Chest High-resolution Tomography Research Articles

Tocilizumab plus Nintedanib for progressive interstitial lung disease in systemic sclerosis: a one-year observational study.

Randomized controlled trials have recently shown that both the IL-6 inhibitor Tocilizumab and the antifibrotic Nintedanib are efficacious for Systemic Sclerosis (SSc)-associated progressive interstitial lung disease (ILD). Since real-world clinical data on Tocilizumab/Nintedanib combination are lacking, we report on their long-term safety and efficacy. Consecutive patients who received off-label Tocilizumab for SSc plus Nintedanib for progressive ILD were retrospectively studied. Adverse events, and changes in Forced Vital Capacity (FVC), Diffucing Capacity for Carbon Monoxide (DLCO) and high resolution chest tomography (HRCT) between baseline and 6 and 12 months were assessed. Tocilizumab/Nintedanib combination was well tolerated by all 20 patients [aged 52 ± 13 years (mean ± SD), 14 women, 15 diffuse SSc, disease duration of 5.7 ± 4.9 years]; 7 of 20 patients received concomitant mycophenolate mofetil safely. No serious adverse events or laboratory abnormalities were noted. Five patients developed persistent diarrhea and 2 of them reduced dosage of Nintedanib. Baseline FVC (74%±12%) and DLCO (45%±10%) remained overall stable both at 6 months (73.5%±13% and 46%±11%, respectively) and 12 months (73%±14% and 45%±11%, respectively), regardless of disease duration. The extent of fibrotic reticular pattern in available pairs of HRCTs (n = 12) remained also stable at 12 months, whereas proportion (%) of ground glass opacities decreased from 29%±16to21%±14% (p = 0.048); improvement in HRCTs by almost 75% was noted in 2 of these12 patients. Tocilizumab/Nintedanib combination for one year was safe and stabilized lung function in real-world SSc patients with progressive ILD. Additional studies of this combination treatment in SSc-ILD are warranted.

Modelling the long-term health impact of COVID-19 using Graphical Chain Models

BackgroundLong-term sequelae of SARS-CoV-2 infection, namely long COVID syndrome, affect about 10% of severe COVID-19 survivors. This condition includes several physical symptoms and objective measures of organ dysfunction resulting from a complex interaction between individual predisposing factors and the acute manifestation of disease. We aimed at describing the complexity of the relationship between long COVID symptoms and their predictors in a population of survivors of hospitalization for severe COVID-19-related pneumonia using a Graphical Chain Model (GCM).Methods96 patients with severe COVID-19 hospitalized in a non-intensive ward at the “Santa Maria” University Hospital, Terni, Italy, were followed up at 3–6 months. Data regarding present and previous clinical status, drug treatment, findings recorded during the in-hospital phase, presence of symptoms and signs of organ damage at follow-up were collected. Static and dynamic cardiac and respiratory parameters were evaluated by resting pulmonary function test, echocardiography, high-resolution chest tomography (HRCT) and cardiopulmonary exercise testing (CPET).ResultsTwelve clinically most relevant factors were identified and partitioned into four ordered blocks in the GCM: block 1 - gender, smoking, age and body mass index (BMI); block 2 - admission to the intensive care unit (ICU) and length of follow-up in days; block 3 - peak oxygen consumption (VO2), forced expiratory volume at first second (FEV1), D-dimer levels, depression score and presence of fatigue; block 4 - HRCT pathological findings. Higher BMI and smoking had a significant impact on the probability of a patient’s admission to ICU. VO2 showed dependency on length of follow-up. FEV1 was related to the self-assessed indicator of fatigue, and, in turn, fatigue was significantly associated with the depression score. Notably, neither fatigue nor depression depended on variables in block 2, including length of follow-up.ConclusionsThe biological plausibility of the relationships between variables demonstrated by the GCM validates the efficacy of this approach as a valuable statistical tool for elucidating structural features, such as conditional dependencies and associations. This promising method holds potential for exploring the long-term health repercussions of COVID-19 by identifying predictive factors and establishing suitable therapeutic strategies.

Clinical profiles and treatment outcomes of outpatients with interstitial lung disease and mechanic's hands: A retrospective and observational cohort.

