Change In FVC Research Articles

Cystic fibrosis and pregnancy in the modern era: A case control study

Increasingly, women with cystic fibrosis become pregnant. Outcomes of these women need further study particularly in the setting of improved survival in CF. We performed a case-control study of pregnant CF women including 22 matched pairs with an average follow-up of 4.5 years. Nutritional outcomes, changes in lung function, and exacerbation rates were compared. Matched pairs were similar in age, sweat chloride, FEV1 and FVC % predicted, BMI, and diabetes status. Change in BMI, FEV1 and FVC % predicted at the end of pregnancy and at last follow-up were similar between groups. Moreover, rates of exacerbation before, during and after pregnancy were similar. On multivariable analysis pregnancy had no effect on change in lung function over the study period. Significant predictors of decline included higher pre-pregnancy lung function and pancreatic insufficiency. Pregnancy does not lead to immediate or medium-term adverse effects for CF patients.

331 A dynamic population-based model for the development of chronic obstructive pulmonary disease

ObjectivesWe present a mathematical model for the development of chronic obstructive pulmonary disease (COPD), incorporating population dynamics, trends in smoking, and occupational exposure to respirable dust, fumes and gases. The...

Mycophenolate Versus Cyclophosphamide for Progressive Interstitial Lung Disease Associated with Systemic Sclerosis: A 2-Year Case Control Study

Cyclophosphamide is considered the treatment of choice for interstitial lung disease (ILD) secondary to systemic sclerosis (SSc), albeit having a minimal effect. Although controlled evidence does not exist, mycophenolate is used increasingly in clinical practice as an alternative. We aimed to compare the long-term efficacy of these drugs. Patients from our SSc cohort who received mycophenolate for over 1year for progressive ILD were 1:1 matched for age, gender, and baseline forced vital capacity (FVC±3%) with cyclophosphamide-treated patients. Changes in FVC, total lung capacity (TLC), diffusion capacity for carbon monoxide (DLCO), and high-resolution computed tomography (HRCT) scans were compared between groups. Changes in pulmonary function tests (PFTs) over at least 1year in six unmatched control patients, who had denied mycophenolate or cyclophosphamide, also were examined. FVC, TLC, and DLCO did not change significantly in either mycophenolate (from 79.0±12.5 to 80.2±8.1 to 81.2±11.4, from 71.5±16.1 to 74.3±10.8 to 71.8±13.0, from 56.8±12.0 to 55.2±9.9 to 50.6±8.5, respectively) or cyclophosphamide group (from 77.3±12.5 to 79.7±10.3 to 82.5±12.9, from 64.7±14.9 to 68.6±16.0 to 66.1±15.5, from 53.1±14.3 to 56.4±13.5 to 56.3±6.7, respectively), after 1 or 2years of treatment. PFTs also remained stable in the control group. In either the mycophenolate or cyclophosphamide groups, six patients remained stable, three improved, and one deteriorated according to the definitions of the American Thoracic Society. However, and despite the fact that patients in the cyclophosphamide group had more extended ILD at baseline, a deterioration of lung HRCT findings at 2years was noticed after mycophenolate (from 10.0±8.9 to 12.7±8.2, p=0.039) but not after cyclophosphamide. Although these results derive from patients selected for receiving at least 1year of treatment and therefore they do not represent an intention-to-treat cohort, an eagerness to replace cyclophosphamide by mycophenolate in SSc-associated ILD treatment is not supported.

Place de la distension pulmonaire dans l’exploration des gros fumeurs de cigarettes

