Relationship among antigen contact, alveolitis, and clinical status in farmer's lung disease

We studied 41 subjects with a history of farmer's lung disease who had been free of acute episodes for at least one year. Twenty-six were still in daily contact with hay (group 1), and 15 had ceased all antigenic exposure (group 2). While the incidence of dyspnea was similar in both groups, coughing and sputum were more frequent in group 1. Inspiratory crackles were frequent in group 1 subjects (15 of 26) and absent in all group 2 subjects. In both groups, a high percentage of lymphocytes was demonstrated by bronchoalveolar lavage: 52.5% +/- 21.1% (mean +/- SD) and 26.3% +/- 18.7%, respectively. Lymphocytic alveolitis (greater than 22% lymphocytes) was more common in group 1 (23 of 26) than in group 2 (6 of 15). There was no relationship between functional abnormalities and the intensity of the alveolitis. We conclude that lymphocytic alveolitis may persist after an acute episode of farmer's lung disease, but the intensity of the alveolitis is not associated with functional alterations.     

Bronchoalveolar mast cells in normal farmers and subjects with farmer's lung. Diagnostic, prognostic, and physiologic significance

To evaluate the specificity and significance of increased lavage mast cells in farmer's lung, we examined the lavage cell differentials of 89 farmers and 19 normal nonfarming control subjects. The farmers were divided into four groups: acute farmer's lung (n = 17), farmers with one or more prior episodes of farmer's lung who remained in daily contact with hay (n = 26) or quit farming (n = 14), and normal farmers (n = 36). A total of 14 of the subjects with prior episodes of farmer's lung and still farming and 15 normal farmers were evaluated twice at a 2-yr interval. The lavage mast cell numbers were significantly higher in acute farmer's lung (7.5 +/- 7.3 x 10(3)/ml, mean +/- SD) and ex-farmer's lung who were still farming (1.2 +/- 1.3 x 10(3)/ml) than in normal farmers (0.1 +/- 0.1 x 10(3)/ml) (p less than 0.01). A total of 8 of 14 exfarmer's lung patients who had quit farming and 18 of 36 normal farmers had an increased number of mast cells in lavage, but mast cell count never exceeded 0.5% of total recovered cells. In the acute farmer's lung and ex-farmer's lung-still farming groups, the mast cell count correlated with the lymphocyte count: r = 0.83 and r = 0.69 (p less than 0.001), respectively. In the two groups evaluated twice, mast cell numbers at the first study did not correlate with changes seen at the second study in chest roentgenogram and pulmonary functions. We conclude that an increase in lavage mast cells occurs commonly as a part of the immune response against thermophilic bacteria.(ABSTRACT TRUNCATED AT 250 WORDS)     

Prognostic significance of bronchoalveolar lymphocytosis in farmer's lung

In 1983, we studied and reported the clinical and bronchoalveolar lavage (BAL) data of 26 patients with a history of previous farmer's lung. Most of these subjects (24 of 26) had a BAL lymphocytosis. In 1985, we restudied 14 of these subjects. Each subject had a clinical evaluation, chest roentgenogram, pulmonary function tests, and bronchoalveolar lavage. Results show that only 1 subject had evidence of an episode of acute recurrent disease, and only 1 had clinically progressive disease; however, 6 complained of chronic cough and sputum production. There were some fluctuations in individual pulmonary functions, with a significant decrease in FVC during the 2-yr period. The total number of cells on repeated lavage was 55.20 X 10(6) +/- 41.10 X 10(6) (mean +/- SD). This was not statistically different from the results obtained 2 yr earlier (69.5 X 10(6) +/- 43.01 X 10(6)). The percentage of BAL lymphocytes was also similar between the 2 studies, with abnormal values (greater than 20%) in 13 of 14 subjects (47.2 +/- 19.2). The BAL lymphocyte subsets, measured by monoclonal antibody in 8 subjects, showed a wide range of values, with individual OKT4/OKT8 ratios remaining similar at both studies. There were no correlations between changes seen in individual pulmonary function and BAL lymphocytosis. We conclude that a high intensity lymphocytic alveolitis, which persists after an acute episode of farmer's lung, does not predict the outcome of the disease.     

