The long‐term impact of severe acute respiratory syndrome on pulmonary function,exercise capacity and health status

Background and objective: Severe acute respiratory syndrome (SARS) emerged in 2003 and its long‐term sequelae remain largely unclear. This study examined the long‐term outcome of pulmonary function, exercise capacity, health and work status among SARS survivors. Methods: A prospective cohort study of SARS patients at the Prince of Wales Hospital, Hong Kong was conducted, with serial assessments of lung function, 6MWD and 36 item Short Form General Health Survey at 3, 6, 12, 18 and 24 months after disease onset. The work status was also recorded. Results: Serial assessments were completed by 55 of the 123 (39.9%) subjects, of whom 27 were health‐care workers (HCW). The mean age of the group was 44.4 (SD 13.2) years and 19 (34.5%) were males. At 24 months, 10 (18.2%), 9 (16.4%), 6 (10.9%) and 29 (52.7%) subjects had FEV₁, FVC, TLC and DL_CO < 80% of predicted values, respectively. The mean (SD) 6MWD increased significantly from 439.0 (89.1) m at 3 months to 460.1 (102.8) m at 6 months (P 0.016) and became steady after 6 months. However, 6MWD and 36 item Short Form General Health Survey scores were lower than the normal population throughout the study. Moreover, 29.6% of HCW and 7.1% of non‐HCW had not returned to work 2 years after illness onset. Conclusions: This 2‐year study of a selected population of SARS survivors, showed significant impairment of DL_CO, exercise capacity and health status persisted, with a more marked adverse impact among HCW.     

Recovery of pulmonary functions,exercise capacity,and quality of life after pulmonary rehabilitation in survivors of ARDS due to severe influenza A (H1N1) pneumonitis

Background

Acute respiratory distress syndrome (ARDS) due to severe influenza A H1N1 pneumonitis would result in impaired pulmonary functions and health‐related quality of life (HRQoL) after hospital discharge.

Objectives

The recovery of pulmonary functions, exercise capacity, and HRQoL in the survivors of ARDS due to 2009 pandemic influenza A H1N1 pneumonitis (H1N1‐ARDS) was evaluated in a tertiary teaching hospital in northern Taiwan between May 2010 and June 2011.

Patients and Methods

Data of spirometry, total lung capacity (TLC), diffusing capacity of carbon monoxide (DL_CO), and 6‐minute walk distance (6MWD) in the patients survived from H1N1‐ARDS were collected 1, 3, and 6 months post‐hospital discharge. HRQoL was evaluated with St. George respiratory questionnaire (SGRQ).

Results

Nine survivors of H1N1‐ARDS in the study period were included. All these patients received 2 months’ pulmonary rehabilitation program. Pulmonary functions and exercise capacity included TLC, forced vital capacity (FVC), forced expiratory volume in the first second (FEV₁), DL_CO, and 6MWD improved from 1 to 3 months post‐hospital discharge. Only TLC had further significant improvement from 3 to 6 months. HRQoL represented as the total score of SGRQ had no significant improvement in the first 3 months but improved significantly from 3 to 6 months post‐discharge.

Conclusion

The impaired pulmonary functions and exercise capacity in the survivors of H1N1‐ARDS improved soon at 3 months after hospital discharge. Their quality of life had keeping improved at 6 months even though there was no further improvement of their pulmonary functions and exercise capacity.     

1-year pulmonary function and health status in survivors of severe acute respiratory syndrome

STUDY OBJECTIVES: To characterize the long-term pulmonary function and health status in a prospectively identified cohort of patients who survived the severe acute respiratory syndrome (SARS). DESIGN: Prospective follow-up cohort study. SETTING: University-affiliated hospital. PATIENTS: Ninety-four patients who recovered from SARS were assessed at a uniform time point of 1 year after hospital discharge. MEASUREMENTS: The study included the measurement of static and dynamic lung volumes, the determination of the diffusing capacity of the lung for carbon monoxide (D(LCO)), and a health status evaluation using the St. George Respiratory Questionnaire (SGRQ). RESULTS: Eleven patients (12%) had mild impairment of FVC, 20 (21%) had mild impairment of FEV1, 5 (5%) had mild impairment of the FEV1/FVC ratio, and 17 (18%) had mild impairment of the D(LCO). There was one patient (1%) who had moderate impairment of FVC, one patient (1%) who had moderate impairment of the FEV1/FVC ratio, and three patients (3%) who had moderate impairment of the D(LCO). No pulmonary function abnormalities were detected in 59 patients (63%). Mean scores were significantly higher (ie, worse) than the population norms in the activity (p < 0.001), impacts (p < 0.001), and total (p < 0.001) domains of the SGRQ. CONCLUSIONS: One year after recovery from SARS, persistent pulmonary function impairment was found in about one third of patients. The health status of SARS survivors was also significantly worse compared with the healthy population. The main determinants of morbidity in recovered SARS patients need to be further defined.     

