A Real-World Multicenter Retrospective Observational Study on Polish Experience with Nintedanib Therapy in Patients with Idiopathic Pulmonary Fibrosis: The PolExNIB Study.

Nintedanib is a disease-modifying agent licensed for the treatment of IPF. Data on Polish experience with nintedanib in IPF are lacking. The present study aimed to describe the safety and efficacy profiles of nintedanib in a large real-world cohort of Polish patients with IPF. This was a multicenter, retrospective, observational study of IPF patients treated with nintedanib between March 2018 and October 2021. Data collection included baseline clinical characteristics, results of pulmonary function tests (PFTs), and a six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), and treatment persistence were also retrieved. A total of 501 patients (70% male) with a median age of 70.9 years (IQR 65-75.7) were included in this study. Patients were followed on treatment for a median of 15 months (7-25.5). The majority of patients (66.7%) were treated with the full recommended dose of nintedanib and 33.3% of patients were treated with a reduced dose of a drug. Intermittent dose reductions or drug interruptions were needed in 20% of patients. Over up to 3 years of follow-up, pulmonary function remained largely stable with the minority experiencing disease progression. The most frequent ADRs included diarrhea (45.3%), decreased appetite (29.9%), abdominal discomfort (29.5%), weight loss (32.1%), nausea (20.8%), fatigue (19.2%), increased liver aminotransferases (15.4%), and vomiting (8.2%). A total of 203 patients (40.5%) discontinued nintedanib treatment due to diverse reasons including ADRs (10.2%), death (11.6%), disease progression (4.6%), patient's request (6.6%), and neoplastic disease (2.2%). This real-world study of a large cohort of Polish patients with IPF demonstrates that nintedanib therapy is safe, and is associated with acceptable tolerance and disease stabilization. These data support the findings of previously conducted clinical trials and observational studies on the safety and efficacy profiles of nintedanib in IPF.

Evaluation of prognostic factors for patients with eosinophilic granulomatosis with polyangiitis recruited at the pneumonological centre and mainly ANCA negativity: A retrospective analysis of a single cohort in Poland.

OBJECTIVES: The aim was to investigate the risk factors for relapse and death in patients with eosinophilic granulomatosis with polyangiitis (EGPA) recruited at the pneumonological centre and mainly antineutrophil cytoplasmic antibody negativity. METHODS: We retrospectively recruited 86 patients. Relapse was defined as the recurrence or appearance of new organ symptoms. The study end-point included the final examination. RESULTS: Relapses occurred in 34.9% of the patients, while 9.3% died. Immunosuppressive therapy (P = 0.042), prolonged low-dose corticosteroid treatments (mainly for asthma) (P = 0.006), and longer follow-up duration (P = 0.004) were associated with a higher relapse risk, while advanced EGPA severity (P = 0.0015) and activity (P = 0.044), older age of onset (P = 0.030), symptomatic cardiac involvement (P = 0.007), and postinflammatory cardiac fibrosis (P = 0.038) were associated with a higher risk of death. Sinusitis (P = 0.028) and prolonged low-dose corticosteroid treatments (P = 0.025) correlated with a better prognosis. Relapses did not have an impact on the mortality (P = 0.693). CONCLUSIONS: Relapses in EGPA remain frequent, although they do not impact mortality. Cardiac involvement is common, but clinically symptomatic cardiomyopathy is associated with a higher risk of death. Asthma requiring chronic corticosteroid treatments is associated with a lower risk of death, although the risk of EGPA recurrence is significantly higher.

The role of peripheral eosinophilia in diagnosing lung disorders: experience from a single pneumonological center.