Association between neutrophil concentration in bronchoalveolar lavage fluid and recent losses in diffusing capacity in men formerly exposed to asbestos.

It has been observed widely that some individuals exposed to asbestos will experience continued losses of lung function after asbestos exposure ceases. Unfortunately, there are few data on factors that determine clinical course, limiting the clinician's ability to determine prognosis in an individual case and restricting the possibility for testing or targeting any potential intervention to alter the course among the millions at risk. In an attempt to address this question, we studied a volunteer population of 50 such men from among a stable, heterogeneous population of asbestos-exposed workers who had been continuously followed in our occupational medicine clinics for up to 12 years (mean, 6.3 years); most had some clinical or roentgenographic sign of asbestos effect, pleural or parenchymal. Each subject was reexamined clinically, functionally, and roentgenographically. Asbestos and tobacco exposure histories were carefully reviewed with the subjects and quantified based on these reports and available data regarding the various work environments from which they came. Subsequently, each underwent a bronchoalveolar lavage to assess cellularity and levels of various proteins. The levels of risk factors, clinical findings, and biologic parameters from lavage were examined for their relationship to serial changes in lung function during the period over which they had been previously followed. Results of the study demonstrate that serial changes in lung function were not closely related to level or length of prior exposure, smoking behavior, chest roentgenographic findings, or lung volumes. Progressive loss of diffusing capacity for carbon monoxide (Dco) was significantly associated with two factors: level of neutrophil concentration in lavage fluid (0.043 +/- 0.016 ml/min/mm Hg/yr drop for each 0.1 x 10(4) neutrophils per milliliter, p = 0.02) and the level of Dco itself (0.17 +/- 0.07 ml/min/mm Hg/yr drop for each 10 percent decrease in percent Dco predicted, p = 0.01). The relationship with neutrophil concentration was statistically independent of the association with Dco itself and stronger; it persisted when loss of Dco was adjusted for baseline value. Lung volume changes were not associated with any predictor variables, alone or in combination. We conclude that the presence of neutrophils in bronchoalveolar lavage fluid is associated with recent disease progression that may have implications in studies of the mechanisms of asbestos-associated disease and in clinical treatment of patients at risk.     

High-resolution computed tomographic characteristics in acute farmer's lung and in its follow-up.

High resolution computed tomography (HRCT) scans are increasingly used in hypersensitivity pneumonitis (HP). This study looked at HRCT findings at different phases of farmer's lung (FL), a common form of HP. A cross sectional analysis of 95 HRCT scans of FL cases (20 acute, 75 with a history of FL, 48 still in contact (Ex +) (dairy farm), and 27 who had ceased contact (Ex-)) was made. All scans were read independently by two, and if needed by three, radiologists blinded to the category. The lungs were divided into six regions (fives lobes + lingula), and read for attenuation/mosaic, ground-glass, micronodules, fibrosis, and emphysema. A score of 0-3 was given for each region and each variable: 0 = absence, 1 =<25% of the surface, 2 = 25-50%, 3 =>50%. Mediastinal lymphadenopathy was also noted. Ground glass, predominating in the lower lobes, was the most frequent feature in the acute and Ex+ cases. Other abnormalities had no preferential distribution. Ex+ had more ground-glass than the Ex- (p=0.0025). Emphysema was more frequently seen than interstitial fibrosis (p=0.004). Mediastinal lymphadenopathy was present in 26 cases (9 acute, 10 Ex+ and 7 Ex-). In conclusion, in farmer's lung: 1) ground-glass predominates in the lower lobes while the other abnormalities have no anatomic predilection; 2) contact avoidance allows a better resolution of computed tomography abnormalities than continued exposure; 3) emphysema is a more frequent finding than interstitial fibrosis; and 4) the presence of mediastinal lymphadenopathy has no negative diagnostic value.     

