Overall drug treatment of idiopathic pulmonary fibrosis patients from national registries - a real-world study from Finland.

BACKGROUND: Currently, two disease-modifying antifibrotic drugs are indicated for the treatment of idiopathic pulmonary fibrosis. The objective of this study was to analyse antifibrotic and overall prescription medication use of IPF patients in the real world. METHODS: Data was collected from the FinnishIPF registry and the Registry of the Social Insurance Institution of Finland (SII). Purchases of all prescription medicines were assessed. The frequency, the initiation interval, the duration, and the breaks of the antifibrotic treatments were defined. The association between the prescription of antifibrotic therapy and different patient-related clinical parameters was studied. Accordingly, the relationships between the delay in starting therapy and patient-related variables were analysed. RESULTS: Of the 263 IPF patients, 132 (50.2%) had started antifibrotic treatment during the study period 2011-2018. The mean interval from the diagnosis to the first purchase was 367 (SD 429) days. The antifibrotic drug was switched in 14% of patients. Discontinuation of therapy occurred most commonly during the first year of the treatment. The one-year persistence was 77.1% for pirfenidone and 78.9% for nintedanib. A tendency of treating patients under 75 years was noticed. Low forced vital capacity predicted earlier initiation of medication. CONCLUSIONS: The initiation of antifibrotics after diagnosis was slow, probably due to reimbursement limitations. Younger age at diagnosis affected treatment initiation although it is unknown which patients benefit most from these medications. The reasons for discontinuation of the antifibrotic therapy during the first year should be a focus in clinical work and further studies.

Demographics and survival of patients with idiopathic pulmonary fibrosis in the FinnishIPF registry.

Idiopathic pulmonary fibrosis (IPF) is characterised by unpredictable disease course and poor survival. After the introduction of novel antifibrotic drugs, the prognosis of patients with IPF is probably changing. FinnishIPF, a nationwide registry of carefully characterised patients, was initiated in Finland in 2011. For the data analysis, we included 453 incident IPF patients diagnosed during 2011-2015. In this study, we describe the demographics and prognosis of these real-life patients. The median overall survival time of registered IPF patients was 4.5 years. The transplant-free survival at 1, 2, 3, 4 and 5 years was 95%, 83%, 70%, 58% and 45%, respectively. Smoking did not have any effect on survival. 117 (26%) patients received pirfenidone or nintedanib. Patients who received ≥6 months of treatment had better survival compared with those who did not receive treatment but this difference disappeared after age adjustment. The transplantation rate was 3%. Although IPF is diagnosed in Finland at a older age, the prognosis is better than expected due to a relatively well preserved lung function at diagnosis. Age and pulmonary function were identified as independent predictors of survival in the entire IPF patient population as well as in patients who had received antifibrotic treatment.

Are physicians in primary health care able to recognize pulmonary fibrosis?

Background: The early diagnosis of idiopathic pulmonary fibrosis (IPF) has become increasingly important due to evolving treatment options. IPF patients experience a significant delay in receiving an accurate diagnosis, thus delayed access to tertiary care is associated with higher mortality independently from disease severity. Objective: The aims were to evaluate whether there had been a delay in the referral process, and to determine whether the referring doctors had suspected IPF or other interstitial lung disease (ILD) already during the time of referral. Methods: Ninety-five referral letters of patients with IPF identified from the FinnishIPF registry were evaluated with respect to time of referral, referring unit, grounds for referral, symptoms, smoking status, occupational history, clinical examinations, co-morbidities, medication, radiological findings and lung function. Results: Fifty-nine percent of referral letters originated from primary public health care. The time from symptom onset to referral was reported in 60% of cases, mean time being 1.5 (0.8-2.3) (95%CI) years. The main reason for referral was a suspicion of interstitial lung disease (ILD) (63%); changes in chest X-ray were one reason for referring in 53% of cases. Lung auscultation was reported in 70% and inspiratory crackles in 52% of referral letters. Conclusions: Primary care doctors suspected lung fibrosis early in the course of disease. Lung auscultation and chest X-rays were the most common investigational abnormalities in the referrals. Providing general practitioners with more information of ILDs might shorten the delay from symptom onset to referral.

[The prognosis of lung cancer continues to be poor--treatment outcome within The Hospital District of Southwest Finland in 2004 to 2011]. / Hoitotulokset Varsinais-Suomen sairaanhoitopiirissä 2004-2011. Keuhkosyövän ennuste pysyy edelleen huonona.