Pulmonary rehabilitation for idiopathic pleuroparenchymal fibroelastosis: A retrospective study on its efficacy,feasibility, and safety

BackgroundThe beneficial effects of pulmonary rehabilitation (PR) for patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE) remain unknown. This study aimed to examine the efficacy, feasibility, and safety of PR for IPPFE.MethodsWe retrospectively investigated 25 patients with IPPFE referred for PR between April 2007 and March 2017. The PR mainly consisted of a 10-week exercise training program. The primary outcome was a change in 6-min walk distance (6MWD). Secondary outcomes included changes in dyspnea (transition dyspnea index [TDI]), anxiety and depression (hospital anxiety and depression scale [HADS]), and health-related quality of life (HRQoL) (St George's respiratory questionnaire [SGRQ]).ResultsThirteen patients participated in the PR program (PRP). Recurrent pneumothorax was the most common reason for patients not participating in the PRP. Four patients discontinued the PRP due to the recurrence of pneumothorax, new onset of pneumomediastinum, stroke, and another reason, respectively. Nine patients completed the PRP. Significant improvement was observed in 6MWD (median [interquartile range], 90 m [55–116 m]; P = 0.033). Clinically important improvements in the 6MWD, and TDI, HADS-anxiety, HADS-depression, and SGRQ total domain scores were observed in seven (78%), five (56%), four (44%), four (44%), and five (56%) of the nine patients, respectively.ConclusionsPatients with IPPFE benefited from PR in terms of exercise capacity, dyspnea, anxiety, depression, and HRQoL. Pneumothorax and pneumomediastinum may impede the implementation of a PRP for patients with IPPFE. While careful patient selection is required, PR may be an efficacious non-pharmacological approach for managing disabilities in patients with IPPFE.     

Effect of lung volume reduction surgery on bony thorax configuration in severe COPD.

STUDY OBJECTIVES: Hyperinflation in patients with severe COPD is associated with an increased anteroposterior (AP) rib cage diameter. We sought to determine whether bilateral lung volume reduction surgery (LVRS) affects bony thorax configuration. DESIGN: Prospective of clinical data collection before and after LVRS. SETTING: Tertiary-care university medical center. PATIENTS: We measured multiple AP and transverse thoracic diameters, by using plain chest roentgenograms (CXRs) in 25 patients (11 men, 14 women), and thoracic CT scans in 14 patients (7 men, 7 women), preoperatively and 3 months postoperatively. A subgroup of 7 patients (reference data) also had CXR thoracic diameter measurements made, using films obtained previously within a year of their presurgical evaluation. Another subgroup of 10 patients had CT scan measurements also made 12 months postoperatively. MEASUREMENTS AND RESULTS: CXR dimensions were taken at the level of the manubrium sterni (M) and thoracic T7 and T11 levels. CT dimensions were taken at T4, T6, T8, and T10 levels. At each level, left (L), midsagittal (C), and right (R) AP and maximal transverse diameters were measured. The sum of the three AP diameters (Total) was used for calculations. Patients also underwent tests such as spirometry, lung volumes, diffusing capacity of the lung for carbon monoxide, 6-min walk distance (6MWD), and transdiaphragmatic pressures during maximum static inspiratory efforts (Pdimax sniff) measured before and 3 months after LVRS. Patients were (mean +/- SD) 58+/-8 years old, with severe COPD and hyperinflation (FEV1, 0.68+/-0.23 L; FVC, 2.56+/-7.3 L; and total lung capacity [TLC], 143+/-22% predicted). After LVRS, AP diameters were reduced at thoracic level T7 (from 24.2+/-2.0 cm to 23.3+/-2.2 cm, p = 0.0002), and transverse diameters were reduced at T7 (from 26.8+/-1.9 cm to 26.4+/-1.7 cm, p = 0.001) and T11 (from 29.9+/-2.2 cm to 29.5+/-2.2 cm, p = 0.03), as measured using the CXR. In contrast, thoracic diameters were similar in subjects with CXRs before LVRS and within 1 year before evaluation. CT-measured AP diameters were significantly reduced 3 months after LVRS at T6, (from 48.8+/-6.0 cm to 46.7+/-5.4 cm, p = 0.02), T8 (from 54.2+/-7.0 cm to 52.3+/-6.5 cm, p = 0.004), and T10 (from 53.8+/-7.5 cm to 51.2+/-8.0 cm, p = 0.001), but not at T4. These AP diameter reductions directly correlated with the postoperative reductions in TLC and residual volume, and also with the increases in Pdimax sniff and 6MWD after LVRS. The reduction in AP diameters at thoracic levels T8 and T10 seen 3 months after LVRS remained stable at 12-month follow-up, whereas those measured at T6 lost statistical significance. CT-measured transverse diameters were unchanged at all levels after LVRS. CONCLUSIONS: We conclude that LVRS decreases mid-to-lower AP rib cage diameter as assessed by CXR and thoracic CT. Although transverse diameters were reduced on CXR, the magnitude was small and was not confirmed with CT. After LVRS, AP diameter reductions are most likely the result of reduction in lung volume, and they are associated with improvements in diaphragm strength and exercise endurance.     