Persistent bronchoalveolar lymphocytosis in asymptomatic farmers

We previously demonstrated that 14 of 23 asymptomatic dairy farmers with positive precipitins and 5 of 20 without precipitins had an alveolar lymphocytosis (greater than 22%). To verify the outcome of this lymphocytosis, we restudied, 2 or 3 yr later, 27 of these subjects. No subject had had symptoms suggestive of farmer's lung disease either prior to the initial study or between the 2 studies. All were still on their farm. Physical examination and chest roentgenograms were normal for all subjects at both studies. Pulmonary function tests showed a small but significant change in RV (101.8 +/- 20.4% to 118.7 +/- 27.6% of predicted, p less than 0.05) and FVC (98.0 +/- 12.6% to 94.3 +/-9.5%, p less than 0.05), whereas TLC, FEV1, and DLCO showed no changes. Lymphocytes from BAL were still increased in 9 of 12 subjects, whereas 3 had returned to normal; of the 15 subjects with previous normal values, 3 now had an abnormal lavage lymphocytosis. There was no correlation between lung function variations and the percentage of lymphocytes in the previous or the present BAL. We conclude that a bronchoalveolar lymphocytosis is a persistent phenomenon in a large number of asymptomatic dairy farmers, and that this finding is not related to significant disease, at least in the time span studied.     

Small airways dysfunction in systemic sclerosis. A controlled study.

A debate exists regarding the importance of small airways disease in systemic sclerosis, while smoking seems to have a major effect on the exact prevalence. In order to evaluate small airways dysfunction (SAD) in a pure systemic sclerosis population, we performed pulmonary function studies in 31 nonsmoking patients and 31 age- and sex-matched nonsmoking control subjects. Patients' FVC, TLC, and Dco mean values were significantly lower compared with the corresponding values of the controls (p less than 0.05), while there was no difference in MEF25, RV, and RV/TLC. Seven (22.6 percent) of 31 patients and four controls (a nonsignificant difference) had evidence of SAD, namely a maximum expiratory flow at 25 percent of vital capacity (MEF25) less than 60 percent of predicted. Positive correlation (p less than 0.001) was found between MEF25 and FEV1/FVC in the patients. Moreover, no differences were found in abnormal lung function patients with and those without SAD in demographic, clinical, roentgenologic, and serologic features and results of pulmonary function tests. These findings suggest that SAD in our patients is not a characteristic and early manifestation of systemic sclerosis and that, when present, it is not correlated with the severity of the pulmonary involvement in scleroderma.     

Pulmonary function in young insulin-dependent diabetic subjects

To clarify the issue of pulmonary dysfunction in diabetes mellitus, lung mechanics and CO transfer were investigated in 22 young (mean age 19.5 +/- 5 years) non-smoking, insulin-dependent diabetic patients and an equal number of matched healthy subjects. Mean closing capacity/total lung capacity (CC/TLC) was significantly greater in the diabetic than in the control group (31.4 +/- 6.8 vs 27.2 +/- 2.9 percent, p less than 0.01), as was the mean value of the volume independent index of lung elasticity (exponent constant, Kst(L)) (0.148 +/- 0.045 vs 0.118 +/- 0.030, p less than 0.05). The transfer factor expressed per unit alveolar volume (TL/VA) was also significantly lower in the diabetic than in the control group (5.25 +/- 0.68 vs 5.61 +/- 0.57 ml/min/mm Hg/L, p less than 0.05) and this could be ascribed to a lower pulmonary capillary blood volume. There was evidence of mildly abnormal lung mechanics and/or a decreased pulmonary capillary blood volume in 16 (73 percent) of the diabetic group. Since pulmonary dysfunction was either an isolated non-endocrine finding or was associated with only early systemic complications in these young patients, our findings suggest that pulmonary dysfunction is an early measurable complication in insulin-dependent diabetes mellitus.     

Comparison of a hand-held nebulizer with a metered dose inhaler-spacer combination in acute obstructive pulmonary disease

