RESUMEN

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.

Asunto(s)

COVID-19 , Miocarditis , Humanos , COVID-19/complicaciones , Miocarditis/epidemiología , Miocarditis/etiología , Incidencia , SARS-CoV-2 , Factores de Riesgo

RESUMEN

The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.

Asunto(s)

Fibrosis Pulmonar Idiopática , Enfermedades Pulmonares Intersticiales , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/terapia , Fibrosis Pulmonar Idiopática/complicaciones , Polonia , Progresión de la Enfermedad , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/terapia , Enfermedades Pulmonares Intersticiales/complicaciones , Fibrosis

RESUMEN

The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.

Asunto(s)

Fibrosis Pulmonar/prevención & control , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Antifibróticos/uso terapéutico

RESUMEN

Background: Pirfenidone and nintedanib are considered as the standard of care in idiopathic pulmonary fibrosis (IPF), but there is no consensus as to which of these two agents should be regarded as first-line treatment. Objective: To provide real-world data on therapeutic decisions of pulmonary specialists, particularly the choice of the antifibrotic drug in patients with IPF. Methods: This was a multicenter, prospective survey collecting clinical data of patients with IPF considered as candidates for antifibrotic treatment between September 2019 and December 2020. Clinical characteristics and information on the therapeutic approach were retrieved. Statistical evaluation included multiple logistic regression analysis with stepwise model selection. Results: Data on 188 patients [74.5% male, median age 73 (interquartile range, 68-78) years] considered for antifibrotic therapy were collected. Treatment was initiated in 138 patients, while 50 patients did not receive an antifibrotic, mainly due to the lack of consent for treatment and IPF severity. Seventy-two patients received pirfenidone and 66 received nintedanib. Dosing protocol (p < 0.01) and patient preference (p = 0.049) were more frequently associated with the choice of nintedanib, while comorbidity profile (p = 0.0003) and concomitant medication use (p = 0.03) were more frequently associated with the choice of pirfenidone. Age (p = 0.002), lung transfer factor for carbon monoxide (TLCO) (p = 0.001), and gastrointestinal bleeding (p = 0.03) were significantly associated with the qualification for the antifibrotic treatment. Conclusion: This real-world prospective study showed that dose protocol and patient preference were more frequently associated with the choice of nintedanib, while the comorbidity profile and concomitant medication use were more frequently associated with the choice of pirfenidone. Age, TLCO, and history of gastrointestinal bleeding were significant factors influencing the decision to initiate antifibrotic therapy.

RESUMEN

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.

Asunto(s)

Antiinflamatorios no Esteroideos/uso terapéutico , Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Cumplimiento de la Medicación/estadística & datos numéricos , Piridonas/uso terapéutico , Anciano , Progresión de la Enfermedad , Femenino , Humanos , Fibrosis Pulmonar Idiopática/cirugía , Pulmón/fisiopatología , Trasplante de Pulmón/estadística & datos numéricos , Masculino , Persona de Mediana Edad , Polonia , Pruebas de Función Respiratoria , Estudios Retrospectivos , Resultado del Tratamiento , Prueba de Paso

RESUMEN

INTRODUCTION: This document presents the guidelines of the Polish Respiratory Society (PTChP, Polskie Towarzystwo Chorób Pluc) for diagnosis and treatment of idiopathic pulmonary fibrosis (IPF), developed by agroup of Polish experts. MATERIAL AND METHODS: The recommendations were developed in the form of answers to previously formulated questions concer-ning everyday diagnostic and therapeutic challenges. They were developed based on acurrent literature review using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. RESULTS: We formulated 28 recommendations for diagnosis (8), pharmacological treatment (12) as well as non-pharma-cological and palliative therapy (8). The experts suggest that surgical lung biopsy (SLB) not be performed in patients with the probable usual interstitial pneumonia (UIP) pattern, with an appropriate clinical context and unanimous opinion of a multidisciplinary team. The experts recommend using antifibrotic agents in IPF patients and suggest their use irrespective of the degree of functional impairment. As regards non-pharmacological and palliative treatment, strong re-commendations were formulated regarding pulmonary rehabilitation, oxygen therapy (in patients with chronic respiratory failure), preventive vaccinations as well as referring IPF patients to transplant centres. Table 1 presents an aggregate list of recommendations. CONCLUSIONS: The Polish Respiratory Society Working Group developed guidelines for IPF diagnosis and treatment.