BACKGROUND: The survival of lung cancer patients is still poor; 10-15% are alive five years after diagnosis. MATERIAL AND METHODS: We evaluated 1 542 patients with new lung cancer diagnosis in 2004-2011. RESULTS: The one-, two- and five-year survival rate was 37.3%, 23.3% and 10.5%, respectively. The prognosis of women was better. There were no changes over the 8 years in the number or age of patients, the share of women, the histology, the number of operated patients or survival. CONCLUSIONS: Compared with earlier Finnish studies the short-term prognosis seems to be little better.

[Commencement of and adherence to CPAP treatment of patients with sleep-disordered breathing]. / Uniapneapotilas 2000-luvulla: kenelle CPAP-hoito aloitetaan ja miten siihen sitoudutaan?

BACKGROUND: We evaluated the changes in nasal CPAP treatment and adherence in patients with sleep-disordered breathing (SDB). MATERIAL AND METHODS: Information from the clinic database of 4,385 patients commencing CPAP treatment during 2002-2009. RESULTS: Average CPAP adherence was 74%. In mild SDB over 60% were adherent whereas in severe SDB 18% did not accept the treatment. Low hours of CPAP use right after commencing the treatment indicated poor long-term adherence. CONCLUSIONS: In the 2000s the number of patients starting CPAP treatment increased, but the selection of patients did not change much. Overall CPAP adherence was good.

Idiopathic pulmonary fibrosis--a systematic review on methodology for the collection of epidemiological data.

BACKGROUND: Recent studies suggest that the incidence of idiopathic pulmonary fibrosis (IPF) is rising. Accurate epidemiological data on IPF, however, are sparse and the results of previous studies are contradictory. This study was undertaken to gain insight into the various methods used in the epidemiological research of IPF, and to get accurate and comparable data on these different methodologies. METHODS: A systematic database search was performed in order to identify all epidemiological studies on IPF after the previous guidelines for diagnosis and treatment were published in 2000. Medline (via Pubmed), Science Sitation Index (via Web of Science) and Embase databases were searched for original epidemiological articles published in English in international peer-reviewed journals starting from 2001. After pre-screening and a full-text review, 13 articles were accepted for data abstraction. RESULTS: Three different methodologies of epidemiological studies were most commonly used, namely: (1) national registry databases, (2) questionnaire-based studies, and (3) analysis of the health care system's own registry databases. The overall prevalence and incidence of IPF varied in these studies between 0.5-27.9/100,000 and 0.22-8.8/100,000, respectively. According to four studies the mortality and incidence of IPF are rising. CONCLUSIONS: We conclude that there are numerous ways to execute epidemiological research in the field of IPF. This review offers the possibility to compare the different methodologies that have been used, and this information could form a basis for future studies investigating the prevalence and incidence of IPF.

Pemetrexed in malignant pleural mesothelioma and the clinical outcome.

INTRODUCTION: Pemetrexed has emerged as standard chemotherapy for malignant pleural mesothelioma (MPM). OBJECTIVES: MPMs at two Finnish University Hospitals during 7 years (2000-2006) were reviewed in order to evaluate the treatments, survival and prognostic factors. The results in two periods (before pemetrexed use in 2000-2002 and with pemetrexed in 2003-2006) were compared. METHODS: Data were collected from the individual patient records retrospectively, and analysed. RESULTS: Altogether 197 patients were diagnosed with following histologies: 136 (69%) epithelioid, 19 (10%) sarcomatoid, 17 (9%) mixed, 25 (13%) not specified; 141 (72%) patients received treatment (five extrapleural pneumonectomy, 36 pleurectomy/decortication, 126 chemotherapy). Median survival was 12.9 months and the 1-, 2- and 3-year survivals were 51.8%, 21.8% and 12.1%, respectively. Univariate analysis showed no significant difference in survival between the patients diagnosed before or during the pemetrexed era (P = 0.124). The patients receiving pemetrexed or other chemotherapy had median survivals of 16.7 and 15.3 months, respectively. The independent prognostic factors for survival were histology and asbestos exposure. Non-epithelioid histology yielded 17 times higher risk of dying than epithelioid. Asbestos exposure doubled the risk of dying, but only in patients diagnosed in 2003-2006. CONCLUSIONS: Pemetrexed is beneficial for selected patients, but it has not changed the outcome of the whole MPM population as much as perhaps anticipated. Patient groups with various treatments or symptomatic care only reached survival results comparable to those reported in chemotherapy trials, thus emphasising the need for better subtyping of mesothelioma and individualising the treatment.