Idiopathic inflammatory myopathies, especially antisynthetase syndrome, often appear outside of the muscles as interstitial lung disease (ILD). Another typical finding is the presence of mechanic's hands. The aim of the present study was to describe the clinical, functional, tomographic, and serological data of patients with ILD and mechanic's hands and their response to treatment and survival rates. This is a retrospective study of ILD with concurrent myopathy. Among the 119 patients initially selected, 51 had mechanic's hands. All the patients were screened for anti-Jo-1 antibodies. An expanded panel of myopathy autoantibodies was also performed in 27 individuals. Of the 51 patients, 35 had 1 or more antibodies. The most common were anti-Jo-1, anti-PL-7, and anti-PL-12, while of the associated antibodies, anti-Ro52 was present in 70% of the 27 tested individuals. A significant response to treatment was characterized by an increase in predicted forced vital capacity (FVC) of at least 5% in the last evaluation done after 6 to 24 months of treatment. A decrease in predicted FVC of at least 5%, the need for oxygen therapy, or death were all considered treatment failures. All patients were treated with corticosteroids, and 71% with mycophenolate. After 24 months, 18 patients had an increase in FVC, 11 had a decrease, and 22 remained stable. After a median follow-up of 58 months, 48 patients remained alive and three died. Patients with honeycombing on high-resolution chest tomography (log-rank = 34.65; P < .001) and a decrease in FVC ≥5% (log-rank = 18.28, P < .001) had a poorer survival rate. Patients with ILD and mechanic's hands respond well to immunosuppressive treatment.

Scleroderma-overlap syndromes: capillaroscopy, laboratory, and clinical manifestations and follow-up compared to scleroderma patients.

Overlap syndrome (OS) is a group of systemic connective tissue diseases (CTDs) that meet the criteria of two or more CTDs. In this study, we evaluated clinical, laboratory, and capillaroscopic manifestations of patients with scleroderma OS (SSc-OS) and its subgroups and follow-up progression compared to patients with limited SSc (LcSSc). In a 10-year cross-sectional study, we evaluated 135 adult patients (70 with SSc-OS and 65 with LcSSc) with the same skin score for their baseline and follow-up clinical, laboratory, high-resolution chest tomography (HRCT), echocardiography, and nailfold capillaroscopy data and compared them. Of the 135 patients, 70 had SSc-OS, including 45 (64.3%) cases of SSc-SS (Sjögren's syndrome), 11 (15.7%) of SSc-RA (rheumatoid arthritis), 9 (12.9%) of SSc-myositis and 5 (1.7%) of SSc-SLE (systemic lupus erythematosus), and 65 had LcSSc. Lung and heart involvement and pulmonary arterial hypertension (PAH) did not differ between the two groups (p > 0.05). Musculoskeletal involvement and non-specific pattern of capillaroscopy were higher (p = 0.035 and p = 0.001), and digital ulcer (DU) and scleroderma patterns of capillaroscopy were lower in the SSc-OS group (p = 0.000).No significant relationship was found between capillaroscopic patterns and organ involvement in the two groups (p-value > 0.05). In the follow-up (3.71 ±2.63 years), new DU and progression of lung involvement (p = 0.002) and the progression in capillaroscopic patterns was lower in SSc-OS (p = 0.000). In the follow-up, new DU was not seen in the SSc-OS, with lower progression of lung involvement, skin score, and capillary damage. In SSc-OS patients, the most common subgroup was SSc-SS. Scleroderma OS was associated with lower major organ involvement and capillaroscopy progression than LcSSc. Major organ involvement in patients with SSc-OS was significantly lower than in LcSSc patients. In the follow-up, new DU was not seen in the SSc-OS with lower progression of lung involvement, skin score, and capillary damage.

Non-cystic fibrosis bronchiectasis: A single-centre retrospective study in Johannesburg, South Africa.

Bronchiectasis, once rarely encountered, appears to be increasing in prevalence in South Africa (SA) and globally. There is a lack of published data on non-cystic fibrosis (CF) bronchiectasis, specifically in low- to middle-income countries, despite the high rates of risk factors such as HIV, pulmonary tuberculosis, and other infections. Given this lack of data, to review the characteristics of adult patients with non-CF bronchiectasis at a tertiary academic hospital in Johannesburg, SA. This was a single-centre, retrospective record review, including all cases of non-CF bronchiectasis that were in the records of the adult pulmonology clinic at Charlotte Maxeke Johannesburg Academic Hospital as of April 2017. There were 197 patients, with a slight predominance of males, and the patients were generally young. The HIV rate was higher than the national average (34.8% v. 13.7%), and the HIV-positive patients had a high TB prevalence (86.9%). Pseudomonas spp. were cultured from sputum in 15.3% of cases. Fewer than half of the cohort had the diagnosis of bronchiectasis confirmed by high-resolution chest tomography. Airway obstruction (forced expiratory volume in 1 second/forced vital capacity ratio <70%) was observed in 47.0% of patients. Treatment with a short-acting beta-2-agonist was prescribed in 62.9%, a long-acting beta-2-agonist in 43.6% and inhaled corticosteroids in 51.3%. Antibiotic therapy during exacerbations was used in 44.2%, mainly amoxycillin-clavulanate (66.7%). While single centre and retrospective, this study adds to the data on non-CF bronchiectasis in sub-Saharan Africa and should encourage further research to increase our understanding of adult non-CF bronchiectasis in SA. What the study adds. This study adds to published data detailing the clinical characteristics of adult non-cystic fibrosis (CF) bronchiectasis in low- and middle-income countries (LMICs).Implications of the findings. As a retrospective descriptive study, the findings summarise the characteristics of adults with non-CF bronchiectasis in a cohort from Johannesburg, South Africa. The findings suggest that the characteristics of bronchiectasis in this region appear to be similar in several ways to those in other LMICs, but quite different from those in the developed world.