Lung hyperinflation (LH) has become a major concern in the management of chronic obstructive pulmonary disease (COPD). To evaluate the role of lung volumes in the positive diagnosis of COPD and in the assessment of airway obstruction reversibility. Three hundred and sixty-six male smokers over the age of 35 with more than 40 pack-years exposure were included in the study. Plethysmographic data were determined before/after taking a bronchodilator (BBD, ABD). Applied definitions: airflow obstruction: BBD FEV1/FVC<0.70. LH: BBD residual volume (RV)>upper limit of normal. Expressions of reversibility: Δvariable=(ABD-BBD) values; Δinit%=Δvariable/BBD value and Δref%=Δvariable/reference value. A 12%init and a 0.2L increase in either FEV1 or FVC or a 10%ref or - 300 mL decrease in RV were considered as clinically significant. Over the 85 smokers without airflow obstruction, 68% had LH. In the hyperinflated group (n=314), and compared to changes in FEV1 and FVC, these RV changes detected more respondents (54% for FEV1 and FVC vs. 65% for RV, P=0.002). This was not the case for the group free from LH (n=52) (23% for FEV1 and FVC vs. 35% for RV, P=0.09). In the 58 hyperinflated groups free from airflow obstruction, and compared to changes in FEV1 and FVC, changes in RV detected more respondents (24% for FEV1 and FVC vs. 71% for RV, P=0.0001). In heavy smokers, it seems essential to include LH as a criterion for a positive diagnosis of COPD and of reversibility evaluation.

Lung function decline in children and adolescents post-infectious bronchiolitis obliterans: a prospective long term cohort study

Aim of this paper was to evaluate the effects of changes in obesity status on lung function decline over an 8-year follow-up.Adults over 24 years (n=1212) from the general population, who participated in both Po River Delta first (PD1, 1980–1982) and second (PD2, 1988–1991) epidemiological surveys, were stratified as “never obese” (BMI<30 Kg/m2 at both PD1 and PD2), “becoming obese” (BMI<30 Kg/m2 at PD1 and ⩾30 Kg/m2 at PD2), “always obese” (BMI⩾30 Kg/m2 at both PD1 and PD2), and “becoming non-obese” (BMI⩾30 Kg/m2 at PD1 and <30 Kg/m2 at PD2). Linear regression models for changes in FEV1, FVC, and VC (computed as absolute differences between the values at PD2 and those at PD1) with longitudinal categories of obesity, gender, age, and baseline smoking habits as covariates were applied.The “becoming obese” and “always obese” categories had a significantly greater decline of lung function than “never obese” group; in the “always obese” group, this was true for vital capacities but not FEV1. Conversely, in the “becoming non-obese” group lung function was at PD2 improved with respect to PD1. Compared with “Never obese” the mean increase in lung function was of 93, 180, and 48 mL for FEV1, FVC, and VC, respectively.In this general population sample, remaining or becoming obese increases the decline in lung function over 8 years, while becoming non-obese decreases it.

Contribution of nitric oxide in the contraction-induced rapid vasodilation in young and older adults

We tested the hypothesis that reduced nitric oxide (NO) bioavailability contributes to the attenuated peak and total vasodilation following single-muscle contractions in older adults. Young (n = 10; 24 ± 2 yr) and older (n = 10; 67 ± 2 yr) adults performed single forearm contractions at 10, 20, and 40% of maximum during saline infusion (control) and NO synthase (NOS) inhibition via N(G)-monomethyl-l-arginine. Brachial artery diameters and velocities were measured using Doppler ultrasound and forearm vascular conductance (FVC; in ml·min(-1)·100 mmHg(-1)) was calculated from blood flow (ml/min) and blood pressure (mmHg). Peak and total vasodilator responses [change (Δ) in FVC from baseline] were attenuated in older adults at all intensities (P < 0.05). NOS inhibition reduced the peak ΔFVC at 10% (88 ± 12 vs. 52 ± 9 ml·min(-1)·100 mmHg(-1)), 20% (125 ± 13 vs. 83 ± 13 ml·min(-1)·100 mmHg(-1)), and 40% (207 ± 26 vs. 133 ± 20 ml·min(-1)·100 mmHg(-1)) in young subjects, (P < 0.05 for all) and in older adults at 10% (59 ± 5 vs. 47 ± 7 ml·min(-1)·100 mmHg(-1), P < 0.05) and 20% (88 ± 9 vs. 68 ± 9 ml·min(-1)·100 mmHg(-1), P < 0.05), but not 40% (128 ± 12 vs. 105 ± 11 ml·min(-1)·100 mmHg(-1), P = 0.11). The relative (%) reduction in peak ΔFVC due to NOS inhibition was greater in young vs. older adults at 20% (-36 ± 5 vs. -23 ± 5%, P < 0.05) and 40% (-35 ± 6 vs. -16 ± 7%, P < 0.05). The reduction in the total vasodilator response (area under the curve) with NOS inhibition was also greater in young vs. older adults at all intensities. Our data suggest that contraction-induced rapid vasodilation is mediated in part by NO, and that the contribution of NO is greater in young adults.