BACKGROUND: Eosinophilia is rare but one of the important reasons to refer patients to pneumonological centers. Determining etiology of eosinophilia has practical implications for therapeutic intervention and disease prognosis. The study aimed to determine the role of peripheral eosinophilia in the diagnosis of lung disorders. METHODS: In this retrospective study were included 46 patients diagnosed with peripheral eosinophilia with coexisting respiratory symptoms and/or radiologically detected lung lesions. All patients underwent standard diagnostic procedures, including a detailed clinical history review, physical examination, routine laboratory tests with basal cardiological examinations, and serological tests to detect parasites and allergies. Other procedures carried out depended on the symptoms of each patient. The relation between eosinophil counts in the blood and patients' clinical manifestation was investigated to identify the degree of eosinophilia requiring immediate diagnostic procedures and treatment. Statistical analyses were performed using scientific computation libraries in the Python programming language, SciPy, v. 1.3.1. Briefly, the following tests were used: parametric Kruskal-Wallis H test, an independent t-test, ANOVA, the Shapiro- Wilk test, Fisher's and Chi-squared tests, and the Holm-Bonferroni method. RESULTS: Severe eosinophilia (≥5,000 cells/µl) was associated with extrapulmonary involvement and constitutional symptoms. Skin, heart, and pleural diseases were more frequent in these patients than in patients with mild or moderate eosinophilia (p=0.010, p=0.040, and p=0.007, respectively), and only these patients showed signs of kidney disease (p=0.006). Vasculitis was significantly more frequent in the severe eosinophilia group (p=0.048) than in the other two groups. In patients with moderate eosinophilia (1,500-5,000 cells/µl), extrapulmonary symptoms were less common, although signs of cardiac involvement were confirmed in 44% of subjects. In this group, vasculitis was the most commonly observed disease (42% of cases). Mild eosinophilia (<1,500 cells/µl) was mainly associated with airway disease. In this group, vasculitis and interstitial lung diseases were identified, but most were not typically connected with eosinophilia. CONCLUSIONS: Identification of peripheral eosinophilia may essentially determine diagnostic procedures in patients with lung disorders and may be a useful indicator of disease etiology.

A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study.

BACKGROUND: Pirfenidone is an antifibrotic agent approved for the treatment of idiopathic pulmonary fibrosis (IPF). The drug is available for Polish patients with IPF since 2017. The PolExPIR study aimed to describe the real-world data (RWD) on the Polish experience of pirfenidone therapy in IPF with respect to safety and efficacy profiles. METHODS: This was a multicentre, retrospective, observational study collecting clinical data of patients with IPF receiving pirfenidone from January 2017 to September 2019 across 10 specialized pulmonary centres in Poland. Data collection included baseline characteristics, pulmonary function tests (PFTs) results and six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), treatment persistence, and survival were also collected up to 24 months post-inclusion. RESULTS: A total of 307 patients receiving pirfenidone were identified for analysis. The mean age was 68.83 (8.13) years and 77% were males. The median time from the first symptoms to IPF diagnosis was 15.5 (9.75-30) months and from diagnosis to start of pirfenidone treatment was 6 (2-23) months. Patients were followed on treatment for a median of 17 (12-22.75) months. Seventy-four patients (24.1%) required dose adjustments and 35 (11.4%) were chronically treated with different than the full recommended dose. A total of 141 patients (45.92%) discontinued therapy due to different reasons including ADRs (16.61%), death (8.79%), disease progression (6.51%), patient's own request (5.54%), neoplastic disease (3.91%) and lung transplantation (0.33%). Over up to 24 months of follow-up, the pulmonary function remained largely stable. The median annual decline in forced vital capacity (FVC) during the first year of pirfenidone therapy was -20 ml (-200-100) and during the second year was -120 ml (-340-30). Over a study period, 33 patients (10.75%) died. CONCLUSIONS: The PolExPIR study is a source of longitudinal RWD on pirfenidone therapy in the Polish cohort of patients with IPF supporting its long-term acceptable safety and efficacy profiles and reinforce findings from the previous randomised clinical trials and observational studies.

Cardiac sarcoidosis: worse pulmonary function due to left ventricular ejection fraction?: A case-control study.