Use of Roflumilast on the incidence of exacerbations in patients with non-fibrocystic supurative bronchiectasis

Bronchiectasis is an irreversible inflammatory disease of the airways, noted on chest high-resolution tomography (HRCT). The dilation is provoked by the elastic and muscular degradation of the bronchial walls and its appearance is conditioned to genetic and acquired factors. Manifested with persistent cough, associated with expectoration of mucopurulent sputum, dyspnea, hemoptysis. After accompanying the treatment of patients in 3 public hospitals and 1 private practice in the city of João Pessoa, this text presents the use of Roflumilast as a therapeutic option for the prevention of exacerbations of bronchiectasis.

Pulmonary hemosiderosis secondary to mitral stenosis: a case report

BackgroundPulmonary hemosiderosis is a rare disease that may be idiopathic or have a secondary etiology, such as mitral stenosis. The disease is a clinical and functional consequence of iron overload in the lungs in the form of hemosiderin. The diagnosis should be considered in patients with miliary nodules on chest tomography.Case presentationWe report a case of a 30-year-old man with severe mitral stenosis who presented with chest pain and dyspnea. High-resolution chest tomography showed bilateral centrilobular micronodules. The diagnosis of pulmonary hemosiderosis secondary to mitral stenosis was suggested by transbronchial biopsy.ConclusionThe diagnosis of idiopathic pulmonary hemosiderosis requires the exclusion of other etiologies of alveolar hemorrhage, including infections and vasculitis. One possible etiology is mitral stenosis.

Evolution of Rheumatoid-Arthritis-Associated Interstitial Lung Disease in Patients Treated with JAK Inhibitors: A Retrospective Exploratory Study.

The aim of this multicenter retrospective study was to investigate the effectiveness and safety of the available JAK-inhibitors (JAKi) in patients with rheumatoid arthritis (RA) and interstitial lung disease (ILD). We retrospectively analyzed patients with classified RA and RA-ILD undergoing JAKi in 6 Italian tertiary centers from April 2018 to June 2022. We included patients with at least 6 months of active therapy and one high-resolution chest tomography (HRCT) carried out within 3 months of the start of JAKi treatment. The HRCT was then compared to the most recent one carried out within 3 months before the last available follow-up appointment. We also kept track of the pulmonary function tests. We included 43 patients with RA-ILD and 23 males (53.48%) with a median age (interquartile range, IQR) of 68.87 (61.46-75.78) treated with JAKi. The median follow-up was 19.1 months (11.03-34.43). The forced vital capacity remained stable in 22/28 (78.57%) patients, improved in 3/28 (10.71%) and worsened in 3/28 (10.71%). The diffusing capacity of lung for carbon monoxide showed a similar trend, remaining stable in 18/25 (72%) patients, improving in 2/25 (8%) and worsening in 5/25 (20%). The HRCT remained stable in 37/43 (86.05) cases, worsened in 4/43 (9.30%) and improved in the last 2 (4.65%). This study suggests that JAKi therapy might be a safe therapeutic option for patients with RA-ILD in a short-term follow-up.

Criteria for progressive fibrotic hypersensitivity pneumonitis in a Portuguese patient cohort.