冠心病患者握力 肺功能 六分钟步行试验左心室射血分数的相关性研究

目的:探讨冠心病患者握力、功能肺活量(FVC)、第一秒用力呼吸量(FEV1)、呼气峰流速(PEF)、6 min步行距离(6MWD)、左心室射血分数(LVEF)的相关性。方法:回顾性收集2017年1月至2018年12月北京医院心内科住院的冠心病患者35例,年龄(70.4±10.6)岁,根据年龄分为非老年组(<65岁)8例...     

Clinical impact of forced vital capacity on exercise performance in patients with chronic obstructive pulmonary disease

BackgroundForced vital capacity (FVC) has been suggested to be a good biomarker for decreased exercise performance in patients with chronic obstructive pulmonary disease (COPD). However, as FVC is highly correlated with forced expiratory volume in 1 second (FEV₁), the relationship between FVC and exercise capacity should be assessed within the category of FEV1, i.e., COPD severity. However, this was not considered in previous studies. Thus, limited data are available on the association between reduced FVC and exercise capacity measured by 6-min walk distance (6MWD) based on COPD severity.MethodsWe performed a cross-sectional study using data from the Korean COPD Subgroup Study (KOCOSS) cohort. We evaluated 1,386 patients with moderate (n=895) and severe-to-very severe (n=491) COPD. Reduced FVC was defined as FVC <80% predicted and short 6MWD as <350 m. Multivariable logistic regression was used to evaluate the association between reduced FVC and short 6MWD.ResultsThere were no significant differences in respiratory symptoms and quality of life between the patients with reduced FVC and those with preserved FVC. However, patients with reduced FVC had shorter 6MWD (30.5 cm in moderate and 34.5 cm in severe-to-very severe COPD) and higher BODE index scores than those with preserved FVC. The cubic spline model revealed 6MWD peaked around 93% predicted of FVC in moderate COPD, whereas FVC showed a positive association with 6MWD in severe-to-very severe COPD. Multivariable analyses showed that reduced FVC was significantly associated with short 6MWD in both moderate [adjusted odds ratio (aOR) =1.44, 95% confidence interval (CI): 1.03–2.02] and severe-to-very severe (adjusted OR =1.55, 95% CI: 1.01–2.40) COPD.ConclusionsReduced FVC was significantly associated with shorter 6MWD in moderate-to-very severe COPD patients, suggesting that reduced FVC might be reflective of 6MWD-measured exercise capacity in moderate-to-very severe COPD.     

