Asthma-COPD overlap szindróma

Absztrakt Az obstruktív tüdőbetegségek világszerte jelentős népegészségügyi problémát jelentenek, magas prevalenciájuk és a társadalmat érintő nagy szocioökonómiai költségeik miatt. Az asthma bronchiale és a krónikus obstruktív tüdőbetegség krónikus légúti gyulladással járó betegségek, amelyeket légúti obstrukció jellemez, azonban külön nozológiai entitások, figyelembe véve kialakulásukat, diagnosztikájukat, a terápiás megfontolásokat és prognosztikai jellemzőiket. A két betegség együttesen is előfordulhat, vagy az egyik állapot képes a másik jellemzőit felvéve, abba átalakulni. Ezt a kevert asthma-COPD fenotípust nevezzük asthma-COPD overlap szindrómának, amelyet úgy jellemeznek, hogy olyan perzisztáló légúti áramláskorlátozottsággal járó betegség, amely rendelkezik mind az asthma, mind a krónikus obstruktív tüdőbetegség egyes jellemzőivel. A szindróma az elmúlt években jelentős figyelmet kapott, azonban a betegek nagy része mostanáig kizárásra került a klinikai és légúti gyógyszer-hatásossági vizsgálatokból, így a jól meghatározott klinikai jellemzők és a megfelelő terápia továbbra sem ismertek. Az összefoglaló közlemény elsődleges célja az elmúlt években leírt patofiziológiai és klinikai jellemzők, terápiás megfontolások és irodalmi újdonságok bemutatása. Orv. Hetil., 2016, 157(33), 1304–1313. | Abstract Obstructive lung diseases represent a major health problem worldwide due to their high prevalence associated with elevated socioeconomic costs. Bronchial asthma and chronic obstructive pulmonary disease are chronic obstructive ventilatory disorders with airway inflammation, however they are separate nosological entities based on thedifferent development, diagnostic and therapeutic approaches, and prognostic features. However, these diseases may coexist and can be defined as the coexistence of increased variability of airflow in a patient with incompletely reversible airway obstruction. This phenotype is called asthma – chronic obstructive pulmonary disease overlap syndrome. The syndrome is a clinical and scientific challenge as the majority of these patients have been excluded from the clinical and pharmacological trials, thus well-defined clinical characteristics and therapeutic approaches are lacking. The aim of this review is to summarize the currently available literature focusing on pathophysiological and clinical features, and discuss possible therapeutic approaches of patients with asthma – chronic obstructive pulmonary disease overlap syndrome. Orv. Hetil., 2016, 157(33), 1304–1313

A szisztémás lupus erythematosus pulmonalis manifesztációi

Absztrakt A kötőszöveti betegségek tekintetében a pulmonalis manifesztáció megjelenése szisztémás lupus erythematosusban az egyik leggyakoribb. Lupusban bármely szervrendszer érintett lehet, mégis a tüdőérintettség az esetek döntő többségében kialakul, és jelentős hatással van a betegség lefolyására. Szisztémás lupus erythematosusban a pulmonalis manifesztációkat az anatómiai érintettség alapján öt csoportba lehet sorolni: pleura, tüdőparenchyma, bronchusok és bronchiolusok, pulmonalis vasculatura, illetve a légzőizmok érintettsége. A leggyakoribb pulmonalis érintettség a pleuritis, amely pleuralis folyadékkal vagy a nélkül is előfordulhat, gyakori a pulmonalis vascularis és a felső és alsó légúti érintettség, a tüdőparenchyma-eltérések és a diaphragmadiszfunkció (shrinking lung szindróma). A szerzők a közlemény kapcsán a lupusban szenvedő betegek pulmonalis eltéréseire, ezek diagnosztikájára, a kezelésükre és a prognózisra szeretnék felhívni a figyelmet. Orv. Hetil., 2016, 157(29), 1154–1160

The repeatability of inspiration performance through different inhalers in patients with chronic obstructive pulmonary disease and control volunteers

Background: Inhalation therapy is a cornerstone of treating patients with chronic obstructive pulmonary disease (COPD). Inhaler types and through-device inhalation parameters influence airway drug delivery. We aimed to measure the repeatability of inhalation performance through four different commercially available inhalers. Methods: We recruited control subjects (n = 22) and patients with stable COPD (S-COPD, n = 16) and during an acute exacerbation (AE-COPD, n = 15). Standard spirometry was followed by through-device inhalation maneuvers using Ellipta(®), Evohaler(®), Respimat(®), and Genuair(®). Through-device inspiratory vital capacity (IVC(d)) and peak inspiratory flow (PIF(d)), as well as inhalation time (t(in)) and breath hold time (t(bh)), were recorded and all measurements were repeated in a random manner. Results: There was no difference in forced expiratory volume in 1 second (FEV(1)) between patients (S-COPD: 39 ± 5 vs. AE-COPD: 32% ± 5% predicted, p > 0.05). In controls, the IVC(d) was significantly reduced by all four devices in comparison with the slight reduction seen in COPD patients. In all subjects, PIF was lowered when inhaling through the devices in order of decreasing magnitude in PIF(d): Evohaler, Respimat, Ellipta, and Genuair. The Bland-Altman analysis showed a highly variable coefficient of repeatability for IVC(d) and PIF(d) through the different inhalers for all COPD patients. Based on the intermeasurement differences in patients, Respimat and Genuair showed the highest repeatability for IVC(d), while Genuair and Ellipta performed superior with regard to PIF(d). Conclusions: Our study is the first to compare repeatability of inhalation performances through different inhalers in COPD patients, showing great individual differences for parameters influencing lung deposition of inhaled medication from a given device. Our data provide new insight into the characterization of inhaler use by patients with COPD, and might aid the selection of the most appropriate devices to ensure the adequate and consistent delivery of inhaled drugs