Hypersensitivity pneumonitis (HP) is a syndrome caused by sensitisation to inhaled antigens that leads to an abnormal immune response in the airways and lung parenchyma. Some patients previously diagnosed with certain types of fibrotic interstitial lung diseases (f-ILDs), including fibrotic HP (f-HP), are susceptible to develop a progressive fibrosing phenotype (PF-ILD), despite initial state-of-the-art management. To characterise a cohort of patients with a multidisciplinary diagnosis (MTD) of chronic f-HP, who were followed up in an ILD outpatient clinic of a hospital in Portugal, and to assess the prevalence of PF-ILD criteria in these patients. Data were collected from all patients with a definite or provisional diagnosis of f-HP after a multidisciplinary team discussion. Patients were followed up between December 2014 and July 2019. Data included clinical characteristics, high-resolution chest tomography (HRCT) disease patterns, lung function tests, bronchoalveolar lavage and further immunological work-up, biopsy reports (conventional transbronchial lung biopsy, transbronchial lung cryobiopsy or surgical video-assisted thoracoscopic lung biopsy), all ILD multidisciplinary team records and diagnostic confidence levels. Patients were assessed according to PF-ILD criteria as defined in the INBUILD trial. We identified 83 patients with an MTD of HP, who had been followed up for at least 12 months. Of these, 63 (75.9%) were diagnosed with f-HP. Of the 63 f-HP patients, 33.3% (n=21) fulfilled the predefined criteria for PF-HP: 66.7% had a relative decline of ≥10% forced vital capacity (FVC); 5% a relative decline of 5 - 9% FVC, with worsening symptoms or increased fibrosis on HRCT; and 23.8% had worsening respiratory symptoms with radiological progression. This single-centre cohort study demonstrated that a third of f-HP patients presented with PF-ILD, as determined by progression during initial standard-of-care treatment. A usual interstitial pneumonia (UIP)/UIP-like pattern was present in >70% of patients with f-HP, and two-thirds of these patients had an FVC decline of ≥10%. PF-HP patients were also more exacerbation prone. According to recent trial data, this segment of patients can be considered possible candidates for antifibrotic treatment, with a reasonable prospect of effectiveness. Further efforts should focus on refining knowledge of longitudinal behaviour of large multicentric cohorts of f-HP patients, establishing a consensual and uniform definition of progression for use in clinical practice, as well as developing prognostic prediction tools to better (and early) inform the disease course.

Chest ultrasound findings in usual interstitial pneumonia patterns: a pilot study

It is unknown what role chest ultrasound plays in distinguishing the various usual interstitial pneumonia (UIP) patterns of high-resolution chest tomography (HRCT). The purpose of this study was to see if there was a link between the results of chest ultrasound (u/s) and HRCT in patients with idiopathic pulmonary fibrosis (IPF). We performed chest u/s in 16 patients with UIP and probable UIP patterns to indeterminate UIP and alternative diagnosis patterns in this single center prospective study to determine any possible relationship with the HRCT findings. A chest radiologist reviewed each HRCT to determine the pattern in accordance with the American Thoracic Society (ATS) / European Respiratory Society (ERS) Guidelines. The local multidisciplinary committee validated the patients' diagnoses before they were included. When compared to the indeterminate for UIP or alternative diagnosis pattern group, there was a trend (p=0.07) toward the presence of more B lines in UIP or probable UIP patterns. There was no statistically significant difference in the presence of small, large, white lung, or pleural line thickening >5mm. Subgroup analysis revealed that patients with honeycombing were more likely to have a fragmented pleural line (p=0.04). To summarize, in our pilot study, chest u/s appears unable to differentiate UIP and probable UIP patterns from indeterminate UIP and alternative diagnosis patterns. However, it appears that this technique can be used to recognize the honeycombing pattern.

Bronquiolitis obliterante pediátrica asociada a síndrome de Stevens-Johnson, reporte de un caso

El síndrome de Stevens-Johnson (SSJ) se caracteriza por ampollas y necrosis generalizadas de la piel y la mucosa. Las secuelas a largo plazo y los desencadenantes en los niños pueden diferir de los reportados en adultos. Los medicamentos e infecciones suelen ocasionarla. Los agentes etiológicos infecciosos más comunes son el Adenovirus y el Mycoplasma pneumoniae. La bronquiolitis obliterante es una complicación poco frecuente que se produce después de una injuria severa del tracto respiratorio inferior que conduce a estrechamiento y/u obliteración de las vías respiratorias pequeñas. Se presenta el caso de una paciente de sexo femenino de 15 años de edad, atendida en el Hospital de Niños Dr. Roberto Gilbert Elizalde, con antecedente de síndrome de Stevens-Johnson secundario a la administración de amoxicilina y paracetamol cuatro meses previo al diagnóstico de bronquiolitis obliterante. El diagnostico se efectuó con base en la clínica presentada al ingreso, el antecedente de SSJ y los hallazgos de la tomografía de tórax de alta resolución. El tratamiento se efectuó con tiotropio, azitromicina y prednisona en días alternos y se observó evidente recuperación en varios controles posteriores.