Bronchodilator efficacy of tiotropium–formoterol via single pressurized meter dose inhaler (pMDI) versus tiotropium alone in COPD

BackgroundBronchodilators form the main stay of treatment for COPD. When symptoms are not adequately controlled with one bronchodilator, addition of another bronchodilator is recommended. We have recently developed a combination of tiotropium and formoterol in a single pressurized metered dose inhaler (pMDI) (Cipla Ltd., India). The aim of this study was to compare the bronchodilator effects of a single dose of 18 mcg of tiotropium versus a single dose of a combination of 18 mcg tiotropium plus 12 mcg formoterol administered via a pMDI in subjects with moderate-to-severe COPD. Study design44 COPD subjects were enrolled in this randomized, double-blind, multi-centre, cross-over study. 18 mcg tiotropium and 18 mcg tiotropium plus 12 mcg formoterol were administered via pressurized metered dose inhalers on two separate days. FEV1, FVC and Inspiratory capacity (IC) were measured before, 15, 30 min, 1, 2, 3, 4, 6, 8, 12 and 24 h after the study drugs were administered. ResultsCompared with tiotropium alone, a combination of tiotropium plus formoterol showed a faster onset of bronchodilator response (p < 0.01 for FEV1 and FVC), a greater mean maximum change in FEV1 (p = 0.01) and FVC (p = 0.008) and greater AUC0–24h values for FEV1, FVC and IC. Trough FEV1 and FVC values were also greater in the combination group. ConclusionA combination of tiotropium plus formoterol administered via a single inhaler produced a superior bronchodilator response than tiotropium alone over a period of 24 h.

Response of the airways and autonomic nervous system to acid perfusion of the esophagus in patients with asthma: a laboratory study

BackgroundGastro-esophageal reflux disease (GERD) predisposes to airway disease through a vagally-mediated esophago-bronchial reflex. This study investigates this vagal response to esophageal acid perfusion.Methods40 asthmatics with mild stable asthma participated. Each subject underwent spirometry and autonomic function testing (valsalva maneuver, heart rate response to deep breathing and to standing from supine position) four times: a) before intubation, b) after intubation, and then immediately after perfusion with, in random order, c) concentrated lime juice solution (pH 2–3) and d) 0.9% saline. Subjects were blinded to the solution perfused.ResultsAsthmatics were of mean (SD) age 34.3 years (1.3), and 67.5% of them were females. pH monitoring demonstrated that 20 subjects had abnormal reflux and 20 did not. In each group 10 subjects had a positive GERD symptom score. Following perfusion with acid compared to saline, all subjects showed significant decreases in FEV1 and PEFR and significant increases in the mean valsalva ratio and heart rate difference on deep breathing from baseline values, but no changes in FVC or heart rate ratio on standing. There were no significant differences in any of the parameters between subjects with and without reflux.ConclusionsAcid stimulation of the distal esophagus results in increased parasympathetic activity and concomitant broncho-constriction in asthmatics irrespective of their reflux state. This strengthens the hypothesis that GER triggers asthma-like symptoms through a vagally mediated esophago-bronchial reflex and encourages a possible role for anti-cholinergic drugs in the treatment of reflux-associated asthma.

FRI0385 Long term follow-up after systemic sclerosis patients treated with intravenous cyclophosphamide pulse therapy for interstitial lung disease: a single eustar center (042) experience.

BackgroundTwo RCT and several open-labeled studies demonstrated various efficacy of cyclophosphamide (CYC) for treatment of Systemic sclerosis-associated interstitial lung disease (SSc-ILD) with follow up for 1-4 years.ObjectivesTo analyze the changes...