Dyspnea and exercise intolerance are usually attributed to pulmonary disease in sarcoidosis patients. However, cardiac involvement may also be responsible for these symptoms. Data regarding the impact of heart involvement on lung function in cardiac sarcoidosis (CS) is limited.The aim of study was to compare the results of pulmonary function tests (PFTs) in patients with and without heart involvement. We performed a retrospective analysis of PFTs in a group of sarcoidosis patients both with and without heart involvement evaluated by cardiovascular magnetic resonance (CMR) study. The study was performed in the period between May 2008 and April 2016.We included data of sarcoidosis patients who underwent testing for possible CS (including CMR study) at a national tertiary referral center for patients with interstitial lung diseases. All patients had histopathologicaly confirmed sarcoidosis and underwent standard evaluation with PFTs measurements including spirometry, plethysmography, lung transfer factor (TL,CO), and 6-minute walking test (6MWT) assessed using the most recent predicted values.We identified 255 sarcoidosis patients (93 women, age 42 ±â€Š10.7 y): 103 with CS and 152 without CS (controls). CS patients had significantly lower left ventricular ejection fraction (LVEF; 56.9 ±â€Š7.0 vs 60.4 ±â€Š5.4, P < .001). Any type of lung dysfunction was seen in 63% of CS patients compared with 31% in the controls (P = .005). Ventilatory disturbances (obstructive or restrictive pattern) and low TL,CO were more frequent in CS group (52% vs 23%, P < .001 and 38% vs 18% P < .01 respectively). CS (OR = 2.13, 95% CI: 1.11-4.07, P = .02), stage of the disease (OR = 3.13, 95% CI: 1.4-7.0, P = .006) and LVEF (coefficient = -0.068 ±â€Š0.027, P = .011) were independent factors associated with low FEV1 but not low TL,CO. There was a significant correlation between LVEF and FEV1 in CS group (r = 0.31, n = 89, P = .003). No significant difference in 6MWD between CS patients and controls was observed.Lung function impairment was more frequent in CS. Lower LVEF was associated with decreased values of FEV1. Relatively poor lung function may be an indication of cardiac sarcoidosis.

Difficulties in recognizing granulomatosis with polyangiitis (GPA) in elderly patients undergoing diagnostic thoracotomy twice - a report of two cases.

Granulomatosis with polyangiitis (GPA) is defined as a necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract with necrotizing vasculitis affecting predominantly small to medium vessels. Because of non-specific symptoms, its radiological presentation, and the diversity of its clinical expression, it is not uncommon to for it to be misdiagnosed, especially in the elderly. Although biopsy and histological examination seem to be essential for GPA diagnosis, their results are sometimes ambiguous and not helpful in making a decision. In this report, we present difficulties in the recognition of GPA in two elderly patients in whom, despite twice performing a diagnostic thoracotomy, GPA was recognized almost 4 and 6 years after the first symptoms.

Carcinogenesis-related changes in iron metabolism in chronic obstructive pulmonary disease subjects with lung cancer.

Chronic obstructive pulmonary disease (COPD) is often accompanied by lung cancer. In our previous work, it was observed that matrix metalloproteinase-3 and haptoglobin (HP) polymorphisms were potential markers of enhanced susceptibility to lung cancer development among male COPD subjects. Here, results are reported on blood serum levels of several proteins involved in iron metabolism, inflammation and the oxidative stress response compared between the same groups of subjects. The blood serum levels of tumor necrosis factor α (TNFα), transferrin, hepcidin, ferritin, soluble transferrin receptor and 8-oxo-2'-deoxyguanosine were compared, as well as total iron-binding capacity (TIBC) and ceruloplasmin ferroxidase activity in two groups of subjects: Male COPD patients (54 subjects) and male COPD patients diagnosed with lung cancer (53 subjects). Statistically significant differences were identified between the two groups in transferrin and TNFα levels, as well as in TIBC; all three parameters were lower in the group consisting of COPD patients diagnosed with lung cancer (P<0.01). It was also revealed that HP genotype 1/2 was concomitant with low transferrin blood level in subjects with COPD; this apparent dependence was absent in the COPD + cancer subjects. The results indicate a role of iron metabolism in the susceptibility to lung cancer in COPD-affected subjects. They also emphasize the importance of individual capacity for an effective response to oxidative stress during the pathogenic process as HP is a plasma protein that binds free hemoglobin and its polymorphism results in proteins with altered hemoglobin-binding capacity and different antioxidant and iron-recycling functions.

Pulmonary tuberculosis mimicking lung cancer progression after 10 years of cancer remission.

Differentiation between pulmonary tuberculosis and lung cancer is often challenging for clinicians, especially that both conditions can coexist. This is due to the fact that the clinical and radiological symptoms of both diseases can be similar. Our case report presents a patient who was treated for advanced lung cancer 10 years earlier and currently has been hospitalized again because of a strong clinical and radiological suspicion of the cancer progression, but whose final diagnosis was tuberculosis.