Instrument accuracy and reproducibility in measurements of pulmonary function

BACKGROUND: The objective of the study was to quantify the accuracy and reproducibility of five commercially available pulmonary function test (PFT) instruments (Collins CPL [Ferraris Respiratory; Louisville, CO]; Morgan Transflow Test PFT System [Morgan Scientific; Haverhill, MA]; SensorMedics Vmax 22D [VIASYS Healthcare; Yorba Linda, CA]; Jaeger USA Masterscreen Diffusion TP [VIASYS Healthcare]; and Medical Graphics Profiler DX System [Medical Graphics Corp; St. Paul, MN]) that are associated with spirometry and the measurement of pulmonary diffusing capacity. METHODS: In a multifactor, single-center, repeated-measures, full factorial 90-day study, a pulmonary waveform generator and a single-breath simulator of diffusing capacity of the lung for carbon monoxide (Dlco) were used to perform simulations of FVC and Dlco maneuvers. Accuracy was assessed as the difference between the observed and simulated values. Reproducibility was determined as the coefficient of variation of all measurements made during the study. RESULTS: All instruments demonstrated a high degree of accuracy in the measurement of FVC and FEV(1). Overall, the accuracies associated with the measurement of peak flow, forced expiratory flow, mid-expiratory phase, and diffusing capacity were generally lower and more variable among the instruments tested. The coefficients of variation of Dlco measurements over 90 days were higher than those observed for spirometry. CONCLUSIONS: This study demonstrates the feasibility of assessing the accuracy and reproducibility of modern PFT instruments using simulation testing. Our results provide an assessment of the component of PFT accuracy and reproducibility that is due to instrumentation alone.     

Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension

STUDY OBJECTIVE: To differentiate the clinical, radiographic, and physiologic profile in patients with sarcoidosis with and without pulmonary hypertension. DESIGN: Retrospective survey. SETTING: Tertiary care center. PATIENTS: One hundred six patients with sarcoidosis were classified by two-dimensional echocardiography into two groups: group 1, 54 patients with pulmonary hypertension; group 2, 52 patients without pulmonary hypertension. INTERVENTIONS: Patients underwent two-dimensional and Doppler echocardiography, chest radiography (CXR), pulmonary function testing, and arterial oxygen saturation determination, and the test results were compared between the two groups. Statistical analysis was performed using independent-sample t test and chi2 test, as appropriate; p < 0.05 was considered to be significant. RESULTS: Predicted spirometric values and lung diffusing capacity were significantly lower in patients in group 1 compared to patients in group 2: FVC, 54% vs 64% (p = 0.0065), FEV(1), 47% vs 61% (p = 0.0005), forced expiratory flow, midexpiratory phase, 35% vs 52% (p = 0.0363), and single-breath diffusing capacity of the lung for carbon monoxide (D(LCO)sb), 39% vs 54% (p = 0.0001). Sixty percent of patients in group 1 had radiographic Scadding stage 4 sarcoidosis, while no radiographic stage predominated in group 2. Arterial oxygen saturation, need for oxygen supplementation, and degree of desaturation after exercise did not differ between groups. CONCLUSIONS: The presence of pulmonary hypertension in patients with sarcoidosis is associated with higher prevalence of stage 4 sarcoidosis by CXR and lower predicted spirometric and D(LCO)sb measurements.     

Prevalence and reversibility of pulmonary dysfunction in refractory systemic lupus: improvement correlates with disease remission following hematopoietic stem cell transplantation

AIM: To report the prevalence and reversibility of pulmonary function test (PFT) abnormalities among systemic lupus erythematosus (SLE) patients, refractory to therapy, undergoing hematopoietic stem cell transplantation (HSCT). METHODS: Thirty-four SLE patients received 200 mg/kg cyclophosphamide and 90 mg/kg equine antithymocyte globulin followed by HSCT. PFTs were performed prior to, at 6 months, and yearly following HSCT. RESULTS: The prevalence of significant PFT abnormalities was high (97%). Low FEV(1) and FVC occurred in 26 of 34 patients (76%). A significant abnormality in diffusion capacity of the lung for carbon monoxide (Dlco) occurred in 26 of 32 individuals able to complete Dlco testing (81%). Dlco 18 months after HSCT. Five of 28 patients had a normal entry FVC; for each, the FVC remains normal. Of the 23 patients with an abnormal baseline FVC, 18 have improved, 15 completely and 3 partially. Eight of these 18 patients also have improved Dlco. The two patients with a diagnosis of SLS and one patient with SLE-related pulmonary hypertension improved in both parameters. Only 5 of 23 patients with an abnormal FVC did not improve. Each of these five patients retained active lupus in spite of HSCT. CONCLUSION: The prevalence of lung impairment among SLE patients requiring long-term immune suppression is high. Following HSCT, pulmonary impairments can improve, which is sustained if disease control is sustained.     

