Introduction:Investigations of muscle dysfunction in patients with idiopathic pulmonary fibrosis (IPF) are limited to peripheral muscles. However, decreased thoracic muscle mass is known and deterioration of chest wall muscle strength is not clear.Objective:The aims of the present study were to evaluate pectoralis muscle strength located on the chest wall and to investigate the relationship of spirometric measurements and respiratory muscle strength with pectoralis muscle strength.Methods:Elderly patient with IPF (mean disease duration 7.47±7.04 years) and the age-and sex-matched healthy volunteers were recruited in this cross-sectional study. The pulmonary function test was performed by a portable spirometer for spirometric variables and a gas analyzer for diffusing capacity for carbon monoxide (DLCO). Maximal inspiratory (MIP) and expiratory pressure (MEP) were measured with mouth pressure device. Modified Medical Research Council Dyspnea Scale (MMRC) was used to determined dyspnea severity. The pectoralis muscle strength was assessed isometrically during shoulder joint horizontal adduction movement with a handheld dynamometer.Results:A total of 17 patients with IPF (9 males, mean age 69.06±3.94 years) and 19 healthy controls (10 males, mean age 70.95 ±4.99 years) were included. Patients with IPF had lower pectoralis muscle strength than healthy controls (p<0.001). Significant relationships were found between pectoralis muscle strength and MIP (r=0.79, p<0.001), MEP (r=0.81, p<0.001), FEV1% (r=0.54, p=0.02), FVC% (r=0.68, p<0.003) and DLCO (r=0.61, p=0.009). With multiple linear regression analysis, pectoralis muscle strength was the only independent predictor of FVC% (adjusted R2=0.37, p<0.05).Conclusion:In patients with IPF, pectoralis muscle strength decreases and is associated with pulmonary function. In particular pectoralis muscle strength is likely to have an important impact on FVC%. Therefore, we consider that this test should be included routinely in chest diseases and rehabilitation clinics. The trial was registered U.S. National Library of Medicine clinical trial registry (https://clinicaltrials.gov, Trial ID: NCT04803617)

Background:Sarcoidosis is a multifactorial immune disorder with an uncertain origin. A single nucleotide polymorphism (G→A, rs2076530) in the butyrophilin-like 2 (BTNL2) gene results in the formation of truncating protein. This study aimed to genotype the predisposition of the BTNL2 rs2076530 polymorphism in Iranian patients with sarcoidosis using the RFLP technique.Materials and Methods:In this study, 80 patients with sarcoidosis and 80 healthy individuals were included. The rs2076530 polymorphism of the BTNL2 gene was genotyped using the PCR-RFLP method by AvrII restriction enzyme and confirmed by DNA sequencing (Capillary electrophoresis 3130, ABI).Results:There was a statistically significant difference between proportions of patients with AA (47,5%) and controls (27.5%) (OR=2.38, 95%CI:1.23-4.61, P=0.009). In addition, a significant difference was observed in the frequency of the A allele (62.5%) in sarcoidosis (OR=2.14, 95%CI:1.37-3.35, P=0.001). A Bonferroni correction with P<0.0038 indicates a statistical difference for genotype AA (P=0.009). In an effective model, binary logistic regression analysis indicates a statistical association between AA genotype and sarcoidosis (P=0.018 with 60% prediction). Based on the gene analysis study using DNA sequencing, all of the mentioned mutations were seen via RFLP.Conclusion:According to our findings, the BTNL2 rs2076530 A allele in the Iranian population is associated with susceptibility to sarcoidosis. This designed PCR-RFLP method for detecting SNPs is effective as DNA sequencing.

Background:Secondary spontaneous pneumothorax (SSP) in interstitial lung disease (ILD) may influence prognosis of any ILD, and SSP onset predicts poor outcome in idiopathic pulmonary fibrosis (IPF). Recently, progressive fibrosing ILD (PF-ILD) has rapidly acquired importance.Objective:We hypothesized that PF-ILD would strongly influence the prognosis of patients with any ILD complicated with SSP.Methods:We retrospectively surveyed and collected data from patients hospitalized for SSP from January 2016 to June 2020. PF-ILD was defined as the following occurring within 24 months before SSP develops: relative decline in %forced vital capacity (FVC) ≥10% or two of the following: relative decline in %FVC between 5% and 10%, worsening respiratory symptoms, or increased extent of fibrosis on high-resolution computed tomography.Results:We analyzed 32 patients hospitalized for SSP in ILD. This study comprised 18 patients with PF-ILD and 14 patients with non-PF-ILD. PF-ILD patients had lower body mass index (BMI) and %FVC. No significant differences in survival regarding follow-up period from the time of ILD diagnosis and hospitalization for SSP were observed between the PF-ILD and non-PF-ILD patients. Older age and lower BMI were significant predictors of mortality by multivariate Cox regression analysis. ROC analysis showed BMI ≤17.8 kg/m2 to reliably predict poor prognosis.Conclusions:Regardless of whether patients have PF-ILD, older age and lower BMI in patients with ILD places them at higher risk of developing SSP, and prognosis is poor if SSP develops. Therefore, clinical management of physique is important to improve the prognosis of ILD patients.