Asunto(s)

Competencia Clínica/normas , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/terapia , Pautas de la Práctica en Medicina/organización & administración , Sociedades Médicas/normas , Centros Médicos Académicos , Humanos , Guías de Práctica Clínica como Asunto/normas

RESUMEN

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.

Asunto(s)

Cardiomiopatías/fisiopatología , Sarcoidosis/fisiopatología , Volumen Sistólico , Adulto , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Pruebas de Función Respiratoria , Estudios Retrospectivos , Índice de Severidad de la Enfermedad

RESUMEN

Up to one fourth of sarcoidosis patients may have cardiac involvement, what is potentially a life-threatening condition and requires aggressive treatment. Corticosteroids are generally effective in cardiac sarcoidosis, however may have significant short and long term adverse effects. We present a case of a 42-year-old male, who was diagnosed with pulmonary and cardiac sarcoidosis. He was treated initially with corticosteroids and satisfactory improvement was achieved in the lungs but not in the heart. Methotrexate was added as a second line therapy, being beneficial for the heart as well as steroid sparing agent. Cardiac improvement was documented during serial CMR imaging. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 178-181).

RESUMEN

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.

Asunto(s)

Factores Biológicos/uso terapéutico , Glucocorticoides/uso terapéutico , Inmunoterapia/métodos , Sarcoidosis/terapia , Adulto , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Alta del Paciente/tendencias , Polonia/epidemiología , Prevalencia , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Sarcoidosis/diagnóstico , Sarcoidosis/epidemiología

RESUMEN

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and debilitating lung disease with a median survival time of 3-5 years. For now, pirfenidone (PIR) and nintedanib (NTB) are the only drugs that can slow down the disease's progression. In Poland, these drugs, although registered for legal use, had not been reimbursed for IPF patients until the end of the year 2016. Aim of the study was to assess what was common practice in terms of diagnosis and treatment in the period before antifibrotic drugs became available for IPF patients in Poland. MATERIAL AND METHODS: We performed a survey among participants of two nationwide pulmonological congresses held in 2016. RESULTS: One hundred and fifty physicians took part in the study. Only 55% of respondents would reach their final diagnosis in collaboration with a radiologist. Just 40% of those sending patients for surgical lung biopsy (SLB) would discuss the case directly with a pathologist. 22% would never refer the patient suspected of having IPF for SLB. 85% believed that bronchoalveolar lavage (BAL) may be useful for diagnosis. 41% of respiratory professionals would not use any drug for the treatment of IPF patients. 23% of physicians would prescribe corticosteroids in high doses (CS), either in monotherapy or in combination with other drugs. Only 43% of respondents would use antacid drugs in case of symptomatic gastro-oesophageal reflux disease (GERD), and only 11% would prescribe these drugs regardless of GERD diagnosis. CONCLUSIONS: The majority of Polish pulmonologists were not supported by radiologists and pathologists in the diagnostic process. Treatment standards were unsatisfactory, mostly due to a lacking of reimbursement regulations. Further education is necessary to improve management of IPF patients in Poland.

Asunto(s)

Fibrosis Pulmonar Idiopática/diagnóstico , Relaciones Interprofesionales , Pautas de la Práctica en Medicina/estadística & datos numéricos , Neumología/métodos , Adulto , Biopsia/estadística & datos numéricos , Lavado Broncoalveolar/estadística & datos numéricos , Femenino , Reflujo Gastroesofágico/diagnóstico , Humanos , Fibrosis Pulmonar Idiopática/patología , Masculino , Persona de Mediana Edad , Polonia , Derivación y Consulta/estadística & datos numéricos

RESUMEN

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.