Is There a Link Between Nailfold Videocapillaroscopy and Pulmonary Function Tests in Systemic Sclerosis Patients?: A 24-Month Follow-up Monocentric Study.

The aim of this study was to explore the associations between nailfold videocapillaroscopy (NVC) and pulmonary function tests (PFTs) in systemic sclerosis (SSc) patients. This was a longitudinal study with follow-up of unselected Brazilian SSc patients. Baseline clinical examination, serological workup, high-resolution chest tomography, and NVC were performed. Pulmonary function test was performed at baseline and after 24 months. Pulmonary function test worsening over time was defined as either a ΔFVC decline ≥10% or a ΔFVC decline ≥5% and <9%, combined with a ΔDLCO decline ≥15%, at 24 months. The NVC parameters were quantitatively (capillary density number, dimension, morphology, and hemorrhages) and qualitatively (NVC pattern) evaluated according to the consented standardized definitions of the EULAR Study Group on Microcirculation in Rheumatic Diseases. Seventy-nine patients were included. Fifty-nine were rated to have a scleroderma pattern (6 "early"/16 "active"/37 "late"). The mean FVC and DLCO were 76.8% and 67.2% at baseline and 73.8% and 64.3% at 24 months, respectively. After multivariate analysis, we found that a reduced baseline FVC was associated with reduced capillary density (odds ratio [OR], 11; 95% confidence interval [CI], 1.5-90.7; p = 0.03) and a reduced baseline DLCO associated with the late scleroderma pattern (OR, 6.75; 95% CI, 1.09-42; p = 0.03). No association between worsening of PFT over time and NVC was found. The association between NVC and PFTs might corroborate the link between microangiopathy and interstitial lung disease in patients with SSc. This finding might strengthen the idea of incorporating NVC as a tool to predict progressive interstitial lung disease in these patients in the future.

Interstitial lung disease in systemic sclerosis: From immunopathogenesis to treatment

Abstract Interstitial lung disease (ILD) is a pulmonary involvement that is commonly manifested in systemic sclerosis (SSc) patients. The immunopathogenesis of SSc-ILD involves several mechanisms, including microvascular injury, alveolar epithelial cell defect, inflammation, genetics, epigenetics, telomeres, telomerase and inflammasome, which result in lung fibrosis. Detection of ILD should be performed in every SSc patient by using high-resolution chest tomography (HRCT) scan of the thorax, in addition to evaluation by pulmonary function tests. When ILD is discovered, the physician might start treatment considering factors such as the extent of the lesion, progressivity of the disease, prognosis and drug toxicity. The current guideline recommends cyclophosphamide, mycophenolate mofetil and nintedanib as the initial choices for SSc-ILD treatment. Other agents such as biologic immunotherapies, haematopoietic stem cell transplantation and lung transplantation could be an option if the disease becomes progressive.

Quantitative chest tomography indexes are related to disease activity in systemic sclerosis: results from a cross-sectional study.

The aim of this study is to verify if there are correlations between quantitative chest tomography (QCT) indexes and disease activity (DA) in a cohort of patients with systemic sclerosis (SSc). SSc patients were assessed for DA and underwent high resolution chest tomography (CT). CT images were analysed with an operator-independent algorithm extracting the QCT indexes. DA assessment was conducted according to the EUSTAR index, where a score ≥2.5 indicates high DA (hDA). Correlations between clinical data and QCT indexes were investigated with the Spearman's test. The Mann-Whitney test assessed the distribution of the QCT indexes among the groups. Receiver operating characteristics (ROC) curve and linear regression analysis were conducted in order to identify the best cut-off value and contribution for each QCT index in assessing hDA in SSc patients. Sixty patients (52 females, mean age 53.2 years, mean disease duration 5.3 years) were enrolled. A significant difference was found in QCT indexes distribution between patients with hDA and those with low DA. A mild strength correlation between QCT indexes and DA was observed. Once performed ROC curves and linear regression, Skewness on parenchymal lung <1.85 gave a significant contribution to the model in identifying subjects with hDA (p<0.001), showing sensitivity 79.5%, specificity 68.7%, and accuracy 76.6%. QCT indexes correlate with SSc DA. These data introduce new possibilities for QCT application in clinical practice, especially in patient's follow-up. Moreover, QCT could be implemented in a new SSc DA score based on operator-independent parameters.