[Bevacizumab in the treatment of non-small cell lung cancer]. / Bevasitsumabi ei-pienisoluisen keuhkosyövän hoidossa.

Treatment of advanced or metastatic non-small cell lung cancer with current cytotoxic agents is at its best able to only slow down the progression of the disease, while no curative treatment is known. Novel antiangiogenetic agents such as Bevacizumab have been expected to bring about a change in the treatment and prognosis of this disease. Two randomized studies have been conducted with Bevacizumab, whereby it was observed to make the therapeutic results more effective when combined with a cytotoxic platinum agent in a selected patient group. In our opinion the current scientific evidence does not yet support the use of Bevacizumab as the standard therapy for lung cancer.

Delays in the diagnosis and treatment of lung cancer.

STUDY OBJECTIVES: This study was undertaken to measure delays of diagnosis and to assess the causes for those delays in patients with lung cancer. In addition, the relation of delay times and survival was analyzed. DESIGN: A retrospective study based on patient records. Dates for symptoms, visits to doctors, investigations, treatment, and death were recorded. SETTING: Patients who were found to have lung cancer at Turku University Hospital, Finland, during 2001. PATIENTS: Records of 132 patients were reexamined. RESULTS: The median delay in patient presentation from first symptoms to first appointment with a general practitioner (GP) was 14 days. The median delay by the GP before writing a referral was 16 days, the median referral delay was 8 days, the median delay from the first visit to a specialist until the diagnosis was 15 days, and the median treatment delay was also 15 days. Thirty percent of patients received treatment within 1 month from the first hospital visit, and 61% received treatment within 2 months. The median symptom-to-treatment delay was almost 4 months. The delay in seeing a specialist was shorter in patients with advanced cancer and small cell lung cancer. About half of our patients fulfilled the criteria of the British Thoracic Society recommendations. A longer specialist treatment delay seemed to correlate with better survival in advanced disease, but it was not an independent significant factor for survival. CONCLUSIONS: Several reasons for long delays were found, but on many occasions patients underwent numerous consecutive procedures before a diagnosis of cancer was confirmed. Shortening the diagnostic and treatment delay times might be possible with little extra cost by a multidisciplinary team approach and by rapid access to carefully planned investigations, but decreasing the patient delay might be more difficult. This study shows that long specialist treatment delays are not correlated with worse prognosis in patients with advanced disease. In patients with more limited disease, the delay time may be more critical, and if curative treatment is the goal, the diagnostic process should proceed without needless delay to avoid a situation in which curable disease becomes incurable.

Gastroesophageal reflux and bronchial responsiveness: correlation and the effect of fundoplication.

BACKGROUND: A causal relationship between gastroesophageal reflux (GER) and asthma has been suggested. Should this be the case, one could expect treatment of GER to diminish bronchial sensitivity. There has been a lack of trials evaluating the efficacy of antireflux surgery on airway reactivity. OBJECTIVES: To investigate the correlation between GER and bronchial responsiveness, and to determine the efficacy of Nissen fundoplication on bronchial responsiveness and pulmonary function. METHODS: A methacholine inhalation challenge was performed on 15 consecutive GER patients preoperatively and approximately 5 months after Nissen fundoplication. Airway responsiveness was quantified with a dose-response slope (DRS), calculated by dividing the decrease in FEV(1) (%) with the dose of methacholine administered (micromoles). RESULTS: A positive correlation between the severity of distal esophageal reflux and bronchial responsiveness was found (r = 0.83, p < 0.001). There was an improvement in FEV(1) after fundoplication (p = 0.03). All 3 asthmatic patients participating in the study presented with bronchial hyperresponsiveness (BHR) which improved clearly in all of these patients after fundoplication. This resulted in an apparent trend for DRS to improve when the entire study population was considered (p = 0.12). CONCLUSIONS: According to the current study there seems to be a positive correlation between the severity of distal esophageal reflux and bronchial responsiveness. These data suggest that operative treatment of GER may ameliorate BHR in asthmatic patients. Moreover, the results of the present study suggest that fundoplication may improve pulmonary function in patients with GER.