Asunto(s)

Cardiomiopatías/epidemiología , Sarcoidosis/epidemiología , Adulto , Cardiomiopatías/diagnóstico , Cardiomiopatías/diagnóstico por imagen , Cardiomiopatías/etiología , Estudios de Casos y Controles , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Prevalencia , Factores de Riesgo , Sarcoidosis/diagnóstico , Sarcoidosis/diagnóstico por imagen , Sarcoidosis/etiología , Población Blanca/estadística & datos numéricos

RESUMEN

We report a case of histologically proven pulmonary sarcoidosis and cardiac involvement in a 53-year old woman with progression leading to the heart failure documented in cardiovascular magnetic resonsnce studies.

Asunto(s)

Cardiomiopatías/etiología , Insuficiencia Cardíaca/etiología , Sarcoidosis Pulmonar/complicaciones , Cardiomiopatías/diagnóstico por imagen , Cardiomiopatías/tratamiento farmacológico , Cardiomiopatías/fisiopatología , Progresión de la Enfermedad , Femenino , Glucocorticoides/administración & dosificación , Insuficiencia Cardíaca/diagnóstico por imagen , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/fisiopatología , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Prednisona/administración & dosificación , Sarcoidosis Pulmonar/diagnóstico , Sarcoidosis Pulmonar/tratamiento farmacológico , Volumen Sistólico , Resultado del Tratamiento , Función Ventricular Izquierda

RESUMEN

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease which predominantly affects the lungs, although granulomas can also involve all other organs, including the heart. Cardiac sarcoidosis (CS) may occur at any stage of the disease and may be the cause of sudden cardiac death, even in a previously asymptomatic patient. The aim of this study was to evaluate the incidence of CS in a large group of patients diagnosed or followed up due to sarcoidosis. METHODS: We performed a retrospective analysis of patients at our institution discharged with the final diagnosis "sarcoidosis" (ICD-10: D86) from January 2008 to October 2012. Only those with biopsy (from respiratory tract or lymph nodes) confirmed diagnosis of sarcoidosis were included. We then selected the subset of patients with cardiac involvement due to sarcoidosis confirmed by positive magnetic resonance imaging. RESULTS: The study covered 1375 consecutive sarcoidosis patients (51 % men), who were hospitalized during 5 years. Multiorgan disease was detected in 160 cases (11.7 %), and cardiac involvement was found in 64 patients (4.7 % of all), 70.3 % of whom were men. Twelve of those with CS were in stage I, 48 in stage II, and four in stage III. The odds ratio for having cardiac involvement in men compared to women was 2.3 (95 % CI 1.36-4.0, p = 0.002). CONCLUSIONS: Cardiac involvement in sarcoidosis was diagnosed in the similar percentage as in previously published data but was significantly more frequently in men.

Asunto(s)

Cardiomiopatías/epidemiología , Sarcoidosis/epidemiología , Factores Sexuales , Adulto , Anciano , Cardiomiopatías/diagnóstico , Femenino , Humanos , Incidencia , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Oportunidad Relativa , Estudios Retrospectivos , Sarcoidosis/diagnóstico