Effect of dupilumab treatment in a severe asthma patient with EGPA

Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis characterized by multisystemic manifestations, including severe asthma.1 Airflow obstruction and asthma symptoms persist in a high percentage of EGPA patients despite steroid treatment.2 We describe a case of a 36-year-old man previously diagnosed with antineutrophil cytoplasmic antibody (ANCA)-negative EGPA in 2012 based on the presence of marked peripheral blood eosinophilia (2.12 × 109 cells/L; 26%), severe asthma, pulmonary infiltrates at high-resolution chest tomography (HRCT) scan, nasal polyps and peripheral neuropathy, but not heart, gastrointestinal and renal involvement, and absence of direct demonstration of vasculitis.

OP0174 SUBCLINICAL INTERSTITIAL LUNG DISEASE IS FREQUENT AND PROGRESSES ACROSS DIFFERENT CONNECTIVE TISSUE DISEASES

Background:Based on the argument that symptoms define disease, physicians commonly apply the terms “preclinical” or “subclinical” disease to describe patients with disease-related findings but no accompanying symptoms for connective tissue disease associated interstitial lung disease (CTD-ILD). The term subclinical frequently applies to patients with mild ILD changes on high resolution chest tomography (HRCT), normal forced vital capacity (FVC), and without respiratory symptoms. Previous work in systemic sclerosis (SSc)-ILD did show that patients with even minor extent of ILD at baseline often progressed and had increased mortality risk, suggesting that it is not appropriate to define these patients as “subclinical.”Objectives:To identify the prevalence of subclinical ILD across CTD diagnoses, and assess the rate of progression of lung fibrosis compared to CTD without ILD and with clinical ILD.Methods:All CTD patients, including SSc, anti-synthetase syndrome (ASS) and mixed connective tissue disease (MCTD) from the Oslo University Hospital diagnosed before 2015 and assessed for the presence of ILD by HRCT were included. The year 2015 was chosen to secure an observation time of at least five years from ILD diagnosis to study end on 01.01.2021 or time of death. All patients fulfilled the respective CTD classification criteria. Subclinical ILD was defined as an ILD extent &lt;5% by semi-quantitative assessment of baseline HRCT, preserved lung function with FVC &gt;80% predicted and without respiratory symptoms. Clinical ILD was defined as &gt;5% extent of ILD or &lt;5% extent of ILD on HRCT with respiratory symtoms or FVC&lt;80%. The outcome was ILD progression, defined as increasing extent of ILD from basline to follow-up HRCT by semi-quantitative assessment. Vital status was available in all patients and mortality was evaluated. Descriptive statistical analyses were conducted and time to ILD progression determined by Kaplan-Meier estimates.Results:We identified 525 CTD patients, including 296 with SSc, 135 with MCTD and 94 with ASS who had conducted a baseline HRCT. Of these, 227 (43%) had no ILD, 67 (13%) subclinical and 231 (44%) clinical ILD (Table). Of the 67 subclinical ILD patients, 45 (15%) had SSc, 13 (10%) MCTD and 9 (10%) ASS of thespecific cohorts. Over a median time of 4.5 years between baseline and follow-up HRCT, 95/395 (24%) showed progression of ILD, including 72 (26%) SSc and 23 (19%) MCTD patients. Disease progression frequently occurred in both subclinical ILD (38%) and clinical ILD (51%) patients (Figure). Age, gender, underlying CTD, and baseline lung function were not predictive for the progression of lung fibrosis. Progression was too infrequent to allow for meaningful multivariable regression analyses. After a median observation period of 12 years, 153 (29%) of the patients died. The 1-, 5- and 10-year survival rates in those without ILD, subclinical and clinical ILD were 97%/97%/99%, 88%/91%/82%, and 82%/85%/68% (p&lt;0.001), respectively.Table 1.Clinical characteristics, demographics and outcomeNo ILD (n=227)Subclinical ILD (n=67)Clinical ILD (n=231)Age, y (SD)50 (15.4)51 (14.4)52 (15.3)Male sex, n (%)89 (39)22 (33)111 (48)Deceased, n (%)50 (22)12 (18)91 (39)Observation period, y median (range)13.7 (18.6)13.9 (17.9)11.5 (17.1)FVC% (SD)97 (18.6)99 (17.9)81 (20.9)FVC decline% (SD)-0.70 (11.1)-0.81 (16.5)-1.61 (15.9)DLCO% (SD)73 (19.4)73 (16.9)55 (17.4)Extent of ILD% (SD)0 (0)2.3 (1.5)19.3 (16.8)ILD progression% (SD)0.08 (1.0)3.1 (6.2)3.6 (9.9)ILD progressors, n (%)3 (2)20 (38)72 (51)Figure 1.Time to ILD progression in CTD without ILD, with subclinical and clinical ILDConclusion:Subclinical ILD is frequently present across CTDs and progresses over time in a substantial subgroup of patients, comparable to patients with clinical ILD. Our findings question the terms sub- and preclinical ILD, which may potentially lead to a suboptimal “watchful waiting management strategy”. Monitoring all CTD patients with any ILD is of high importance to identify disease progression early.Disclosure of Interests:Anna-Maria Hoffmann-Vold Speakers bureau: Actelion, Boehringer Ingelheim, Roche, Merck Sharp &amp; Dohme, Lilly and Medscape, Consultant of: Actelion, Boehringer Ingelheim, Bayer, ARXX, and Medscape, Grant/research support from: Boehringer Ingelheim, Helena Andersson: None declared, Silje Reiseter: None declared, Håvard Fretheim Consultant of: Actelion, Bayer., Imon Barua: None declared, Torhild Garen: None declared, Øyvind Midtvedt: None declared, Ragnar Gunnarsson: None declared, Mike Durheim Speakers bureau: Boehringer Ingelheim and Roche, Consultant of: Boehringer Ingelheim, Grant/research support from: Boehringer Ingelheim, Trond M Aaløkken: None declared, Øyvind Molberg: None declared

