversible disease on thin section computed tomography.

Thin-section computed tomography (CT) provides a reproducible method of quantifying global disease extent and can also discriminate between fibrotic disease, with predominance of reticular abnormalities, and reversible inflammatory cell infiltration, shown as ground-glass attenuation. The aim of this study was to determine whether functional impairment varied according to the presence of ground-glass attenuation on CT, independently of extent of disease on CT, demographic factors, smoking history, therapeutic status, and the type of fibrosing alveolitis (lone cryptogenic fibrosing alveolitis (CFA) versus fibrosing alveolitis associated with systemic sclerosis (FASSc)). Patients with concurrent emphysema on CT (n = 16) and FASSc patients with end-stage pulmonary hypertension (n = 5) were excluded. One hundred and eleven patients were studied (CFA, n = 54; FASSc, n = 57). The severity of functional impairment did not vary independently with the presence of predominant ground-glass attenuation, mixed appearance and predominant reticulation on CT. In 34 treated patients undergoing serial CT scanning, the severity of functional impairment did not differ independently between patients with and without regression of ground-glass attenuation at follow-up. We conclude that the severity of functional impairment does not discriminate between inflammatory and fibrotic disease in fibrosing alveolitis, as judged by initial and serial computed tomographic scanning, after adjustment for the morphological extent of disease on computed tomography.

Female↗
PubMed1995

Accuracy of CT in predicting the cause of bronchiectasis.

There are many reports that state that the distribution and pattern of bronchiectasis may be sufficiently characteristic for a specific cause to be suggested. The aim of this study was to determine whether experienced chest radiologists could confidently and accurately diagnose various aetiologies of bronchiectasis from the computed tomography (CT) pattern of disease alone. CT scans of 108 patients with bronchiectasis of various causes (67 with idiopathic bronchiectasis, 10 with allergic bronchopulmonary aspergillosis, 12 with syndromes of impaired mucociliary clearance, 12 with hypogammaglobulinaemia and seven with adult cystic fibrosis) were assessed by three chest radiologists without knowledge of clinical data. Each observer listed the three most likely diagnoses in order of probability. In addition, a level of confidence on a 3-point scale was assigned to the first choice diagnosis. A correct first-choice diagnosis was made in 45% of readings. A high confidence level was given in only 9% of the first choice readings. Of these, a correct diagnosis was reached in 35%. There was poor interobserver agreement (mean kappa = 0.20). In conclusion, we found that the causes of bronchiectasis cannot be reliably diagnosed on the basis of CT appearances alone.

Adult↗
PubMed1994

Observer variation in pattern type and extent of disease in fibrosing alveolitis on thin section computed tomography and chest radiography.

In fibrosing alveolitis the pattern type on thin section computed tomography (CT) predicts histological appearances at open lung biopsy and the likelihood of response to treatment. To test the level of inter- and intra-observer variability on CT and chest radiography (CXR), the pattern type and extent of disease were assessed by four observers (two experienced, two inexperienced). A total of 126 CT examinations and 108 concurrent postero-anterior chest radiographs were scored on two occasions, at least 8 weeks apart. A confidence rating was assigned to each observation. Three out of four observers agreed on pattern type in 81% of cases on CT compared with 54% on CXR (kappa coefficient 0.48 and 0.16 for CT and CXR, respectively). Inter-observer variability in categorizing pattern type on CT was lowest in patients with the highest confidence scores (kappa = 0.63). Confident observations were associated with extensive or moderately extensive disease (P < 0.001), and with a predominantly reticular pattern (P < 0.0001). Intra-observer variability for pattern type on CT was less for the experienced observers (kappa = 0.78 and 0.70) than for the inexperienced group (kappa = 0.50 and 0.37). Inter-observer variability for extent of disease was significantly less on CT than on CXR (standard deviations 7.8% and 9.2% respectively, P < 0.001). This study shows that observer variability using a clinical grading system is lower with CT than with chest radiography in fibrosing alveolitis.

Attitude of Health Personnel↗
PubMed1994

Intrapleural streptokinase in the management of empyema.