The efficacy and safety of low-dose sirolimus for treatment of lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare disease caused by dysregulated activation of the mammalian target of rapamycin (mTOR). Sirolimus, an inhibitor of mTOR, has been reported to decrease the size of angiomyolipomas and stabilize pulmonary function in patients with LAM. However, the optimal dose for the treatment of LAM remains unclear. We conducted a retrospective, observational study of 15 patients with LAM who underwent sirolimus therapy for more than 6 months. The efficacy was evaluated by reviewing the patients' clinical courses, pulmonary function and chest radiologic findings before and after the initiation of sirolimus treatment. All patients had blood trough levels of sirolimus lower than 5ng/mL. Sirolimus treatment improved the annual rates of change in FVC and FEV1 in the 9 patients who were free from chylous effusion (FVC, -101.0 vs. +190.0mL/y, p=0.046 and FEV1, -115.4 vs. +127.8mL/y, p=0.015). The remaining 7 patients had chylous effusion at the start of sirolimus treatment; the chylothorax resolved completely within 1-5 months of treatment in 6 of these cases. These results resembled those of previous studies in which blood trough levels of sirolimus ranged from 5 to 15ng/mL. Low-dose sirolimus (trough level, 5ng/mL or less) performed as well as the higher doses used previously for improving pulmonary function and decreasing chylous effusion in patients with LAM.

Can Serum Surfactant Protein D or CC-Chemokine Ligand 18 Predict Outcome of Interstitial Lung Disease in Patients with Early Systemic Sclerosis?

To examine the predictive significance of 2 pneumoproteins, surfactant protein D (SP-D) and CC-chemokine ligand 18 (CCL18), for the course of systemic sclerosis (SSc)-related interstitial lung disease. The pneumoproteins were determined in the baseline plasma samples of 266 patients with early SSc enrolled in the GENISOS observational cohort. They also were measured in 83 followup patient samples. Pulmonary function tests were obtained annually. The primary outcome was decline in forced vital capacity (FVC percentage predicted) over time. The predictive significance for longterm change in FVC was investigated by a joint analysis of longitudinal measurements (sequentially obtained FVC percentage predicted) and survival data. SP-D and CCL18 levels were both higher in patients with SSc than in matched controls (p < 0.001 and p = 0.015, respectively). Baseline SP-D levels correlated with lower concomitantly obtained FVC (r = -0.27, p < 0.001), but did not predict the short-term decline in FVC at 1 year followup visit or its longterm decline rate. CCL18 showed a significant correlation with steeper short-term decline in FVC (p = 0.049), but was not a predictor of its longterm decline rate. Similarly, a composite score of SP-D and CCL18 was a significant predictor of short-term decline in FVC but did not predict its longterm decline rate. Further, the longitudinal change in these 2 pneumoproteins did not correlate with the concomitant percentage change in FVC. SP-D correlated with concomitantly obtained FVC, while CCL18 was a predictor of short-term decline in FVC. However, neither SP-D nor CCL18 was a longterm predictor of FVC course in patients with early SSc.

Lung fluid regulation at high‐altitude

BackgroundWhether lung fluid accumulates in acclimating individuals at high altitude (HA) remains controversial.PurposeTo assess the effect of HA on lung fluid regulation.Methods/ResultsSystolic pulmonary artery pressure (sPAP) (echo), lung diffusing capacity for CO (DLCO), pulmonary capillary blood volume (Vc), alveolar‐capillary conductance (Dm) (single breath method), ultrasound lung comets (ULCs) and pulmonary function (PFTs) were assessed in 8 healthy adults at sea‐level and after 1 and 9 days at Mt. Everest Base Camp (EBC), Nepal (5150 m). Also, after 5 days at EBC, DLCO and PFTs were assessed in 5 subjects pre‐and post‐acute albuterol administration. EBC was reached after an 8 d hike at progressively increasing altitudes starting at 2800 m. Altitude exposure increased sPAP, had no effect on DLCO, increased Dm and decreased Vc with a subsequent increase in Dm/Vc, and decreased the number of ULCs (Table 1). There was no change in FVC or FEV1 but an increase in MEF50. Albuterol administration increased PEF (11.85±1.52 vs. 12.40±1.45 L/s) and MEF50 (4.85±0.77 vs. 6.00±1.30 L/s) but had no effect on DLCO, Dm or Dm/Vc.ConclusionThese data suggest that lung fluid is decreased in acclimating healthy humans at high altitude.Funding:Mayo Clinic &amp; The North Face.