This study compared the effect of "standard" dose metaproterenol delivered by hand-held nebulizer (HHN) with two puffs of metaproterenol delivered by a metered dose inhaler (MDI) via a spacer (InspirEase) (MDI-spacer). Seventeen patients with an acute exacerbation of obstructive pulmonary disease were studied. Each patient received both MDI-spacer and HHN. Alternate patients were randomized to either MDI-spacer or HHN as initial treatment. Each subject was tested four different times: before and 30 minutes after the initial aerosol delivery technique, and before and 30 minutes after the alternate aerosol delivery technique. Testing consisted of spirometry, lung auscultation, and measurement of vital signs. The interval between treatments for all subjects was 2.96 +/- 0.27 hours (mean +/- SEM) and was not different for subjects who received therapy via MDI-spacer first or HHN first. The patient population studied demonstrated severe airways obstruction (baseline FEV1 33.3 percent predicted +/- 4.9 percent). There was a statistically significant improvement in FVC and FEV1 after metaproterenol delivered by HHN, but not after MDI-spacer. Metaproterenol treatment with HHN resulted in a greater improvement in FEV1 (p less than .05) than MDI-spacer when the data were reported as absolute improvement (0.19 +/- 0.05 L for HHN) vs (0.06 +/- 0.03 L for MDI-spacer) or reported as percent change (23.2 +/- 6.6 percent for HHN) vs (9.5 +/- 3.4 percent for MDI-spacer). Asthmatic patients exhibited a significantly greater (p less than 0.05) improvement in FEV1 after HHN (23.4 +/- 4.7 percent change) than after MDI-spacer (6.6 +/- 4.5 percent change). Patients with chronic obstructive pulmonary disease (COPD) exhibited a greater improvement in FVC (p less than 0.05) after HHN (25.2 +/- 6.7 percent change) than after MDI-spacer (5.8 +/- 4.7 percent change). We conclude that the "standard" dosage of metaproterenol delivered by HHN results in greater spirometric improvement in patients with acute obstructive pulmonary disease than the conventional dosage of metaproterenol delivered by MDI-spacer. It is likely that this reflects the fact that the recommended dose of metaproterenol delivered by MDI is too low and should be increased.     

Alveolitis correlates with clinical pulmonary involvement in primary Sj?gren's syndrome

Bronchoalveolar lavage (BAL) was performed in 23 patients with primary Sj?gren's syndrome (1Ss) and ten healthy controls to evaluate alveolitis and correlate it with pulmonary and systemic manifestations. Patients with 1Ss had higher BAL total cell count (9.2 +/- 6.7 millions/ml vs 6.1 +/- 2.9 millions/ml) and higher percentage of lymphocytes 23.3 +/- 15.6 percent vs 6.5 +/- 2.9 percent, p less than 0.001) than controls. Twelve patients (group A) constituted the "high alveolitis" group (lymphocytes greater than 15.2 percent) and ten (group B) constituted the "low alveolitis" group (lymphocytes less than 15.2 percent). Group A had more frequent cough (6/12 vs 2/10, p = 0.07), dyspnea (4/12 vs 1/10), and roentgenologic evidence of interstitial lung disease (5/12 vs 0/10, p less than 0.05). They also had lower total lung capacity (85.6 +/- 14.2 percent pred vs 105.8 +/- 23.3 percent pred, p less than 0.05) and Dco (87.7 +/- 20.6 percent pred vs 103.6 +/- 21.0 percent pred). All patients with +3 or +4 grading or lymphocytic infiltrates in lip biopsy specimen belonged in group A (5/12). Finally, T-helper/T-suppressor ratio was lower in group A than in group B. The intensity of alveolitis was not correlated with clinical or serologic manifestations of systemic disease.     

Pulmonary function in normal subjects and patients with sarcoidosis after bronchoalveolar lavage

To determine if fiberoptic bronchoscopy (FOB) with bronchoalveolar lavage impairs pulmonary function in normal subjects or those with sarcoidosis, we measured flow-volume loops, thoracic gas volume, and single breath carbon monoxide diffusing capacity before, one half hour and 24 hours after lavage. We studied 12 normal subjects; six underwent a large volume lavage (approximately 500 ml saline instilled), and six underwent a small volume lavage (approximately 175 ml). Five subjects with sarcoidosis also had a small volume lavage. Six control subjects underwent FOB without lavage. The FOB alone produced no significant changes in pulmonary function one half hour after the procedure. Small volume lavage in normal subjects produced no change except for a 16.3 +/- 5.1 percent (mean- +/- SEM) decline in peak expiratory flow rate (p less than .05) one half hour postlavage which returned to normal by 24 hours. This contrasts with sarcoidosis subjects in whom forced expiratory volume in one second, peak expiratory flow rate, and vital capacity declined by 20 +/- 4.8 percent, 26.7 +/- 7.3 percent, and 15.2 +/- 4.1 percent, respectively, (all p less than 0.05) one half hour postlavage. No change occurred in total lung capacity or diffusing capacity. Only with large volume lavage did decrements in lung function occur in normal patients that were comparable to those seen in the sarcoidosis subjects. Our findings suggest that bronchoalveolar lavage in normal patients can be associated with a significant and volume-related decline in pulmonary function and that in subjects with sarcoidosis, the deterioration is more pronounced.     