Although interstitial pneumonia is an important respiratory manifestation in microscopic polyangiitis (MPA), no studies have examined the detailed pathogenesis of interstitial pneumonia during the clinical course of MPA. In addition, it is considered that MPA develops at a certain incidence rate from myeloperoxidase (MPO)- antineutrophil cytoplasmic antibody (ANCA) positive interstitial pneumonia. However, there is a lack of consensus among pulmonologist and rheumatologist regarding whether MPO-ANCA positive interstitial pneumonia, which does not accompany other organ damage related to ANCA-associated vasculitis (AAV) other than interstitial pneumonia, should be included in AAV. In this review article, the clinical questions regarding MPO-ANCA positive interstitial pneumonia have been set, and evidence to date and problems to be solved in future are outlined.

Background:Erythema nodosum-like sarcoid lesion is a specific form of cutaneous sarcoidosis sometimes observed in Japanese patients, with a female predominance, involving the lower extremities. By contrast, erythema nodosum as a non-specific cutaneous manifestation associated with sarcoidosis is rare in Japan. Erythema nodosum-like sarcoid lesion resembles erythema nodosum only in appearance, but histologically shows sarcoid granulomas.Objectives:To determine the clinical characteristics of erythema nodosum-like sarcoid lesion.Methods:We examined the clinical characteristics of erythema nodosum-like sarcoid lesion in our patients with sarcoidosis. Clinical charts were retrospectively examined from 2000 to 2019. The patients’ data, such as age, gender, types of cutaneous lesion, serum levels of angiotensin-converting enzyme, and extracutaneous organ involvement, were evaluated. We also reviewed cases of erythema nodosum-like sarcoid lesion reported in previous literature.Results:Among 101 cutaneous sarcoidosis patients, eight were diagnosed as having erythema nodosum-like sarcoid lesion in our department. The patients were one male and seven females, and their ages ranged from 30 to 74 years old. All cases involved the lower extremities. The serum angiotensin-converting enzyme level was elevated in five cases. Lung sarcoidosis was observed in all cases, and ocular sarcoidosis was observed in six cases, whereas no patients had cardiac sarcoidosis.Conclusions:Although erythema nodosum-like sarcoid lesion may not be major skin manifestation of sarcoidosis, erythema nodosum-like sarcoid lesion frequently accompanies lung and ocular sarcoidosis. Thus, we should pay much attention to erythema nodosum-like sarcoid lesion for early identification of patients needing workup for systemic illness.

Introduction:The aim of our study is to investigate the etiological distribution of ILD in Turkey by stratifying the epidemiological characteristics of ILD cases, and the direct cost of initial diagnosis of the diagnosed patients.Material-Method:The study was conducted as a multicenter, prospective, cross-sectional, clinical observation study. Patients over the age of 18 and who accepted to participate to the study were included and evaluated as considered to be ILD. The findings of diagnosis, examination and treatment carried out by the centers in accordance with routine diagnostic procedures were recorded observationally.Results:In total,1070 patients were included in this study. 567 (53%) of the patients were male and 503 (47%) were female. The most frequently diagnosed disease was IPF (30.5%). Dyspnea (75.9%) was the highest incidence among the presenting symptoms. Physical examination found bibasilar inspiratory crackles in 56.2 % and radiological findings included reticular opacities and interlobular septal thickenings in 55.9 % of the cases. It was observed that clinical and radiological findings were used most frequently (74.9%) as a diagnostic tool. While the most common treatment approaches were the use of systemic steroids and antifibrotic drugs with a rate of 30.7% and 85.6%, respectively. The total median cost from the patient’s admission to diagnosis was 540 Turkish Lira.Conclusion:We believe that our findings compared with data from other countries will be useful in showing the current situation of ILD in our country to discuss this problem and making plans for a solution.

Aim and introduction:Diagnosing of interstitial lung disease (ILD) is difficult and expensive. The standard diagnostical approaches to ILD are bronchoalveolar lavage, transbronchial lung biopsy, transbronchial lung cryobiopsy (TBLC) and surgical lung biopsy (SLB). SLB is gold standard for the confident diagnosis of ILD but because of the poor performance of the patients it’s use is limited. We conducted a retrospective study to point out that TBLC plays an important role in diagnosis of ILD and has fewer complications and lower cost than awake video-assisted thoracic surgery (AVATS).Material and methods:132 patients who underwent TBLC and AVATS with a pre-diagnosis of ILD in our hospital between 2015 and 2020 were evaluated retrospectively. Diagnosis rates, complications and costs were recorded.Results:There were no non-diagnostic materials in 44 patients in AVATS arm. Prolonged air leak was observed in 11(25.0%) of the patients, and six of them (13.6%) were discharged with Heimlich Valve (HV). Median length of stay in the hospital was 8 days, while average patient cost was $515.9 (415.2-2662.9) in the AVATS arm. Non-diagnostic material was obtained from 10 (11.3%) of 88 patients in TBLC arm. Six (6.8%) of them had pneumothorax, only one of them required a chest tube. No patient was discharged with HV (p=0.001). Median cost for each patient with a median hospital stay of 2.0 (1.0-21.0) (p<0.001) days was $171.9 (80.8-1493.3) (p<0.001).Discussion:Although TBLC is behind AVATS in terms of diagnostic accuracy, it may be an alternative diagnostic tool in the diagnosis of interstitial lung disease due to its acceptable safety profile and cost-effectiveness.