Pulmonary Functions after Injection Sclerotherapy of Esophageal Varices and after Band Ligation

Aim of the study: To study changes in pulmonary function tests after esophageal variceal injection sclerotherapy in comparison to changes after esophageal variceal band ligation. Patients & methods: This study was designed as non randomized controlled study. Thirty patients with hepatic cirrhosis & portal hypertension who were admitted to Hepatology, Gastroenterology and Infectious Diseases department , Benha university hospital for elective esophageal variceal therapy (secondary prophylaxis) were classified into group I:15 patients treated with sclerotherapy by using 5-15 ml ethanolamine oleate and group II:15 patients treated with band ligation. Patients were subjected for full clinical evaluation including history, general, chest, and abdominal examination. Laboratory and radiological investigation including complete blood count, liver function tests, kidney function tests and blood sugar and plain chest x-ray (P.A. and lateral views) were done. Pulmonary function tests and arterial blood gasses were done before, one day after and one week after the procedure. Results: The results revealed , significant decrease in vital capacity in group I one day after injection ( p ˂ 0.05),but no significant decrease in vital capacity in group II. There was a significant decrease in PaO2 in group I one day after injection sclerotherapy ( p ˂ 0.05) but no significant changes in group II and no significant changes in PaCO2 in both groups one day after the procedures. The study has also revealed no significant changes in FVC,FEV1/FVC and PaCO2 in group I and group II after procedures. The study revealed significant linear correlation between the decreases in PaO2 and the percentage decrease in vital capacity( p ˂ 0.05). One week after procedure, pulmonary functions that has been affected in group I returned back to baseline values. Conclusions: esophageal injection sclerotherapy produces restrictive defect (decrease in PaO2 and vital capacity) in pulmonary functions one day after injection and improved within one

Pregnancy after Lung Transplantation: A Single Center Experience

Purpose Advances in lung transplantation have enabled women to successfully undertake pregnancies. We maintain they be at least 2 years post transplant and complication free before pregnancy. This study explores maternal outcomes in pregnancy, including changes in lung and renal function, and calcineurin inhibitor (CNI) levels. Methods and Materials A retrospective audit of lung transplants in Newcastle was undertaken. We identified patients who had ever become pregnant; manual reviews of their medical records were completed. Results of spirometry, serum creatinine, CNI doses and trough levels, and comorbidities were collected. Changes in spirometry were compared using a student t-test; p-values Results There have been 16 pregnancies (complete records available for 12), with 7 live births (44% success rate), amongst 13 patients. Six patients have since died, with one death occurring early post-pregnancy. 5 pregnancies were unplanned, with only one resulting in birth. In all, 5 pregnancies ended with spontaneous miscarriage, while 2 were terminated for medical reasons (one ectopic, one for unstable BOS preconception). Mean age at time of pregnancy was 30.4 years (range 22–39), and mean time from transplant was 35.9 months (range 26–139). Complications included preeclampsia (2, 17%), diabetes of pregnancy (1, 8%), and abnormal liver enzymes (1, 8%). Within six months of delivery, there were 2 pneumonias (17%), 2 cases of BOS (17%), one case of TB (8%) and one case of mild acute rejection (8%). Mean change in FEV1 during full-term pregnancies was -2.4% (range -8.7% to 12.4%, p=0.42) and mean change in FVC was +2.7% (range -1.7% to 11.2%, p=0.55). FEV1 was stable 3 and 12 months after pregnancy (-0.3%, p=0.55 and +2.5%, p=0.69). In the first trimester, 60% had a fall in creatinine and a universal fall in CNI trough levels (mean change -42%) was seen. Conclusions In carefully selected patients, pregnancy after lung transplant can be successful. However, complications are common and close monitoring of immunosuppression levels and renal function are needed.