The 6-min walk distance, peak oxygen uptake, and mortality in COPD

In patients with COPD, the maximal oxygen uptake (Vo2) measured at peak exercise and the 6-min walk distance (6MWD) have been associated with survival; however, no study has compared the strength of the association in the same patients. In this study, we compared the association between the 6MWD and peak Vo2 and mortality in 365 patients with COPD. Patients' cardiopulmonary cycle ergometry test results and 6MWD were determined at entry, and patients were followed up for a mean period of 67 months. There were 171 deaths. Compared with survivors, nonsurvivors were older (mean [+/- SD] age, 67.9 +/- 8 vs 65.9 +/- 8 years, respectively; p = 0.008), had worse mean FEV1 (36.5 +/- 12 vs 42.6 +/- 14 L, respectively; p = 0.02), had lower mean peak Vo2 (9.8 +/- 3 vs 11.8 +/- 3.6 mL/Kg/min, respectively; p < 0.0001), lower mean 6MWD (312 +/- 104 vs 377 +/- 95 m, respectively; p < 0.0001), and lower mean exercise minute ventilation (37.4 +/- 12 vs 42.3 +/- 13 L/min, respectively; p = 0.004). Univariate analysis showed that peak Vo2 and 6MWD as well as comorbidity, FEV1, and body mass index were associated with death. Logistic regression analysis with mortality as the dependent variable revealed that 6MWD (hazard ratio [HR], 0.996; 95% confidence interval [CI], 0.993 to 0.999; p < 0.01) had a stronger association than the peak Vo2 (HR, 0.971; 95% CI, 0.959 to 1.000; p = 0.050) with mortality. This study shows the 6MWD is as good predictor of mortality as the peak Vo2 in patients with COPD.     

Change in generic and disease-specific health-related quality of life during a one-year period in patients with newly detected chronic obstructive pulmonary disease

BACKGROUND: Although the health-related quality of life (HRQoL) in patients with chronic obstructive pulmonary disease (COPD) has been assessed by generic or disease-specific HRQoL measures, the responsiveness of the generic HRQoL measures is generally weak. OBJECTIVES: To investigate the responses generated by generic and disease-specific HRQoL questionnaires, we prospectively followed the clinical course of patients with newly detected COPD after the initiation of treatment. METHODS: A prospective, longitudinal study with a 1-year follow-up was designed. The forced expiratory volume in 1 s (FEV(1)), forced vital capacity (FVC), the Nottingham Health Profile (NHP) as a generic HRQoL measure, and the Chronic Respiratory Disease Questionnaire (CRQ) as a disease-specific HRQoL measure were measured at baseline and at 3, 6, and 12 months after the initiation of standard treatment. RESULTS: Eighty-two patients completed the study. The FEV(1), FVC, and NHP and CRQ scores improved significantly during the first 3 months (p < 0.05). During the last 6 months, although the FEV(1) declined (p < 0.05), the HRQoL assessed by the NHP and CRQ remained elevated. Except for the score on the social isolation section of the NHP at 12 months, all HRQoL scores at 6 and 12 months were significantly improved compared to baseline (p < 0.05). CONCLUSION: In new patients with COPD, the NHP as well as the CRQ was able to detect changes in the HRQoL associated with effective medical interventions. The influence of the changes in airflow limitation on the HRQoL was weak.     

Physiological and symptom determinants of exercise performance in patients with chronic airway obstruction