Bronchoalveolar Lavage Complements Transbronchial Cryobiopsy Diagnosis in Diffuse Interstitial Lung Diseases.

Bronchoalveolar lavage (BAL) is a technique classically used for the study of diffuse interstitial lung diseases (DILDs). Given the recent advances in the diagnosis of DILD by transbronchial cryobiopsy (TBCB), it is relevant to assess what BAL can contribute to TBCB. This is a retrospective descriptive study that included patients with DILD who, between 2013 and 2017, underwent BAL and TBCB in the same bronchoscopy intervention. We evaluated the complementary information provided by BAL to TBCB that facilitated the diagnosis by a multidisciplinary committee. Epidemiological, clinical, and functional variables and high-resolution chest tomography findings were recorded, along with complications associated with the procedures. A total of 60 patients were included. TBCB, conditioned by the underlying radiologic pattern, provided diagnostic information in 75% of cases. BAL provided complementary information that supported the diagnosis and treatment in 22% of cases. Differential BAL findings were related to microbiology, cell count, and immunology. Regarding the safety of the procedure, 47% of the patients experienced complications, although none were serious. BAL findings contribute to TBCB findings in the diagnosis of DILDs, with no serious complications associated with their combined use.

Chronic Bronchitis in COPD Patients Creates Worse Symptom Burden Regardless of the Presence of Bronchiectasis in the COPDGene Cohort.

To assess whether the presence or absence of bronchiectasis has an impact on the patient-reported symptoms of chronic obstructive pulmonary disease (COPD) patients. The study included participants from the COPD Genetic Epidemiology Study (COPDGene®) cohort with available high-resolution chest tomography reporting the presence or absence of bronchiectasis (BE+/BE-) and survey data reporting the presence or absence of chronic bronchitis symptoms (CB+/CB-). Patient symptoms based on the St George's Respiratory Questionnaire (SGRQ) were then compared for the different groups. The study population included 7976 participants, mean age 60, Global initiative for chronic Obstructive Lung Disease (GOLD) stages 0 to 4, 18.8% BE+, and 19.5% CB+. The presence or absence of radiographic bronchiectasis was not associated with higher frequency of chronic bronchitis (GOLD 0 group odds ratio [OR] 1.01 [0.78,1.31], GOLD 1-2 group OR 1.19 [0.95, 1.50], GOLD 3-4 group OR 1.26 [0.99, 1.60]). Similarly, CB+ participants had higher SGRQ scores than CB- participants regardless of the presence of BE. Across all GOLD stages, chronic bronchitis symptoms are associated with worse pulmonary symptoms and significant impairment in quality of life. For patients with chronic bronchitis, the presence or absence of bronchiectasis is not associated with a significant difference in SGRQ symptom scores. Symptoms of chronic bronchitis impose a heavy burden on patients and should be treated regardless of the presence or absence of underlying bronchiectasis.

Health-related quality of life in patients with bronchiolitis obliterans.