Vitamin D deficiency is associated with impaired disease control in asthma-COPD overlap syndrome patients

INTRODUCTION: The association between vitamin D and clinical parameters in obstructive lung diseases (OLDs), including COPD and bronchial asthma, was previously investigated. As asthma-COPD overlap syndrome (ACOS) is a new clinical entity, the prevalence of vitamin D levels in ACOS is unknown. AIM: Our aim was to assess the levels of circulating vitamin D (25-hydroxyvitamin D [25(OH)D]) in different OLDs, including ACOS patients, and its correlation with clinical parameters. METHODS: A total of 106 men and women (control, n=21; asthma, n=44; COPD, n=21; and ACOS, n=20) were involved in the study. All patients underwent detailed clinical examinations; disease control and severity was assessed by disease-specific questionnaires (COPD assessment test, asthma control test, and modified Medical Research Council); furthermore, 25(OH)D levels were measured in all patients. RESULTS: The 25(OH)D level was significantly lower in ACOS and COPD groups compared to asthma group (16.86+/-1.79 ng/mL and 14.27+/-1.88 ng/mL vs 25.66+/-1.91 ng/mL). A positive correlation was found between 25(OH)D level and forced expiratory volume in 1 second (r=0.4433; P<0.0001), forced vital capacity (FVC) (r=0.3741; P=0.0004), forced expiratory flow between 25% and 75% of FVC (r=0.4179; P<0.0001), and peak expiratory flow (r=0.4846; P<0.0001) in OLD patient groups. Asthma control test total scores and the 25(OH)D level showed a positive correlation in the ACOS (r=0.4761; P=0.0339) but not in the asthma group. Higher COPD assessment test total scores correlated with decreased 25(OH)D in ACOS (r=-0.4446; P=0.0495); however, this was not observed in the COPD group. CONCLUSION: Vitamin D deficiency is present in ACOS patients and circulating 25(OH)D level may affect disease control and severity

Bentall procedure: quarter century of clinical experiences of a single surgeon

BACKGROUND: We retrospectively analyzed 25 years of experiences with the button Bentall procedure in patients with aortic root pathologies. Even though this procedure has become widespread, there are only a few very long term follow-ups available in the clinical literature, especially regarding single surgeon results. METHODS: Between 1988 and 2013, a total of 147 patients underwent the Bentall procedure by the same surgeon. Among them there were 62 patients with Marfan syndrome. At the time of the surgery the mean age was 46.5 +/- 17.6 years. The impact of surgical experience on long-term survival was evaluated using a cumulative sum analysis chart. RESULTS: The Kaplan-Meier estimated overall survival rates for the 147 patients were 91.8 +/- 2.3 %, 84.3 +/- 3.1 %, 76.3 +/- 4.9 % and 59.5 +/- 10.7 % at 1,5,10 and 20 years, respectively. Multivariate Cox regression analysis identified EuroSCORE II over 3 % (OR 4.245, 95 % CI, 1.739-10.364, p = 0.002), acute indication (OR 2.942, 95 % CI, 1.158-7.480, p = 0.023), use of deep hypothermic circulatory arrest (OR 3.267, 95 % CI, 1.283-8.323, p = 0.013), chronic kidney disease (OR 6.865, 95 % CI, 1.339-35.189, p = 0.021) and early complication (OR 3.134, 95 % CI, 1.246-7.883, p = 0.015) as significant risk factors for the late overall death. The survival rate for freedom from early complication was 94.3 +/- 2.2 %, 88.0 +/- 3.3 %, 82.9 +/- 4.7 % and 69.2 +/- 8.4 % at 1,5,10 and 20 years. The main pathological findings of the aortic wall were cystic medial degeneration in 75 %, fibrosis in 6 %, atherosclerosis in 13 % and no pathological alteration in 6 % of the samples. The overall survival rate was significantly lower in patients operated in first 15 years compared to patients operated in the last decade (log-rank p = 0.011). CONCLUSION: According to our long-term follow-up the Bentall operation provides an appropriate functional result by resolving the lesions of the ascending aorta. Based on our results, 25-30 operations done is necessary to gain such a level of confidence and experince to aquire better results on long-term survival. In addition, we discussed that there were no co-morbidities affecting on the survival of Marfan patients and prophylactic aortic root replacement ensures a longer survival among patients with Marfan syndrome