Diagnosis of Restrictive Allograft Syndrome (RAS) without Using Total Lung Capacity

Purpose RAS is a form of chronic lung allograft dysfunction (CLAD) distinct from bronchiolitis obliterans syndrome (BOS). The major limitation of the original diagnostic criteria depending on total lung capacity (TLC) is the need for plethysmography rather than simple spirometry. We intended to seek for the use of spirometry in the diagnosis of RAS. Methods and Materials CLAD was diagnosed based on a persistent FEV1 decline Results According to TLC, 46 patients showed RAS phenotype and 137 showed BOS. Although the %change in FVC compared with baseline showed significant difference between BOS and RAS ( Figure 1A ) (mean±SD: BOS, −17±18.9%; RAS, −43.9±16.5%; P Figure 1B ). Twice as much %decline in FEV1 as in FVC was found to be a simple good separator of RAS and BOS, with sensitivity of 91.3% and specificity of 78.8%. Conclusions Although FVC alone cannot well distinguish RAS from BOS, “ΔFVC(%)/ΔFEV1(%)>1/2”is a sensitive indicator distinguishing RAS from BOS. In combination with other characteristics of RAS including persistent interstitial reticular shadow, radiographically evident graft volume loss, and late-onset diffuse alveolar damage in transbronchial biopsies, precise diagnosis of RAS is expected to be made without measuring TLC.

A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis

We reviewed our experience with immunosuppressive agents in patients with steroid-resistant Interstitial Lung Disease in the setting of Polymyositis/Dermatomyositis (PM/DM-ILD) to determine whether there were major differences in outcomes. We identified all patients treated for PM/DM-ILD and assessed cyclophosphamide (CYC), azathioprine (AZA) and mycophenolate (MMF) when used as first-line steroid sparing therapy for effects on pulmonary function variables, dyspnea and tolerance at six and twelve months. Among 46 patients meeting the inclusion criteria, 24 were treated with CYC, 13 with AZA and 9 with MMF. There were no baseline differences between the three treatment groups for any of the demographic or physiologic variables, dyspnea score, the presence of >30% fibrosis on CT, or the baseline steroid dose. At the six months assessment, the overall median change in FVC was 5.0% (25th, 75th percentile -3, 11.5%), corresponding to +.20L (.09, 0.42L) and the DLCO increased by 2.93% (-4, 9%), corresponding to 1mm/ml/Hg (-.58, 2.3). The severity of dyspnea decreased substantially, prednisone dose could be reduced and no important difference in side effects was found in the whole group of patients. This effect was sustained after twelve months of therapy. In patients with PM/DM-ILD related, treatment with CYC, AZA or MMF was associated with stabilization of pulmonary physiology, improved dyspnea, and a reduction of steroid dose.

English

PURPOSE: Salbutamol is the most common β2-agonist being currently used in the treatment of asthma. It was only available as a racemate, a 50:50 mixture of the dextro [(S)-salbutamol] and levo [(R)-salbutamol] rotatory forms. Regular and excessive use of racemate can induce paradoxical reactions in some subjects with asthma. The present study, compared MDI Salbutamol with levo [(R)-salbutamol] isomer over 8 weeks in terms of clinical efficacy and safety. METHODS: A prospective, parallel design, comparative study lasting eight weeks, involving 80 patient of either sex after taking their informed consent. Patients were randomly divided into 2 groups, A and B of 40 patients each. Patients in Group A and Group B were administered Salbutamol MDI two puffs 100 mcg per puff four times daily and Levosalbutamol MDI two puffs each containing 50 mcg four times daily respectively. Rescue dose two or more puffs was allowed as required and the number of additional doses were counted by patients. Observations were made and analyzed at 0, 4 and 8 weeks for FEV1, FVC, and PEFR and side effects. RESULTS: Highly significant (p < 0.001) mean percent change in FEV1, FVC and PEFR at 4 and 8 weeks of Group B (Levosalbutamol) patients as compared to mean percent change in FEV1 of Group A (Salbutamol) patients at 4 weeks and 8 weeks. CONCLUSION: High therapeutic efficacy was observed with the use of the Levosalbutamol MDI with lesser side effects. Clinical Implications: Levosalbutamol can be preferred over salbutamol for better control of