Lung elastic recoil does not correlate with pulmonary hemodynamics in severe emphysema

BACKGROUND: It has been postulated that right ventricular (RV) function may improve after lung volume reduction surgery (LVRS) for severe emphysema due to improvement in lung elastic recoil. Improved lung elastic recoil after LVRS is hypothesized to "tether" open extraalveolar vessels, thereby leading to a decrease in pulmonary vascular resistance (PVR) and improved RV function. Whether a relationship exists between static elastic lung recoil and pulmonary hemodynamics in severe emphysema, however, is unknown. METHODS: We prospectively studied 67 patients with severe emphysema (32 women; mean age, 65.3+/-6.6 years [SD]; mean FEV1, 0.79+/-0.25 L) who had hyperinflation (total lung capacity [TLC], 122.5+/-12.3% of predicted) and gas trapping (residual volume, 209.1+/-41.1% of predicted), and were referred to the National Emphysema Treatment Trial. Lung elastic recoil was measured both at TLC (coefficient of retraction [CR]) and at functional reserve capacity (CR at functional residual capacity [CRfrc]) in each patient. RESULTS: CR and CRfrc values were 1.3+/-0.6 cm H2O/L and 0.61+/-0.5 cm H2O/L, respectively. Hemodynamic measurements revealed a pulmonary artery (PA) systolic pressure of 35.9+/-8.9 mm Hg, mean PA pressure of 24.8+/-5.6 mm Hg, and PVR of 174+/-102 dyne*s*cm(-5). No significant correlations were found between CR and PVR (R=-0.046, p=0.71), PA systolic pressure (R=0.005, p=0.97), or mean PA pressure (R=-0.028, p=0.82). Additionally, no significant correlations were found between CRfrc and PVR (R=-0.002, p=0.99), PA systolic pressure (R=-0.062, p=0.62), or mean PA pressure (R=-0.041, p=0.74). CONCLUSIONS: We conclude there is no correlation between lung elastic recoil and pulmonary hemodynamics in severe emphysema, suggesting that elastic lung recoil is not an important determinant of secondary pulmonary hypertension in this group. Registered with www. clinicaltrials.gov, #NCT00000606.     

Severe hypoxemia in farmer's lung disease with normal findings on chest roentgenogram

A 33-year-old woman farmer developed an acute episode of fever, cough, and shaking chills with persistent shortness of breath. Her PaO2 was 51 with a restrictive pattern on pulmonary function. Her diffusion capacity was 36 percent of predicted. In spite of these abnormalities, she always had normal chest roentgenographic findings. Further studies, including a lung biopsy, led to the diagnosis of farmer's lung disease.     

Reduction of pulmonary capillary blood volume following cold exposure in patients with Raynaud's phenomenon

In a previous study we induced digital vasospasm with cold pressor stimulus, and an acute decrease in the lung diffusing capacity for carbon monoxide (Dsb) resulted. We hypothesized its cause to be spasm occurring simultaneously in the pulmonary vasculature and the digital arteries. We measured in this study the Dsb, the diffusing capacity of the pulmonary membrane (Dm), and the volume of blood in the pulmonary capillaries (Vc) after cold-induced digital vasospasm in patients with Raynaud's phenomenon. Control subjects showed no significant decrease in Dsb, Dm, or Vc after cold exposure. Eight of 12 subjects with Raynaud's phenomenon had a significant decrease in Dsb 60 min after testing (25.3 +/- 6.6 vs 19.8 +/- 6.1 ml/min/mm Hg, p less than 0.01). The acute decrease in Dsb was due to a significant decrease in Vc (54 +/- 20 vs 39 +/- 10 ml, p less than 0.05), while Dm was unchanged (52 +/- 17 vs 51 +/- 20 ml/min). Four subjects who had a decrease in Dsb after cold challenge had repeated studies later after pretreatment with sublingual nifedipine. The magnitude of change in Dsb was similar to that observed in the untreated state (23.6 +/- 10.6 vs 20.9 +/- 9.6 ml/min/mm Hg). We conclude that digital vasospasm is accompanied by an acute reduction in Vc in both primary and secondary Raynaud's phenomenon and indicates concurrent vasoconstriction within the pulmonary vaculature.     