To evaluate the physiological and symptom determinants of exercise performance (EP) as measured by a 6-min walking test (6MWD), Watt(max), and peak oxygen consumption (VO2 ml/min/kg), 105 patients with chronic airway obstruction (CAO) [50 chronic obstructive pulmonary disease (COPD): 44 men, aged 63+/-7 years, forced expiratory volume in 1 sec (FEV1) forced vital capacity (FVC)(-1)% 54+/-13; and 55 asthmatic: 23 men, aged 55+/-10 years, FEV1 FVC(-1) % 65+/-10] underwent evaluation of 6MWD, symptom limited cyclo-ergometer exercise test, spirometry, respiratory muscle function, arterial blood gases and sensation of dyspnoea [using the Borg scale, Visual Analogue Scale (VAS) and Baseline Dyspnoea Index (BDI)]. A hierarchical method of analysis identified the residual volume (RV), total lung capacity (TLC)(-1) ratio, BDI and the patient's age as the strongest and most consistent correlates of EP (r2 = 0.14-0.21). The correlation between EP and its various determinants was not influenced by diagnosis. The relationship between breathlessness and EP was different between men and women: at any given level of exercise, women were more breathless than men. In multivariate analyses that contained both RV TLC(-1) and BDI, the RV TLC(-1) ratio was the strongest correlate of EP, although the BDI remained a significant covariate. Overall, age was the major determinant of EP but inclusion of the RV TLC(-1) ratio and the BDI into the model explained a further 9-15% of the variance in EP. These three covariates together explained 26-34% of the variance between patients. We conclude that in stable CAO patients, the prediction of exercise capacity by anthropometric, demographic, clinical and physiological variables is likely to be low. Age, pulmonary hyperinflation and dyspnoea are the strongest and most consistent correlates of impaired exercise performance. Airways obstruction, measured during expiration using FEV1, does not appear to be a predictor of physiological impairment. These results underline the importance of performing exercise evaluation in CAO patients.     

肺一氧化碳弥散量对博莱霉素致人肺毒性作用的监测

目的　探讨肺一氧化碳弥散量 (DLCO)是否可以作为博莱霉素致肺的毒性作用 (BILT)的早期监测指标。方法　选择接受 2次含博莱霉素化疗方案化疗且至少有 2次肺功能测定结果的卵巢生殖细胞恶性肿瘤患者 2 3例 ,根据博莱霉素的累积剂量 ,将 2 3例患者分为 4组 :用药前组、博莱霉素 <10 0mg组、博莱霉素 10 1～ 2 0 0mg组和博莱霉素 >2 0 1mg组 ,记录每例患者所做的每次肺功能数据及其对应的博莱霉素累积剂量 ,对各组间的肺功能指标行方差分析。并对DLCO与博莱霉素累积剂量行直线回归分析。结果　各组间第 1秒钟用力呼气容积、用力肺活量、肺总量差异无显著性 ,DLCO的校正值与博莱霉素累积剂量间存在负相关性 (y =10 0 .5 9- 0 .11x ,r =- 0 .6 4 9,P <0 .0 0 1)。结论　DLCO是监测BILT的最敏感的亚临床指标     

Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease

OBJECTIVE: To determine whether pulmonary arterial hypertension (PAH) is a prognostic factor for mortality in diffuse cutaneous systemic sclerosis (dcSSc), independent of interstitial lung disease (ILD). METHODS: ILD was diagnosed by high-resolution computed tomography and PAH (pulmonary arterial systolic pressure [PASP] > or =45 mm Hg) by echocardiography. All patients with ILD underwent testing for total lung capacity (TLC), forced vital capacity (FVC), and diffusing capacity for carbon monoxide. RESULTS: Eighty-six patients with dcSSc (mean age at diagnosis 44.5 years) were followed up for a median of 72.5 months. ILD was found in 52 patients (60%) and PAH in 18 (21%). ILD was associated with PAH in 15 patients. Seventeen patients died (19.8%), 9 of whom had PAH (P = 0.001) and 10 of whom had ILD (P = 0.99). By multivariate analysis, age at SSc diagnosis and PAH were the only independent predictors of death (hazard ratio [HR] 1.057, 95% confidence interval [95% CI] 1.009-1.109, P = 0.020 and HR 4.09, 95% CI 1.47-11.5, P = 0.007, respectively). Mean TLC and mean FVC were similar in ILD patients with and those without PAH (P = 0.71 and P = 0.40, respectively). Among ILD patients, age at SSc diagnosis and PAH were again the sole predictors of death (HR 1.073, 95% CI 1.003-1.149, P = 0.042 and HR 5.07, 95% CI 1.09-23.8, P = 0.038, respectively). Twenty ILD patients received at least 6 monthly pulses of intravenous cyclophosphamide (CYC). In CYC-treated patients with PAH (n = 8), PASP increased significantly during the CYC regimen (mean +/- SD 55 +/- 14.5 mm Hg; P = 0.015 versus baseline), while TLC remained stable during the same period. CONCLUSION: These results indicate that, independent of ILD, PAH is a major prognostic factor for survival in dcSSc.     