Characteristics of reversible and nonreversible COPD and asthma and COPD overlap syndrome patients: an analysis of salbutamol Easyhaler data

The choice of inhaler device for bronchodilator reversibility is crucial since suboptimal inhalation technique may influence the result. On the other hand, bronchodilator response also varies from time to time and may depend on patient characteristics. In this study, patients with airway obstruction (forced expiratory volume in 1 second [FEV1]/forced vital capacity [FVC] ratio <70% in chronic obstructive pulmonary disease [COPD]; <80% in asthma) were included (n=121, age: 57.8+/-17.3 years). Bronchodilator reversibility (American Thoracic Society/European Respiratory Society criteria) was tested in patients with COPD (n=63) and asthma and COPD overlap syndrome (ACOS; n=12). Forty-six asthmatics served as controls. Reversibility was tested with 400 microg salbutamol dry powder inhaler (Buventol Easyhaler, Orion Pharma Ltd, Espoo, Finland). Demographic data and patients' perceptions of Easyhaler compared with beta2-agonist pressurized metered dose inhalers (pMDIs) were analyzed. American Thoracic Society/European Respiratory Society guideline defined reversibility was found in 21 out of 63 COPD patients and in two out of 12 ACOS patients. Airway obstruction was more severe in COPD patients as compared with controls (mean FEV1 and FEV1% predicted both P<0.0001). Average response to salbutamol was significantly lower in COPD patients compared with asthma controls (P<0.0001). Reversibility was equally often found in smokers as in never-smokers (33% vs 34%). Nonreversible COPD patients had higher mean weight, body mass index, and FEV1/FVC compared with reversible COPD patients. Most patients preferred Easyhaler and defined its use as simpler and more effective than use of a pMDI. Never-smokers and patients with asthma experienced Easy-haler somewhat easier to use than smokers and patients with COPD. In conclusion, a substantial part of patients with COPD or ACOS showed reversibility to salbutamol dry powder inhaler. Nonreversible patients with COPD were characterized by higher weight and body mass index, and a higher FEV1/FVC ratio. Most patients preferred Easyhaler compared with a pMDI

Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery

Marfan syndrome is a genetic disorder affecting the connective tissue. Changes in lung tissue might influence respiratory function; however, a detailed respiratory functional assessment according to the need for major thoracic surgery is missing.Comprehensive pulmonary examinations were performed in 55 Marfan patients including respiratory symptoms, lung function (LF) testing using European Coal and Steel Community (ECSC) reference values, TLCO and quality of life measurements. Groups included patients who did not need surgery (Mf, n = 32) and those who underwent major thoracic surgery (Mfop, n = 23).Respiratory symptoms affected 20% of patients. Scoliosis was significantly more frequent in the Mfop group. LF demonstrated in all Marfan patients a tendency towards airway obstruction (FEV1/FVC = 0.77 ± 0.10), more prominent in Mfop patients (0.74 ± 0.08 vs. Mf: 0.80 ± 0.11; p = 0.03). Correction of LF values using a standing height modification by arm span (Hcorrected) revealed additional changes in FVC and FEV1. TLCO and quality of life did not differ between groups.Marfan syndrome is associated with airway obstruction, especially in patients who have undergone major thoracic surgery, indicative of more severe connective tissue malfunction. The use of arm span for height correction is suitable to evaluate LF changes in this special patient group including patients with significant scoliosis

Possible extracardiac predictors of aortic dissection in Marfan syndrome

BACKGROUND: According to previous studies, aortic diameter alone seems to be insufficient to predict the event of aortic dissection in Marfan syndrome (MFS). Determining the optimal schedule for preventive aortic root replacement (ARR) aortic growth rate is of importance, as well as family history, however, none of them appear to be decisive. Thus, the aim of this study was to search for potential predictors of aortic dissection in MFS. METHODS: A Marfan Biobank consisting of 79 MFS patients was established. Thirty-nine MFS patients who underwent ARR were assigned into three groups based on the indication for surgery (dissection, annuloaortic ectasia and prophylactic surgery). The prophylactic surgery group was excluded from the study. Transforming growth factor-beta (TGF-beta) serum levels were measured by ELISA, relative expression of c-Fos, matrix metalloproteinase 3 and 9 (MMP-3 and -9) were assessed by RT-PCR. Clinical parameters, including anthropometric variables - based on the original Ghent criteria were also analyzed. RESULTS: Among patients with aortic dissection, TGF-beta serum level was elevated (43.78 +/- 6.51 vs. 31.64 +/- 4.99 ng/l, p < 0.0001), MMP-3 was up-regulated (Ln2alpha = 1.87, p = 0.062) and striae atrophicae were more common (92% vs. 41% p = 0.027) compared to the annuloaortic ectasia group. CONCLUSIONS: We found three easily measurable parameters (striae atrophicae, TGF-beta serum level, MMP-3) that may help to predict the risk of aortic dissection in MFS. Based on these findings a new classification of MFS, that is benign or malignant is also proposed, which could be taken into consideration in determining the timing of prophylactic ARR