Systemic treatment for sarcoidosis was needed for 16% of 1810 Caucasian patients.

INTRODUCTION AND OBJECTIVE: Sarcoidosis is a systemic granulomatous disease predominantly affecting the lungs, although granulomas can also involve all other organs. Fortunately, it is often a self-limiting disease and aggressive treatment is not indicated in majority of cases. However, treatment is recommended when critical organs are affected or the disease is progressive. So far, there is lack of reliable information regarding the frequency of treatment in Caucasian population or data are discordant. The aim of this study was to evaluate the prevalence of systemic immune-modulating treatment in a large cohort of sarcoidosis patients. METHODS: We performed a retrospective analysis of patients discharged from our institution with the final diagnosis 'sarcoidosis' (ICD-10: D86) from January 2010 to December 2013. RESULTS: 1810 sarcoidosis patients were hospitalized during these four years, 47.6% were females, mean age was 43.5 ± 12.2 years. The majority (80.6%) were discharged as pulmonary and/or lymph node sarcoidosis (D86.0, D86.1, D86.2). Only 281 (15.5%) patients were discharged with systemic treatment, 60.1% of them were men (OR 1.5; 95% CI: 1.1-1.9, P = .0047). The 44.8% of patients who had sarcoidosis also involving an organ other than the lungs (D86.8) were much more likely to be treated: OR 5.6; 95% CI: 4.2-7.4, P < .0001). Older age (>50) was also identified as a risk factor for treatment (odds ratio: 1.8; 95% CI: 1.5-2.4). CONCLUSIONS: Less than 16% sarcoidosis patients required systemic treatment. Older men with multiorgan sarcoidosis were more likely to be treated.

Lung Function in Pregnancy in Langerhans Cell Histiocytosis.

Pulmonary Langerhans cell histiocytosis (LCH) is a rare disease, affecting usually young people. The course of the disease is variable. In some pulmonary LCH patients a severe lung destruction and progression in spite of chemotherapy is observed, but in others just a cessation of smoking induces a regression of the disease. In the present study we seek to determine the influence of pregnancy on pulmonary function in LCH patients, an unchartered area of research. We addressed the issue by investigating eight pregnant women out of the 45 women hospitalized with the diagnosis of pulmonary LCH in the period from 2000 to 2015. For five of the eight pregnant women it was the second gestation. The median follow-up period was 120 months (range 72-175 months). Ten healthy children were born by a C-section. Two spontaneous miscarriages in the seventh week of gestation, and one tubal ectopic pregnancy were recorded. We found that pregnancy did not significantly influence pulmonary function assessed by the following indices: forced expiratory volume in 1 s (FEV1), lung vital capacity (VC), total lung capacity (TLC), residual volume (RV), diffusing capacity of the lungs for carbon monoxide (DLCO), and the distance and arterial oxygen saturation in 6-min walk test. Only one patient in the third trimester of pregnancy experienced bilateral pneumothorax, with persistent air leak. In all patients, delivery and postpartum period were uneventful. We conclude that pregnancy in pulmonary LCH patients is safe and not associated with deterioration of pulmonary function or blood oxygenation.

Pulmonary langerhans cell histiocytosis - insight into the incidence of alfa-1-antitrypsin (A1ATD) deficiency alleles.

INTRODUCTION: The alpha-1 antitrypsin deficiency (A1ATD) is one of the three most common genetic disorders in Caucasians. It considerably increases the risk of progressive obstructive lung diseases, mostly chronic obstructive pulmonary disease. There is no data regarding prevalence of main, clinically most important A1ATD alleles PI*Z and PI*S in patients with pulmonary Langerhans cell histiocytosis (PLCH). PLCH is not only strongly linked to the cigarette smoking, but is also characterised by polycystic lung lesions. The goal of the study was to assess the incidence of A1ATD alleles in patients with PLCH. MATERIAL AND METHODS: Blood samples were collected from 34 adult patients (14 women and 20 men), with histologically confirmed PLCH. AAT serum concentration was assessed by nephelometry and PI-phenotype, identified by isoelectrofocusing. The PI*S and PI*Z alleles were confirmed by genotyping usisng real-time PCR. RESULTS: Deficiency alleles PI*Z and PI*S were detected in 3 patients (one woman and 2 men), respectively in 5.88% and 2.94%. The estimated incidence of deficiency alleles was 29.4/1000 (95% CI; 10-69.5) for PI*Z and 14.7/1000(95%CI; 13.9-43.3) for PI*S. According to our previous reports, the expected prevalence of PI*Z and PI*S alleles in general Polish population was 13.7/1000 (95% CI 5.8-21.5), and 7,6/1000 (95% CI 1.7-13.5) respectively. CONCLUSIONS: The incidence of main A1AT deficiency alleles in patients with PLCH seems higher than in general Polish population. The study is on-going.