Zinc chloride (smoke bomb) inhalation lung injury: clinical presentations, high-resolution CT findings, and pulmonary function test results

STUDY OBJECTIVES: Zinc chloride smoke inhalation injury (ZCSII) is uncommon and has been rarely described in previous studies. We hypothesized that structural changes of the lung might correlate with pulmonary function. To answer this question, we correlated findings from high-resolution CT (HRCT) scan and the results of pulmonary function tests (PFTs) in patients with ZCSII. DESIGN: Retrospective cohort study. SETTING: University hospital. PATIENTS: Twenty patients who had been hospitalized with ZCSII-related conditions. MEASUREMENTS: The study included HRCT scan scores (0 to 100), static and dynamic lung volumes, and diffusing capacity of the lung for carbon monoxide (D(LCO)). RESULTS: HRCT scans and PFTs were performed initially after injury (range, 3 to 21 days) in all patients and during the follow-up period (range, 27 to 66 days) in 10 patients. The predominant CT scan findings were patchy or diffuse ground-glass opacities with or without consolidation. The majority of patients showed a significant reduction of FVC, FEV1, total lung capacity, and D(LCO), but normal FEV1/FVC ratio values. Changes of functional parameters correlated well with HRCT scan scores. Substantial improvements in CT scan abnormalities and pulmonary function were observed at follow-up. CONCLUSIONS: The majority of our patients with ZCSII presented with a predominant parenchymal injury of the lung that was consistent with a restrictive type of functional impairment and a reduction in Dlco rather than with obstructive disease. Our results suggest that HRCT scanning and pulmonary function testing may reliably predict the severity of ZCSII.     

Six minute walk distance is a predictor of survival in patients with chronic obstructive pulmonary disease undergoing pulmonary rehabilitation

BACKGROUND:Chronic obstructive pulmonary disease (COPD) is a progressive and distressing disease with a trajectory that is often difficult to predict.OBJECTIVE:To determine whether initial 6 min walk distance (6MWD) or change in 6MWD following inpatient pulmonary rehabilitation (PR) predicted survival.METHODS:Patients referred for PR in 2010 were studied in a retrospective chart review. Measures of 6MWD before and following PR were recorded. Initial 6MWD was categorized as ≥250 m, 150 m to 249 m and ≤149 m. Government databases provided survival status up until December 2013 and survival analyses were performed. Initial 6MWD and a minimally important difference (MID) of ≥30 m were used for survival analysis.RESULTS:The cohort consisted of 237 patients (92 men, 145 women) with severe COPD. Mean (± SD) forced expiratory volume in 1 s (FEV₁) was 0.75±0.36 L, with a mean FEV₁/forced vital capacity (FVC) ratio of 0.57±0.16. Overall three-year survival was 58%. Mean survival for the study period as per predefined categories of 6MWD of ≥250 m, 150 m to 249 m and ≤149 m was 42.2, 37.0 and 27.8 months (P<0.001), respectively, with a three-year survival of 81%, 66% and 34% observed, respectively. Overall mean change in 6MWD was 62±57 m, and a minimal improvement of ≥30 m was observed in 72% of patients. In the lowest walking group, early mortality was significantly higher among those who did not achieve minimal improvement. Older age, male sex and shorter initial 6MWD were negative predictors of survival.CONCLUSION:In patients with severe COPD, initial 6MWD was predictive of survival. Overall survival at three years was only 58% and was especially poor (34%) in patients with low (<150 m) initial walk distance.     

Clinical determinants of abnormalities in pulmonary functions in survivors of the adult respiratory distress syndrome

