National data on prevalence of idiopathic pulmonary fibrosis and antifibrotic drug use in Finnish specialised care

Introduction The previous data concerning the prevalence of idiopathic pulmonary fibrosis (IPF) and the frequency of antifibrotic drug use in Finland were based on research registries and medical records whereas nationwide data on the number of patients with IPF in specialised care and those on antifibrotic treatment have not been published. Methods We made an information request to the Finnish National Hospital Discharge Register (Hilmo) covering the whole population of Finland to find out the annual numbers of patients with IPF treated in specialised care in 2016-2021. The numbers of the patients initiating and using pirfenidone and nintedanib were requested from the Social Insurance Institution of Finland (Kela) for the same time period. Results The estimated prevalence of IPF in specialised care was 36.0 per 100 000 in 2021, having increased since 2016. The number of antifibrotic drug users and their proportion of outpatients with IPF had also risen during the follow-up period. In 2021, 35% of the patients with IPF used pirfenidone or nintedanib. The number of inpatients treated in specialised care because of IPF had declined during 2016-2021. Conclusions The prevalence of IPF was higher than expected in Finnish specialised care and had increased during the 6-year follow-up time. The increase in the number of patients with IPF using antifibrotic drugs might have diminished the need for IPF-related hospitalisations.Peer reviewe

Interstitiaaliset keuhkosairaudet : sidekudossairauksiin liittyvät interstitiaaliset keuhkosairaudet

Kun sidekudossairautta sairastavalla potilaalla on ollut yli kuukauden ajan hengenahdistusta, yskää ja ¬limaisuutta tai keuhkoista kuullaan rahinoita, on syytä tutkia keuhkojen toiminta ja ohutleike-TT. Sidekudossairauksien lääkkeet ja infektiot ovat tavallisia keuhkomuutosten aiheuttajia. Keuhkokudossairautta epäiltäessä status tutkitaan huolellisesti ja etsitään keuhkojen ulkopuolisia löydöksiä sekä tutkitaan autoimmuuniserologia. Keuhkolääkärin ja reumatologin kokonaisvaltaista arviota on syytä harkita. Sidekudossairauksiin liittyvien keuhkomuutosten hoidot perustuvat lähinnä tutkimuksiin sklerodermaan liittyvän keuhkosairauden hoidosta ja sidekudossairauksien muissa elinmanifestaatioissa käytettäviin hoitoihin. euhkomuutoksia on tärkeää seurata tiiviisti ja pyrkiä ennakoimaan niiden etenemistä. Lääkitys suunnitellaan potilaskohtaisesti taudinkulun mukaan. Hoidot perustuvat lähinnä tutkimuksiin sklerodermaan liittyvän keuhkosairauden hoidosta sekä sidekudos¬sairauksien hoito-ohjeisiin.Peer reviewe

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.Peer reviewe

The fate of aspergilloma patients after surgical treatment-experience from 22 cases

Background: Patients with pulmonary aspergillomas occasionally undergo surgery but it is somewhat unclear who of these patients benefit from surgical treatment. Methods: We retrospectively evaluated all 22 patients that underwent surgery in Helsinki University Central Hospital between 2004 and 2017. We assessed their clinical backgrounds, anti-fungal medication, indication for surgery, complications, recurrent infections and survival. Results: Of the 22 patients, 14 male and 8 female, mean age 56, an underlying pulmonary disease was present in 20. On immunosuppressive medication were 8 (36%). Most received anti-fungal medication preoperatively (n=12) and/or postoperatively (n=17), 3 patients did not receive anti-fungal medication. Length of the medication periods were diverse. Main indication for surgery was haemoptysis. One in-hospital-death occurred, and other complications included prolonged air-leak, postoperative pneumonia, pneumothorax and pneumomediastinum. No Aspergillus empyema or pleurites occurred. Five-year survival was 54%. One in-hospital-death and one other death were the result of Aspergillus disease, other deaths were unrelated to Aspergillus. Recurrent disease occurred in four cases. Three of these patients were asthma patients with allergic bronchopulmonary aspergillosis (ABPA). Conclusions: Overall results of surgery in this cohort were good and number of complications was low. Therapy with antifungals was diverse. Surgical treatment of aspergilloma can be life-saving for patients suffering of haemoptysis, and patients with restricted disease and well-preserved pulmonary capacity may benefit from surgery. Careful patient selection is crucial.Peer reviewe