Background:Sarcoidosis can lead to variable periods of sickness and unemployment. Rehabilitation is recommended in sarcoidosis to improve exercise capacity. Therefore, focus on creating different and flexible rehabilitation options adapted to the needs of working patients is warranted to keep patients with sarcoidosis employed and to reduce the socioeconomic burden. Telerehabilitation (TR) might be an alternative. We investigated the usefulness and effectiveness of TR on exercise capacity in patients with sarcoidosis.Method:Single-center, prospective, randomized study including stable patients with sarcoidosis who were enrolled in either a control group where they received the usual standard of care (not including rehabilitation) or in the 3 months TR group composed of video and chat-consultations with a physiotherapist and workout sessions with a virtual autonomous physiotherapist agent (VAPA) (1). 6-minute-walk-test (6MWT), forced vital capacity (FVC), diffusion capacity of the lung for carbon monoxide (DLCO), isometric voluntary contraction (MVC), 7 days pedometry, Saint George Respiratory Questionnaire for interstitial lung disease (SGRQ-I), The King’s Brief Interstitial Lung Disease Questionnaire (KBILD) and General Anxiety Disorder-7 Questionnaire (GAD7) were tested before and after 3 months of TR, and after 3- and 6 months follow-up. Patient satisfaction was measured with a 5-point scale (5 very satisfied) and adherence was calculated as percent of tasks and time spent training. Adverse events were documented.Results:Thirty patients aged 53.9±13.5 years, male 63.3%, FVC% 88.9±18.8, DLCO% 65.2±16.0, 6MWT 513.1±141.3 were included. Fifteen patients were randomized to TR with VAPA and 15 patients to the control group. Differences in meters walked (6MWTD) between groups was at baseline (-28.9 m (p=0.58)), after 3 (+25.8 m (p=0.57)), 6 (+48.4 m (p=0.39)) and 9 months (+77.3 m (p=0.18)) follow-up in favor of telerehabilitation. No differences were observed in MVC, 7 days pedometry, SGRQ-I, KBILD or GAD7. Exercise adherence in the intervention group was 64% and average exercise time was 28 minutes per exercise session during the first 3 months. Patient satisfaction scored 3.8 ± 0.7. No adverse events were reported.Conclusion:VAPA TR did not result in any change in exercise capacity or patient-reported outcomes in this pilot study in patients with sarcoidosis. However, a statistically non-significant trend for improved 6MWTD was observed during follow-up. VAPA TR was safe, had high patient satisfaction and acceptable adherence. Further randomized studies including larger numbers of participants are needed.

Introduction:Echocardiographic measurement of the right ventricular systolic pressure (RVSP) is commonly used for estimating systolic pulmonary artery pressure (PASP) measured during right heart catheterization (RHC) in patients suspected for pulmonary hypertension (PH). Generally, there seems to be a strong correlation. However, this has been reported as less robust in sarcoidosis. We aim to investigate the correlation between RVSP and RHC measurements using real world data and analyzed factors influencing the relationship between RVSP and PASP in sarcoidosis.Methods & Results:Data of patients with and without sarcoidosis associated PH who had both a measurable echocardiographic RVSP and invasive PASP were collected from the RESAPH registry, PULSAR study and Cincinnati Sarcoidosis Clinic database (n=173, 60.1% female, mean age 56.0±9.5 years). Among them, 124 had PH confirmed by RHC. There was a strong correlation between RVSP and PASP (r=0.640). This correlation was significant in both male and female, white or non-white, forced vital capacity (FVC) >60%, and presence of fibrosis (p<0.001). However, it was less robust in patients with FVC of 50% or less. RVSP was considered inaccurate if the difference with PASP was > 10mmHg. Inaccurate echocardiographic estimation of the invasive PASP occurred in 50.8%, with overestimation mostly in patients without PH, and underestimation in patients with severe PH. An RVSP>50mmHg was associated with worse survival.Conclusions:In this real world multicenter cohort of sarcoidosis patients, we found a significant correlation between RVSP as determined by echocardiography and invasive PASP. Over- or underestimation of PASP occurred frequently. Therefore, echocardiographic RVSP measurement alone to screen for PH in sarcoidosis should be used with caution.