Pulmonary function in children and young adults with ataxia telangiectasia

Pulmonary disease contributes to significant morbidity and mortality in people with ataxia telangiectasia (A-T). To determine the association between age and lung function in children and young adults with A-T and to identify factors associated with decreased lung function, pulmonary function tests were performed in 100 consecutive people with A-T. Children and adults ranging from 6 to 29 years of age and with the diagnosis of A-T were recruited, and underwent pulmonary function tests. The mean forced vital capacity % predicted (FVC %) in the population was 56.6 ± 20.0. Males and females between 6 and 10 years of age had similar pulmonary function. Older females were found to have significantly lower FVCs % than both older males (P < 0.02) and younger females (P < 0.001). The use of supplemental gamma globulin was associated with significantly lower FVC %. A modest correlation was found between higher radiation-induced chromosomal breakage and lower FVC % in males. No significant change in FVC % was found in a subset of subjects (n = 25) who underwent pulmonary function testing on two or more occasions over an average of 2 years. In children and young adults with A-T, older females and people who required supplemental gamma globulin had significantly lower lung function by cross-sectional analysis. Stable lung function is possible over a 2-year period. Recognition of groups who are at higher risk for lower pulmonary function may help direct care and improve clinical outcomes in people with A-T.

Alterações espirométricas em doenças obstrutivas: afinal, o quanto é relevante?

OBJECTIVE: To establish the upper limits for changes in FEV1, slow vital capacity (SVC), FVC, and inspiratory capacity (IC) after placebo administration in patients with airflow obstruction. METHODS: One hundred and two adults with airflow obstruction (FEV1 = 62 ± 19% of predicted) were included in the study. All of the participants performed SVC and FVC maneuvers before and after the administration of placebo spray. The changes in FEV1, SVC, FVC, and IC were expressed as absolute values, percentage of change from baseline values, and percentage of predicted values, 95% CIs and 95th percentiles being calculated. Factor analysis was performed in order to determine how those changes clustered. RESULTS: Considering the 95% CIs and 95th percentiles and after rounding the values, we found that the upper limits for a significant response were as follows: FEV1 = 0.20 L, FVC = 0.20 L, SVC = 0.25 L, and IC = 0.30 L (expressed as absolute values); FEV1 = 12%, FVC = 7%, SVC = 10%, and IC = 15% (expressed as percentage of change from baseline values); and FEV1 = 7%, FVC = 6%, SVC = 7%, and IC = 12% (expressed as percentage of predicted values). CONCLUSIONS: In patients with airflow obstruction, IC varies more widely than do FVC and SVC. For IC, values greater than 0.30 L and 15% of change from the baseline value can be considered significant. For FVC, values greater than 0.20 L and 7% of change from the baseline value are significant. Alternatively, changes exceeding 0.20 L and 7% of the predicted value can be considered significant for FEV1 and FVC. On factor analysis, spirometric parameters clustered into three dimensions, expressing changes in flows, volumes, and dynamic hyperinflation.

Effect and Safety of Mycophenolate Mofetil in Chronic Pulmonary Sarcoidosis: A Retrospective Study

Background: Treatment of chronic pulmonary sarcoidosis (CPS) is challenging and often requires long-term therapy with systemic corticosteroids and supplementary use of steroid-sparing agents. Objective: To examine the efficiency and safety of mycophenolate mofetil (MMF) as a steroid-sparing agent in patients with CPS. Methods: We conducted a retrospective study of patients with biopsy-proven pulmonary sarcoidosis, who were treated with MMF and systemic corticosteroids for >6 months between 2004 and 2010. Corticosteroid dose, pulmonary function parameters and radiological and clinical follow-up before and after treatment with MMF were assessed. Results: Ten patients received MMF for >6 months. MMF was introduced due to side effects (5/10 patients) and due to an inadequate response to prior therapy (5/10 patients). Median duration of treatment with MMF was 31 months (range 8-66). Therapeutic MMF plasma trough levels of 1-3 mg/l were reached with daily doses of 1,722 ± 440 mg MMF. Daily corticosteroid dose could be significantly reduced from 14.3 ± 13.3 to 6.5 ± 2.3 mg prednisolone during treatment. During follow-up, pulmonary function, symptoms and radiological signs improved in 4 patients, while 6 patients remained stable. Median change in FVC was +8.5 % (range -2 to 16). No severe adverse events that were related to combined immunosuppressive therapy occurred. Conclusion: This study indicates that the addition of MMF to corticosteroids is a viable and safe treatment option in CPS. MMF allows a significant reduction of maintenance corticosteroids to levels <10 mg/day while preserving a stable or improved clinical condition.