RESUMEN

BACKGROUND: Sarcoidosis is a systemic granulomatous multiorgan disease with the most common manifestation is in the chest, although the granulomas can also involve all other organs causing variety of symptoms mimicking different diseases. OBJECTIVES: To evaluate the incidence of comorbidity in a large group of patients with sarcoidosis diagnosed or followed in referral center for lung diseases in Poland. PATIENTS AND METHODS: We performed a retrospective analysis in a group of 1779 patients discharged with the final diagnosis "sarcoidosis" (ICD-10: D86) from January 2008 to October 2011. RESULTS: The majority (79.2%) were diagnosed as pulmonary and/or lymph node sarcoidosis (D86.0, D86.1, D86.2). Sarcoidosis of other and combined sites (D86.8) were diagnosed in 15.8% and unspecified (D86.9) in 5.0% of patients. At least one comorbid condition was noted in 54% of the patients, most frequently arterial hypertension (22.4%), thyroid disorders (5.6%), diabetes mellitus (5.0%), COPD (4.3%) and obesity (3.3%). Using linear regression models, the associations between the number of comorbidities and age and extent of the disease were found (p<0.001). Patients with multiorgan sarcoidosis were more likely to have a comorbid condition. CONCLUSIONS: More than half of patients with sarcoidosis have a comorbid condition, which is more likely in older patients and those with multiorgan involvement.

Asunto(s)

Comorbilidad , Sarcoidosis/clasificación , Sarcoidosis/epidemiología , Factores de Edad , Estudios de Cohortes , Diabetes Mellitus/diagnóstico , Diabetes Mellitus/epidemiología , Femenino , Humanos , Hipertensión/diagnóstico , Hipertensión/epidemiología , Incidencia , Clasificación Internacional de Enfermedades , Modelos Lineales , Masculino , Obesidad/diagnóstico , Obesidad/epidemiología , Polonia , Pronóstico , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Enfermedad Pulmonar Obstructiva Crónica/epidemiología , Estudios Retrospectivos , Medición de Riesgo , Sarcoidosis/fisiopatología , Índice de Severidad de la Enfermedad , Factores Sexuales , Análisis de Supervivencia , Enfermedades de la Tiroides/diagnóstico , Enfermedades de la Tiroides/epidemiología , Resultado del Tratamiento

Asunto(s)

Cardiomiopatías/tratamiento farmacológico , Metotrexato/administración & dosificación , Prednisolona/administración & dosificación , Sarcoidosis/tratamiento farmacológico , Femenino , Humanos , Masculino

RESUMEN

Liver diseases are often associated with respiratory complications. They may manifest as pleural effusion, interstitial lung disease, as well as various combinations of symptoms affecting the pulmonary vasculature. The duration and severity of liver disease is not always reflected by the degree of lung function impairment. On the other hand, progressive damage to the lungs significantly worsens prognosis in the course of severe liver disease. The diagnosis is difficult and often requires multidisciplinary cooperation. The effects of pharmacological treatment are not always satisfactory. In numerous cases, liver transplantation proves to be the best option.

Asunto(s)

Síndrome Hepatopulmonar/diagnóstico , Comorbilidad , Diagnóstico Diferencial , Hepatitis Autoinmune/diagnóstico , Síndrome Hepatopulmonar/epidemiología , Síndrome Hepatopulmonar/terapia , Humanos , Cirrosis Hepática Biliar/diagnóstico , Trasplante de Hígado , Pronóstico , Fumar/epidemiología , Deficiencia de alfa 1-Antitripsina/diagnóstico , Deficiencia de alfa 1-Antitripsina/epidemiología

RESUMEN

Airway reversibility test (or bronchodilatation test) is performed routinely in the diagnosis of obstructive lung diseases. The results of this test may be helpful in the diagnosis and differential diagnosis of asthma and chronic obstructive pulmonary disease (COPD), but sometimes bring confusion. Unfortunately, the nomenclature used for test result is not uniform, which also leads to confusion. Next the reader will find an attempt to order the state of affairs and the introduction of a uniform nomenclature, which would be consistent with the logic and allow for unambiguous classification of reversibility test results to facilitate future decisions based on the differential diagnosis.

Asunto(s)

Asma/diagnóstico , Broncodilatadores , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Pruebas de Función Respiratoria , Terminología como Asunto , Obstrucción de las Vías Aéreas/diagnóstico , Diagnóstico Diferencial , Humanos