Pulmonary function tests (PFTs) were performed in 39 survivors of the adult respiratory distress syndrome (ARDS) in whom clinical data had been prospectively collected during the acute episode. PFTs stabilized within 6 months of the episode and had returned to normal in most survivors. Persistent abnormalities were found after 6 months in diffusing capacity (14 of 23 patients, 61%), vital capacity (10 of 23 patients, 43%), and total lung capacity (five of 24 patients, 21%). To clarify the mechanisms underlying these persistent abnormalities, we attempted to correlate long-term PFT outcomes with estimates of the severity of initial lung injury as assessed from clinical data and with other features of the patient's course. The severity of lung function impairment during the first 3 days of ARDS was not related to long-term PFT values. However, a lower DLCO was related to a higher AaDO2, higher pulmonary artery pressure, and worse radiographic appearance on Days 4 through 7 and to the occurrence of sepsis. A lower FVC was related to higher pulmonary vascular resistance in Days 4 through 7 of ARDS. Long-term values for FVC and TLC were directly related to increasing levels of PEEP applied from Days 4 through 7 of ARDS in patients with peak airway pressures less than 50 cm H2O. Long-term abnormalities of pulmonary function of survivors of ARDS were not related to initial lung impairment but were directly related to persistence of impaired lung function during the acute episode. Recovery of lung function may also have been directly related to therapeutic modalities such as PEEP and impaired by the occurrence of sepsis.     

Factors associated with dyspnea in adult patients with sickle cell disease

OBJECTIVE: The aim of this prospective study was to determine the cardiorespiratory factors associated with dyspnea in patients with sickle cell SS-hemoglobin disease, with a specific interest in lung vascular involvement. MEASUREMENTS: Forty-nine patients (29 women and 20 men; mean [+/- SD] age: women, 29 +/- 6 years; men, 31 +/- 11 years) underwent direct evaluations (Borg scale evaluation during a 6-min walk test) and indirect evaluations (modified Medical Research Council [MRC]score) of their dyspnea, pulmonary function tests (PFTs) [spirometry, volumes, diffusing capacity of the lung for carbon monoxide (Dlco), diffusing capacity of the alveolar-capillary membrane, and pulmonary capillary blood volume measurements], echocardiography, and biological evaluation. RESULTS: Thirty-four patients complained of significant breathlessness (MRC score, > 1). Indirect and direct evaluations of dyspnea were correlated. PFT results depicted a very mild restrictive pattern (mean total pulmonary capacity, 86 +/- 11% predicted) and an impairment of Dlco (mean Dlco corrected for the degree of anemia, 69 +/- 13% predicted). The statistical analysis demonstrated that dyspnea and exercise performance were closely linked to indexes of Dlco but not with any echocardiographic or biological measure including anemia. Nevertheless, only approximately 25% of the variability was explained by these associations. Despite having a similar history of vasoocclusive crisis events, women had more severe anemia, dyspnea, decreases in Dlco (corrected for the degree of anemia), and a higher capillary blood volume (corrected for alveolar volume) than men. CONCLUSION: Lung vascular disease contributes to dyspnea and the exercise limitation of patients with sickle cell disease. A sequential assessment of Dlco would therefore constitute one of the objective functional end points for follow-up studies of these patients.     

Reliability,repeatability and sensitivity to change of externally and self-paced walking tests in COPD patients

In COPD, several walking tests have been proposed to measure exercise tolerance but their relative merits are uncertain. We studied 57 moderate-to-severe, stable COPD patients (mean FEV1 35 +/- 12% predicted). Within a 2-month period, we compared reliability (inter-subject variability) and repeatability (intra-subject variability) of the most widely used 6-min walks (6MWD), with self-paced 2-min walks (2MWD) and externally paced, incremental shuttles (Shuttle). On 9 separate days either of the three 6MWD, 2MWD or Shuttles were performed (nine walks of each type). Then, each walk was performed before and after bronchodilators (BD) to assess sensitivity to change (mean change/standard deviation of change (sensitivity index--SI)). For all exercise tests, reliability was >90% (2MWD 95%, 6MWD 92% and Shuttle 91%). Repeatability was excellent (overall <10% intra-subject variation; for 2MWD 5%, 6MWD 8% and Shuttle 9%). On average, the first walking distance was significantly shorter, but there were no significant differences between second and third walks. Dyspnoea scores were much less reproducible. BD produced highly significant improvements in Shuttle (pre-BD 27 SD=11 --> post-BD 30 SD=11), 6MWD (424 m SD=115 --> 462 m SD=106) and 2MWD (153 m SD=35 --> 162 m SD=34), (P < 0.0001). SI was similar for all walks (6MWD 0.84, 2MWD 0.75 and Shuttle 0.76). In moderate-to-severe COPD, 2MWDs are as informative as 6MWDs without their disadvantages. Self-paced walks are as useful as externally paced Shuttles.