BACKGROUND: Significant morbidity and mortality result from the ineffective evacuation of empyema. Failure of conventional first line treatment with closed intercostal tube drainage and antibiotic therapy may result in fibrin deposition and loculated empyema. Enzymatic debridement using intrapleural instillation of streptokinase is a non-invasive therapeutic option which may obviate the need for surgical intervention. METHODS: Eleven adults with multiloculated post-pneumonic empyemas who had failed to respond satisfactorily to intercostal tube drainage and antibiotic therapy were treated with intrapleural streptokinase between November 1992 and January 1994. A small catheter was inserted under ultrasound guidance into a loculation within the pleural space. Aliquots of 250,000 units of streptokinase in 100 ml normal saline were instilled into the pleural cavity and the tube clamped for four hours. Response was assessed by clinical outcome, measurement of drain output after unclamping, and subsequent pleural ultrasound, chest radiography, or both. RESULTS: Streptokinase enhanced drainage in all patients. Complete resolution of the empyema with re-expansion of the underlying lung was effected in eight patients, all of whom remain well. Further resolution of minimal pleural thickening was shown on subsequent chest radiographs. Two patients with considerably thickened visceral pleura following empyema drainage underwent successful decortication. The other, with myocarditis and a pyopneumothorax, underwent surgery for non-resolution of the pneumothorax but died perioperatively from cardiac failure. The number of streptokinase instillations per patient ranged from two to six (median three), and the volume of empyema fluid drained per patient ranged from 100 ml to 4870 ml (median 900 ml). Streptokinase was well tolerated in all patients. CONCLUSIONS: Intrapleural streptokinase is an effective adjunct in the management of complicated empyema and may reduce the need for surgery.

Adult↗
PubMed1994

Bronchiectasis: functional significance of areas of decreased attenuation at expiratory CT.

PURPOSE: To characterize areas of decreased attenuation on expiratory computed tomographic (CT) scans in patients with bronchiectasis. MATERIALS AND METHODS: CT scans were obtained at full inspiration and end expiration in 70 patients with chronic purulent sputum production. Inspiratory scans were scored at a lobar level for the extent of bronchiectasis, and inspiratory and expiratory scans were scored for the presence and extent of areas of decreased attenuation. Total lobar observations were analyzed (n = 840). RESULTS: Bronchiectasis was identified in 434 of 840 lobar observations. The total decreased attenuation score was related to extent of bronchiectasis (r = .49; P < .001), independent of functional indexes. Negative correlations were found between forced expiratory volume in 1 second (FEV1) and total decreased attenuation score (P < .002) and FEV1 to forced vital capacity ratio (P < .004) but not with indexes of gas transfer. CONCLUSION: Areas of decreased attenuation on expiratory CT scans are common in severe bronchiectasis. Such areas in lobes without overt bronchiectasis suggest that small airways disease may precede the development of bronchiectasis.

Adolescent↗
PubMed1994

Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis.

Fibrosing alveolitis associated with systemic sclerosis (FASSc) is considered to be histologically and radiologically indistinguishable from lone cryptogenic fibrosing alveolitis (CFA). To date, the natural history of the two diseases has not been compared directly in large groups of patients followed at a single institution. We evaluated the survival of 205 patients with CFA and 68 patients with FASSc. Deaths during the follow-up period were reported in 142 patients with CFA (70%) and in 11 patients with FASSc (16%). Unadjusted survival from the onset of dyspnea was less in CFA than in FASSc (odds ratio, 3.3; p < 0.001); this difference persisted after adjustment for age of onset of dyspnea and smoking history. Survival from presentation was less in CFA (odds ratio approximately 3.3) after adjustment for age of presentation, smoking history, and initial pulmonary function indices. These differences persisted when analysis was confined to patients with histologic confirmation of fibrosing alveolitis and after adjustment for treatment. In patients undergoing computed tomography (CT) of the lungs, survival was less in CFA after adjustment for extent of abnormal lung on CT and CT pattern of disease (odds ratio, 3.9; p < 0.001). These findings indicate that despite their histologic and radiologic similarities, lone CFA and FASSc have different courses.

Adult↗
PubMed1994

Fibrosing alveolitis in systemic sclerosis. Bronchoalveolar lavage findings in relation to computed tomographic appearance.