Diffuse alveolar haemorrhage complicated by pulmonary embolism - problems with treatment.

Diffuse alveolar haemorrhage (DAH) refers to a clinical syndrome resulting from injury of the alveolar capillaries, arterioles and venules leading to red blood cel accumulation in the distal air spaces. The conditions associated with DAH and underlying disease determine the prognosis and the treatment regimen. The coexistence of DAH with venous thromboembolism (VTE) is a seroius problem for clinicians and poses a challenge in the therapeutic management. We describe a young patient who developed massive DAH in the course of anti-glomerular basement membrane (anti-GBM) disease (formerly called Goodpasture's syndrome) complicated by pulmonary embolism (PE).

Solitary pulmonary nodule - the role of imaging in the diagnostic process.

A solitary pulmonary nodule is a round opacity less than 30 mm in diameter surrounded by normally aerated lung tissue. Determination of the character of the lesion following its detection (particularly when the identification was incidental) may require a complex diagnostic process. In most cases, nodules are benign in character; however, the probability of malignancy increases significantly for part-solid lesions. The main features that describe the solitary pulmonary nodule in computed tomography scans include their size, shape, density, presence of calcification and rate of growth. PET-CT examination provides additional information on the metabolic activity of the lesions, and MRI is helpful in assessment of local invasion of surrounding structures. Due to limited availability and highly specialized character, these examinations are not routinely used. Therefore, despite development of other imaging modalities, computed tomography remains the most important and crucial diagnostic tool. Clinical risk factors such as age or smoking status are very important for evaluation of the likelihood of malignancy of a nodular lesion. Due to the multidisciplinary nature of data required for complex assessment of a solitary nodular lesion, management routines are needed in the diagnostic process such as those proposed by the Fleischner Society.

Cryptogenic organizing pneumonia-Results of treatment with clarithromycin versus corticosteroids-Observational study.

BACKGROUND: Cryptogenic organizing pneumonia (COP) is a clinicopathological syndrome of unknown origin. Corticosteroids are the standard treatment, but clarithromycin (CAM) is also effective. The aim of this observational retrospective study was to compare the results of CAM versus prednisone (PRE) treatment in patients with biopsy-proven OP without respiratory insufficiency. MATERIAL AND METHODS: In a 15-year period, 40 patients were treated with CAM (500 mg twice daily orally for 3 months) and 22 with PRE (mean initial dose of 0.67 ± 0.24 mg/kg/d for a mean of 8.59 ± 3.05 months). RESULTS: The clinical presentation, laboratory, and radiological findings did not differ markedly between patients treated with CAM and PRE, with the exception of a higher frequency of sweats (55% vs. 23%; p < 0.015), ground glass opacities (95% vs. 50%; p <0.0001) and nodular lesions (45% vs. 18%; p = 0.036) in the CAM group. A complete response was achieved in 35(88%) patients treated with CAM and in all treated with PRE. Patients treated with PRE relapsed more frequently than those treated with CAM (54.5% vs. 10%; p < 0.0001). Corticosteroid-related adverse events were noticed in 8(6.5%) patients (with one death), but CAM caused only one (2.5%) allergic reaction. A FVC >80% identified patients who might be successfully treated with CAM with a sensitivity of 60% and a specificity of 88.57% (AUC 0.869; 95% CI 0.684-1; p = 0.008); the figures for the FEV1 were >70%, a sensitivity of 60%, and a specificity of 91.43% (AUC 0.809; 95%CI 0.609-1; p = 0.027). CONCLUSIONS: CAM can be used to treat COP patients in whom the pulmonary function parameters are within normal limits. Such therapy is shorter, better tolerated, and associated with fewer adverse events and relapses than is PRE. However, the therapy is ineffective in some patients.