Exponential analysis of the lung pressure-volume curve in patients with chronic pigeon-breeder's lung.

Pigeon-breeder's lung (PBL) is extremely common in Mexico City and often progresses to irreversible pulmonary fibrosis. The exponential analysis of the lung pressure-volume (PV) curve (V = A - Be-kp) has been suggested as a method to separate the lung restriction caused by inflammation from that caused by pulmonary fibrosis; a significantly decreased value for the exponential constant, k, suggests a change in the mechanical properties of the functioning lung parenchyma, while a normal value accompanied by restriction suggests subtraction of lung units without a change in the mechanical properties of the functioning units. We measured lung volumes and static PV curves in 29 patients who had persistent lung restriction following a biopsy-proven diagnosis of PBL. Mean values in the 29 subjects were as follows: age, 43 +/- 13 years; TLC, 61 +/- 15 percent of predicted; VC, 46 +/- 19 percent of predicted; and k, 55 +/- 17 percent of predicted. Twenty-four of the 29 patients had values for k that were below the 95 percent confidence level, and five had "normal" values. There was no difference in TLC and VC (percent of predicted) between those with or without a decreased value for k. Four of five patients with a normal value for k improved subsequent to diagnosis, while only one of 21 patients with a decreased k improved. We conclude that increased lung elasticity manifested by a low value for k is common in patients with chronic PBL. These results support the observation of frequent irreversible lung fibrosis in these patients. Measurements of k could prove a good prognostic indicator at the time of initial diagnosis.     

Pulmonary function after heart-lung transplantation using larger donor organs

Restrictive pulmonary function after heart-lung transplantation (HLT) has been attributed to the use of smaller donor lungs and/or an inability to generate normal negative pleural pressures. Pleural pressure generation depends on both the size of the recipient thoracic cage and its neuromuscular integrity. To determine whether lung volumes after heart-lung transplantation are more dependent on donor lung size or on recipient chest wall characteristics, seven HLT recipients were evaluated before and after transplantation. Postoperative values initially (average, 2 months), 6, and 12 months after transplantation were compared with predicted lung volumes for the recipient and donor organs. TLC dropped from a mean of 5.2 +/- 0.5 L preoperatively to 3.7 +/- 0.3 L (p less than 0.05) 2 months after HLT, but it improved with time and ultimately was not different from preoperative values. The predicted TLC of the HLT donor organs were significantly larger than those of the recipient's predicted TLC, with a mean of 6.9 +/- 0.4 versus 5.3 +/- 0.3 L (p less than 0.05). DLCO, arterial PO2, and PCO2 did not change after surgery. Within limits, larger donor lungs appear to adapt to the constraints of the recipient chest and may be used with clinical success, without apparent adverse effects.     

Reduction of lung distensibility in acromegaly after suppression of growth hormone hypersecretion.

Whether the growth of the lungs in acromegaly is due to alveolar hypertrophy or alveolar hyperplasia is a subject of debate. To discriminate these hypotheses, we compared pulmonary distensibility and diffusing capacity among 11 patients with active acromegaly and 11 matched control subjects, evaluating the response of pulmonary distensibility and diffusing capacity to suppression of growth hormone (GH) hypersecretion. We performed lineal and exponential analyses of quasistatic pressure-volume curves. Patients with active acromegaly had a greater TLC, lung compliance, and shape constant, K, than did normal subjects. We found no significant differences between the study groups in carbon monoxide diffusing capacity or diffusing capacity per unit of alveolar volume. After treatment, patients with inactive acromegaly showed a reduced TLC (6.95 +/- 1.40 [mean +/- SD] L versus 6.35 +/- 1.23 L), reduced lung compliance (3.61 +/- 0.90 L/kPa versus 2.36 +/- 0.79 L/ kPa), reduced K coefficient (2.62 +/- 0.65 kPa(-)(1) versus 1.35 +/- 0.40 kPa(-)(1)), and increased maximal recoil pressure (1.74 +/- 0.38 kPa versus 2.28 +/- 0.25 kPa). We conclude that the increased lung distensibility with normal diffusion capacity demonstrated in patients with active acromegaly, which was partly reversible after suppression of GH hypersecretion, suggests that lung growth in acromegaly may result from an increase in alveolar size.     