Virtual communication is commonly used in Finnish interstitial lung disease multidisciplinary meetings

Multidisciplinary meeting (MDM) is a core element in the diagnosis of interstitial lung diseases (ILD). The aim of the study was to investigate the implementation and key elements related to ILD MDMs in Finnish specialized care, which is characterized by long travel distances and a large number of small centers treating patients suffering from ILDs. An electronic questionnaire was sent to ILD experts working at five academic centers of Finland regarding the implementation of ILD MDMs with the focus on utilization of virtual communication. Responses were received from all academic centers of Finland (n = 5) whose catchment areas cover all of Finland. ILD MDMs were organized in each center approximately every two weeks and the core participants included a radiologist, respiratory physicians, junior staff, pathologist and a rheumatologist. All non-academic centers could refer their patients to be evaluated in ILD MDM of an academic center. Virtual communication was utilized by all academic centers in the implementation of ILD MDMs, being most common among small centers located in Eastern and Northern Finland. Virtual access to ILD MDM of an academic center was available in most parts of Finland, enabling small centers to benefit from the ILD expertise of academic centers.Peer reviewe

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

Peer reviewe

Idiopaattinen keuhkofibroosi - muuttuva diagnostiikka ja hoito

English summaryPeer reviewe

Thoracic gas compression during forced expiration in patients with emphysema, interstitial lung disease and obesity

Abstract Background Dynamic gas compression during forced expiration has an influence on conventional flow-volume spirometry results. The extent of gas compression in different pulmonary disorders remains obscure. Utilizing a flow plethysmograph we determined the difference between thoracic and mouth flows during forced expiration as an indication of thoracic gas compression in subjects with different pulmonary diseases characterized by limitations in pulmonary mechanics. Methods Patients with emphysema (N = 16), interstitial lung disease (ILD) (N = 15), obesity (N = 15) and healthy controls (N = 16) were included. Compressed expiratory flow-volume curves (at mouth) and corresponding compression-free curves (thoracic) were recorded. Peak flow (PEF) and maximal flows at 75%, 50% and 25% of remaining forced vital capacity (MEF75, MEF50 and MEF25) were derived from both recordings. Their respective difference was assessed as an indicator of gas compression. Results In all groups, significant differences between thoracic and mouth flows were found at MEF50 (p < 0.01). In controls, a significant difference was also measured at MEF75 (p <0.005), in emphysema subjects, at PEF and MEF75 (p < 0.05, p < 0.005) and in obese subjects at MEF75 (p <0.005) and MEF25 (p < 0.01). ILD patients showed the lowest difference between thoracic and mouth flows at MEF75 relative to controls and emphysema patients (p < 0.005, p < 0.001). Obese subjects did not differ from controls, however, the difference between thoracic and mouth flows was significantly higher than in patients with emphysema at MEF50 (p < 0.001) and MEF25 (p < 0.005). Conclusions Alveolar gas compression distorts the forced expiratory flow volume curve in all studied groups at the middle fraction of forced expiratory flow. Consequently, mouth flows are underestimated and the reduction of flow measured at 75% and 50% of vital capacity is often considerable. However, gas compression profiles in stiff lungs, in patients with decreased elastic recoil in emphysema and in obesity differ; the difference between thoracic and mouth flows in forced expiration was minimal in ILD at the first part of forced expiration and was higher in obesity than in emphysema at the middle and last parts of forced expiration