Clinical presentation and characteristics of 25 adult cases of pulmonary sequestration.

BACKGROUND: Pulmonary sequestration (PS) is a rare congenital abnormality of lung tissue. Only few series of adult cases are reported. The aim was to describe clinical characteristics in adult cases of PS and to compare outcomes in different clinical situations. METHODS: Using MSD engine we searched for cases of PS that have been diagnosed between Jan 1st, 2005 and Dec 31st, 2015. Clinical data was retrospectively gathered. Statistica v.12 (StatSoft, Inc.) was used for statistical analyses. RESULTS: We found 25 cases (18 females, 7 males), which underwent surgery and were histologically proven. There were 22 cases of intralobar PS. 7 cases were asymptomatic, 12 had infectious history (including 3 cases of lung abscess and pleural empyema), 4 presented with hemoptysis, 2 with chest pain. The average age to undergo surgery was 38.24, in the asymptomatic group 34, in symptomatic 39.89. In the latter the symptoms preceded the surgery for 2.45-year. Great majority of sequestrations was located in lower lobes (96%), 52% on the left. Symptomatic cases were at higher than expected risk of surgical complications, comparing to asymptomatic (chi2, P=0.04). In most cases there were surgical and histological signs of infection, only in 9 cases etiological factor was determined: in 5 cases it was A. fumigatus. A 0.53-day longer post-surgical hospital stay was observed in the symptomatic group, no statistical significance was found (U-test, P=0.45). CONCLUSIONS: Surgical treatment of symptomatic cases of PS is characterized by slightly longer post-surgical hospital stay and higher risk of surgical complications. Fungal infections are the most likely to occur in PS.

Fire-eater's lung.

Fire eater's lung (FEL) is an acute hydrocarbon pneumonitis caused by aspiration or inhalation into airways of liquid hydrocarbons. This disorder is classified into distinct form of chemical toxic pneumonitis. An amateur fire-eater is presented in this work. He accidentally aspirated into airways about 1/3 of glass of grill lighter fluid composed of mixture of liquid hydrocarbons. A few hours after this incident he had severe symptoms like weakness, high temperature, midsternal pleuritic chest pain, myalgia of the back, shortness of breath, and dry cough. Radiologic examination revealed consolidations with well-defined cavitary lesions (pneumatoceles) in lower lobes mainly in the left lower lobe. After one week of this event clinical improvement was observed. The lesions resolved nearly completly during three months. The review of the literature connected with fire-eater's lung is also presented.

Clarithromycin Decreases IL-6 Concentration in Serum and BAL Fluid in Patients with Cryptogenic Organizing Pneumonia.

BACKGROUND: Inflammatory cytokines are involved in the development of cryptogenic organizing pneumonia (COP). It has been shown that macrolides inhibit cytokine production in the alveolar macrophages of COP patients. OBJECTIVES: The aim of the study was to assess the concentrations of interleukin 1ß (IL-1ß), IL-6, IL-8 and transforming growth factor ß (TGF-ß) in serum and in bronchoalveolar lavage fluid (BAL-f) in COP patients treated with clarithromycin (CAM). MATERIAL AND METHODS: The study involved 26 patients (18 women and 8 men, mean age 56.46 ± 8.83 years) with biopsy-proven COP. After being treated with CAM, a complete recovery was achieved in 22 patients, while four patients did not respond to the treatment. The ELISA method was used to measure the serum and BAL-f concentrations of IL-1ß, IL-6, IL-8 and TGF-ß. RESULTS: Before treatment, the serum IL-1ß1, IL-6, IL-8 and TGF-ß1 concentrations were similar in responders and non-responders. Significant decreases in serum concentrations of IL-6 (8.98 ± 13.26 pg/mL vs. 3.1 ± 6.95 pg/mL; p = 0.005), IL-8 (20.14 ± 25.72 pg/mL vs. 10.14 ± 6.8 pg/mL; p = 0.007) and TGF-ß1 (37.89 ± 12.49 ng/mL vs. 26.49 ± 12.45 ng/mL; p = 0.001) were found after treatment, as well as a significant decrease in the BAL-f concentration of IL-6 (30.56 ± 56.78 pg/mL vs. 4.53 ± 5.84 pg/mL; p = 0.036). Clarithromycin treatment resulted in a significantly lower mean value of serum IL-6 responders than non-responders. CONCLUSIONS: In COP patients, response to clarithromycin treatment was associated with decreases in serum concentrations of IL-6, IL-8 and TGF-ß, and of rations, and of the BAL-f concentration of IL-6.