Fibrosing alveolitis in systemic sclerosis is histologically identical to lone cryptogenic fibrosing alveolitis (CFA) (idiopathic pulmonary fibrosis). The inflammatory cell content of bronchoalveolar lavage (BAL) samples has been used as a guide to prognosis and treatment in CFA. In this study, the relationship was explored between BAL findings and the extent and pattern of disease within the lavaged lobe, as judged by thin-section computed tomography (CT), in systemic sclerosis. Thirty-eight nonsmoking patients were studied; none had been treated with corticosteroids or immunosuppressive agents, and 11 had no evidence of fibrosing alveolitis on CT. BAL neutrophils were markedly increased in association with extensive disease on CT compared with less extensive disease (p < 0.001) and normal appearances (p < 0.001); the extent of a reticular pattern on CT (denoting fibrosis) correlated with the neutrophil percentage count (p < 0.005) and total neutrophil count/ml (p < 0.01). BAL eosinophils were increased in less extensive as well as in extensive disease when compared with lobes with a normal CT appearance (p < 0.01); eosinophil percentage counts but not total eosinophil counts/ml correlated with the extent of a ground-glass pattern on CT (through to denote inflammation) (p < 0.05). These findings indicate that in systemic sclerosis a BAL neutrophilia is generally associated with extensive fibrotic disease, whereas a BAL eosinophilia is often seen in less advanced disease, particularly when CT appearances suggest lung inflammation.

Bronchoalveolar Lavage Fluid↗
PubMed1993

Accuracy of the typical computed tomographic appearances of fibrosing alveolitis.

BACKGROUND: Open lung biopsy is often performed to confirm the diagnosis in patients with suspected fibrosing alveolitis. The superior sensitivity and specificity of high resolution computed tomography (CT) over chest radiography in various diffuse lung diseases suggest that the characteristic appearance of fibrosing alveolitis on high resolution CT might render biopsy confirmation unnecessary. METHODS: The chest radiographs and high resolution CT scans of 86 patients (41 with fibrosing alveolitis and 45 with various other diffuse lung diseases) were examined individually and independently by two observers. No clinical information was given and the observers gave a level of confidence when the diagnosis was thought to be fibrosing alveolitis. RESULTS: The observers correctly and confidently discriminated between fibrosing alveolitis and other diffuse lung diseases on high resolution CT with an accuracy of 88% and on chest radiography with an accuracy of 76%. The false negative rate for fibrosing alveolitis diminished from 29% on chest radiography to 11% on high resolution CT. The false positive rate on chest radiography was 19% and on high resolution CT 13%; the false positive diagnoses on CT were the result of a few conditions (extrinsic allergic alveolitis, sarcoidosis, cryptogenic organising pneumonia, and pulmonary eosinophilia) which mimicked some of the CT features of fibrosing alveolitis. The superficial similarity of the CT patterns of these conditions are discussed. CONCLUSIONS: High resolution CT is superior to chest radiography in establishing the diagnosis of fibrosing alveolitis and the typical CT appearances are virtually pathognomonic. The diagnostic advantages of CT over chest radiography should further reduce the need for open lung biopsy in this condition.

Biopsy↗
PubMed1993

The predictive value of appearances on thin-section computed tomography in fibrosing alveolitis.

Fibrosing alveolitis, a condition characterized by a poor prognosis and a limited response to treatment, is readily identified by thin-section computed tomography (CT). Fibrotic and inflammatory histologic results, obtained at open lung biopsy, both have characteristic CT patterns. To evaluate whether CT could predict prognosis and response to therapy, we examined the CT appearances of 76 patients with lone cryptogenic fibrosing alveolitis and 66 patients with fibrosing alveolitis associated with systemic sclerosis. CT abnormalities were categorized as predominantly a ground-glass pattern (Grade 1), mixed (Grade 2), or predominantly a reticular pattern (Grade 3). In cryptogenic fibrosing alveolitis, 4-yr survival was highest in association with CT Grade 1 and higher with CT Grade 2 than with CT Grade 3, independent of the extent of abnormal lung on CT, duration of dyspnea, or severity of depression of DLCO or FVC, p < 0.001. A response to therapy in previously untreated cryptogenic fibrosing alveolitis was seen most frequently with CT Grade 1 and more frequently with CT Grade 2 than with CT Grade 3, p < 0.025. In systemic sclerosis, CT appearances were not predictive of 4-yr survival; a response to therapy was seen more frequently with CT Grade 2 (three of seven patients) than with CT Grade 3 (zero of six patients). These data have shown that CT appearances are of prognostic value in fibrosing alveolitis and that they are likely to play an increasing role in disease-staging in this condition.

Adult↗