Patients with collagen vascular disease and dyspnea. The value of gallium scanning and bronchoalveolar lavage in predicting response to steroid therapy and clinical outcome

Patients with collagen vascular disease with or without pulmonary symptoms were studied to determine the value of gallium scan and bronchoalveolar lavage (BAL) in predicting clinical outcome and response to steroid therapy. Thirty-six subjects, 20 with progressive dyspnea, were studied. Gallium uptake was seen in the lung in 17 of the 20 progressively dyspneic patient's and none of the 16 nonprogressive patients (chi square = 22.5, p less than 0.001). The BAL fluid in the progressive patients had a higher percentage of neutrophils (13.4 percent +/- 2.88) and lymphocytes (16.1 percent +/- 2.75) than in the nonprogressive patients (neutrophils = 3.3 +/- 1.30 percent; lymphocytes = 5.6 +/- 1.57 percent, p less than 0.02 for both). Of the 19 progressive patients who were treated with steroids or cyclophosphamide, six had only increased neutrophils in their BAL fluid and all died. The remaining 13 treated progressive patients had increased lymphocytes or a normal BAL (two patients): six had improvement in their vital capacity, six have had stable function, and one died. We found gallium scan and BAL useful in assessing progressive pulmonary fibrosis in collagen vascular disease.     

Pulmonary involvement in mixed connective tissue disease

To determine the frequency of pulmonary function abnormalities in mixed connective tissue disease (MCTD), we studied 16 consecutive patients. Spirometry, total lung capacity (TLC), diffusing capacity (Dco), static lung compliance (Cst), and tests of respiratory muscle function (peak inspiratory pressure [PIP] and the maximum change in transdiaphragmatic pressure [delta PDI] during a diaphragmatic Müeller maneuver) were obtained. Airway resistance (RAW) and frequency dependence of compliance were also measured. Static lung compliance was less than 0.2 L/cm H2O in four of nine patients. The PIP was less than 75 cm H2O in only one patient and delta PDI was greater than 45 cm H2O in all ten patients evaluated. Frequency dependence of compliance was abnormal in seven of ten patients, whereas RAW was increased in only three of eight patients. The TLC and DCO were less than 80 percent of the predicted values in ten of 16 and 14 of 16 patients respectively. There was no correlation between the level of extractable nuclear antigen and pulmonary function abnormalities. The study did not identify significant abnormalities in respiratory muscle function.     

Long-term prognosis of survivors of Pneumocystis carinii pneumonia. Structural and functional correlates

Limited data exist detailing the long-term sequelae of Pneumocystis pneumonia. Open lung biopsies were obtained in seven renal transplant recipients within 48 hours of the onset of respiratory failure. Biopsy specimens and simultaneous chest roentgenograms were graded without clinical information according to the severity of alveolar damage and pulmonary infiltrates, respectively. Evaluation of pulmonary function and exercise physiology were performed 15 to 21 months after their illness. Pulmonary function indices were normal except FRC (2.65 +/- 0.56 L or 77 +/- 16 percent of predicted) and Dsb (20.0 +/- 7.2 ml/min/mm Hg or 79 +/- 19 percent of predicted). Two patients developed arterial desaturation with exercise. Alveolar damage scores correlated with later exercise arterial desaturation (r = 0.88, p less than 0.05). Simultaneous roentgenographic scores correlated with later abnormalities of Dsb (r = 0.81, p less than 0.05). Mild residual abnormalities of pulmonary function were found in five of seven adult survivors of Pneumocystis pneumonia. These abnormalities correlated with pathologic and radiographic features of the acute illness.