Intratracheal Dilation-injection Technique in the Treatment of Granulomatosis with Polyangiitis Patients with Subglottic Stenosis.

OBJECTIVE: An analysis of subglottic stenosis (SGS) occurrence frequency in patients with granulomatosis with polyangiitis (GPA) based on the time of appearance of clinical symptoms, and an assessment of treatment effectiveness, in particular with the intratracheal dilation-injection technique (IDIT). METHODS: Review and treatment with IDIT of 34 patients with SGS associated with GPA. RESULTS: SGS developed in 34 of 250 patients with GPA (13.6%) and was not reflective of disease activity in the organs in 15 of 34 patients (44%): 11 cases after and 4 cases during immunosuppressive therapy (IST) when patients did not have organ symptoms. All patients underwent IDIT and in total, the treatment resulted in immediate improvement. In addition, in 21 cases, IST was applied because of other organ involvement or of the lack of longterm efficacy of IDIT. The median time of response was 37 months and the median interval between sessions was 5 months. None of the patients required tracheostomy after beginning IDIT in our hospital. CONCLUSION: SGS often occurs independently of other features of active GPA. IDIT is a safe and effective technique in the treatment of GPA-related SGS. It should be performed in all patients with GPA who develop significant SGS and in those with multiorgan disease concomitantly with IST. In patients with isolated SGS, IDIT also makes IST and tracheostomy unnecessary.

Prevalence of cardiac sarcoidosis in white population: a case-control study: Proposal for a novel risk index based on commonly available tests.

Cardiac sarcoidosis (CS) is a life-threatening and underdiagnosed manifestation of the disease, which requires a complicated and expensive diagnostic pathway. There is a need for simple tool for practitioners to determine the risk of CS without access to specialized equipment.The aim of study was to determine the prevalence of CS in a group of patients diagnosed with or followed up because of sarcoidosis. A secondary objective was the search for factors associated with heart involvement.We performed a prospective case-control study (screening analysis) in consecutive sarcoidosis patients collected from October 2012 to September 2015. Cardiac magnetic resonance (CMR) imaging was performed to confirm or exclude cardiac involvement in all patients. The study was conducted in a hospital-based referral center for patients with sarcoidosis and other interstitial lung diseases.Analysis was performed in a group of 201 patients (all white) with biopsy-proven sarcoidosis, mean age 41.4 ±â€Š10.2, 121 of them (60.2%) males. Four patients with previously recognized cardiac diseases, which make CMR imaging for CS inconclusive, were not included.Cardiac involvement was detected by CMR in 49 patients (24.4%). Factors associated with an increased risk of CS (univariate analyses) included male sex (odds ratio [OR]: 2.5; 1.21-5.16, P = 0.01), cardiac-related symptoms (OR: 3.53; 1.81-6.89, P = 0.0002), extrathoracic sarcoidosis (OR: 3.48; 1.77-6.84, P = 0.0003), elevated serum NT-proBNP (OR: 3.82; 1.55-9.42, P = 0.004), any electrocardiography abnormality (OR: 5.38; 2.48-11.67, P = 0.0001), and contemporary radiological progression sarcoidosis in the lungs (OR: 2.98; 1.52-5.84, P = 0.001). Abnormalities in echocardiography and Holter ECG were also risk factors, but not significant in multivariate analyses. A CS Risk Index was developed using a multivariate model to predict CS, achieving an accuracy of 82%, sensitivity of 50%, specificity of 94%, and likelihood ratio 8.1.CS was detected in one fourth of patients. A CS Risk Index based on the results of easily accessible tests is cost-effective and may help to identify patients who should be urgently referred for further diagnostic procedures.