Bronchiolitis obliterans (BO) is mainly caused by infections and hematopoietic stem cell transplantation (HSCT). This study aimed to investigate the health-related quality of life (HRQOL) of children with BO compared to the healthy children and also to assess the HRQOL according to the etiology. Postinfectious (group 1) and post-HSCT BO (group 2) patients and healthy children were included in the study. HRQOL was assessed by the Short Form-36 (SF-36) and St George's Respiratory Questionnaire (SGRQ). Correlations between demographic and clinical characteristics, pulmonary function tests, high-resolution chest tomography scores, and HRQOL were assessed. Thirty-seven postinfectious and post-HSCT BO patients and 34 healthy children were included in the study. Mean age was 13.8 ± 0.7 years. Mean forced vital capacity and forced expiratory volume1 were 60.7 ± 2.7% predicted, and 49.8 ± 3.1% predicted, respectively. The SF-36 scores were lower in BO patients compared to healthy children (P < .01). Patients with better lung functions had higher SF-36 scores, but lower SGRQ. The number of inhaled therapies, acute exacerbations, hospitalizations were inversely correlated with SF-36. A positive correlation was found between these parameters and total SGRQ scores (r = .507, P = .02; r = .409, P = .12; r = .326, P = .049, respectively). SF-36 scores were better in group 1 for subscales of physical role functioning and social role functioning compared to group 2. (P = .01, P = .01, respectively). The HRQOL of patients with BO measured by SF-36 was low compared to healthy children. SF-36 scores were more affected in post-HSCT BO patients. HRQOL of children with chronic lung disease should be taken into consideration in the management of these patients.

CASE REPORT: BI-APICAL PULMONARY FIBROSIS WITH BRONCHIECTASIS IN A PATIENT WITH ANKYLOSING SPONDYLITIS

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Ankylosing spondylitis (AS) is a chronic inflammatory disease primarily affecting joints of the axial skeleton. In addition to disabling joint changes, pulmonary manifestations beyond the mechanical restrictive syndrome imposed by costovertebral joint ankylosis have been described [1]. These changes are seen on chest X-ray (CXR), pulmonary function tests (PFTs), bronchoalveolar lavage (BAL), and high-resolution chest tomography (HRCT) in varying degrees. They do not correlate with severity and duration of the primary disease [2]. Of the pulmonary findings, apical fibrosis is among the most distinctive. CASE PRESENTATION: We report a case of a 56-year-old male with long-standing AS (on indomethacin) who developed a dry cough with intermittent dyspnea and lung changes that were followed radiographically for several years. An initial CXR obtained for unrelated pre-operative purposes was unremarkable 21 years into his AS disease course. Two years after the first CXR, a right apical mass-like density with associated pleural thickening was detected. A QuantiFERON test was negative. Further evaluation by a computed tomography (CT) scan of the chest demonstrated volume loss of the right upper lobe with associated ground-glass opacities and pleural thickening. PFTs were consistent with a restrictive pattern, though the results were technically confounded by cough. Three years later, a follow-up CT scan of the chest showed significant progression to severe bi-apical fibrosis of the lungs with bronchiectasis and pleural thickening. Throughout this time, his joint disease remained stable, but his chronic cough worsened to the point of post-tussive emesis. DISCUSSION: The literature reports that a patient with AS with a normal CXR, normal PFTs, and lack of respiratory symptoms can still have pulmonary manifestations detectable by HRCT [3]. This patient’s cough first started 12 years before his first abnormal CXR. Despite his initial normal CXR, it is likely that radiographic evidence of the pulmonary changes predates this if evaluated by advanced chest imaging modalities. The pathogenesis of these pulmonary complications remains unknown; however, studies of BAL fluid have shown that subclinical inflammation of the alveoli is not a contributor [2-3]. CONCLUSIONS: The clinical significance of these early pulmonary changes is not known. There is a lack of studies evaluating benefit in obtaining advanced chest imaging on asymptomatic patients with AS. These changes were thought to be rare; however, newer studies suggest a higher prevalence. For patients presenting with respiratory symptoms with inflammatory arthritis, it is important to consider pleuropulmonary changes (ranging from airway disease to parenchymal scarring) in setting of AS. Reference #1: Kiris A, Ozgocmen S, Kocakoc E, Ardicoglu O, Ogur E. Lung findings on high resolution CT in early ankylosing spondylitis. Eur J Radiol. 2003;47(1):71-76. Reference #2: Kchir MM, Mtimet S, Kochbati S, et al. Bronchoalveolar lavage and transbronchial biopsy in spondyloarthropathies. J Rheumatol. 1992;19(6):913-916. Reference #3: Quismorio FP. Pulmonary involvement in ankylosing spondylitis. Curr Opin Pulm Med. 2006;12(5):342-345. https://doi.org/10.1097/01.mcp.0000239551.47702.f4 DISCLOSURES: No relevant relationships by Sudhir Rajan, source=Web Response No relevant relationships by Eduardo Solbes, source=Web Response No relevant relationships by Paulina Sun, source=Web Response