Usual Interstitial Pneumonia Pattern Research Articles

Prognostic impact of home oxygen therapy on patients with resected non-small-cell lung cancer with interstitial lung disease.

Non-small-cell lung cancer (NSCLC) patients with interstitial lung disease (ILD) have a poor prognosis. The present study evaluated the prognostic impact of home oxygen therapy (HOT) in NSCLC patients with ILD. Overall, 3099 consecutive patients underwent complete resection of stage IA to IIIA NSCLC at our institution between 2002 and 2016. ILD was diagnosed and categorized based on high-resolution computed tomography. The criteria for HOT included less than 90% resting oxygen saturation in the peripheral arteries and severe exertional dyspnea. We retrospectively compared the overall survival between ILD patients with and without HOT. ILD was observed in 150 (5%) patients. Seventeen (11%) patients needed HOT at discharge. The incidences of usual interstitial pneumonia (UIP) pattern (p = 0.03) and blood loss (p < 0.01) were significantly higher in the patients requiring HOT than in those without HOT. Significantly more patients developed complications (p = 0.04) in the HOT group than in the non-HOT group, with three (18%) having acute exacerbations. The 3-year overall survival rate was significantly lower in the HOT patients than in those without HOT (28% vs. 63%, p = 0.03). Patients requiring postoperative HOT showed a significantly poorer prognosis after complete resection than those without HOT. Therefore, the indication for surgery should be investigated cautiously in order to prevent the need for postoperative HOT.

ЕВОЛЮЦІЯ ПРИНЦИПІВ ДІАГНОСТИКИ І ТЕРАПІЇ ІДІОПАТИЧНОГО ЛЕГЕНЕВОГО ФІБРОЗУ У ПОЛОЖЕННЯХ МІЖНАРОДНИХ КЛІНІЧНИХ НАСТАНОВ

EVOLUTION OF PRINCIPLES OF DIAGNOSIS AND TREATMENT OF IDIOPATHIC PULMONARY FIBROSIS IN THE INTERNATIONAL GUIDELINE STATEMENTS Y. I. Feshchenko, V. K. Gavrysyuk, N. G. Gorovenko, Y. A. Dziublyk, I. V. Liskina Abstract Idiopathic pulmonary fibrosis (IPF) is specific form of chronic progressive interstitial fibrosing pneumonia of unknown nature, mainly occurring in patients &gt; 50 years of age, limited to the lungs and associated with histological and/or radiological pattern of usual interstitial pneumonia. Epidemiological studies estimate that prevalence of ILF in different countries varies between 1,25 and 63 cases per 100 000 persons. Along with that, ILF is characterized by unfavorable prognosis — median survival time ranges within 2,5−3,5 years from the time of diagnosis. In 2000 American thoracic society (ATS) and European respiratory society (ERS) published first international statement on diagnosis and treatment of ILF — American Thoracic Society, European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Data from studies, accumulated during next 10 years of research, determined the necessity of update of certain diagnostic criteria and principles of therapy. In this regard, a new guideline for diagnosis and treatment of IPF was published in 2011 and approved by ATS, ERS, Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT) — An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. A new update on “Treatment of IPF” was published in 2015, and chapter “Diagnosis” was updated later in 2018. Current literature review focuses on the principles and algorithms of IPF treatment and the changes in guidelines, occurred from the time of first Statement published. Summarizing review results, we can conclude, that evolution of diagnostics principles, which limited the indications for surgical lung biopsy for the purpose of diagnosis verification, is caused by explosive technological advances in the field of chest computed tomography. The capability of computed tomography has grown to such an extent, that in terms of morphological diagnosing this method can compete with histological examination. The changes in management principles were evoked by the revision of IPF pathogenesis mechanisms. Since 2000, most of the experts concluded, that fibrosis, rather than inflammation, was the leading link of pathogenesis. A fibrosing process, initially playing a reparative role, further due to unknown reasons, gains an uncontrolled progressive character. Accordingly, antifibrotic compounds, such as pirfenidone and nintedanib, came to substitute glucocorticosteroids, known for their powerful antiinflammatory potential. The publication also presents the review of most relevant randomized clinical trials on safety and efficacy of antifibrotic drugs — pirfenidone and nintedanib. Key words: idiopathic pulmonary fibrosis, diagnostics, treatment, algorithms, pirfenidone, nintedanib. Ukr. Pulmonol. J. 2021;29(3):5–23.

Clinical characteristics and outcomes in patients with primary Sjogren's syndrome-associated interstitial lung disease.

BACKGROUND:Diagnosing primary Sjogren's syndrome (pSS)-associated interstitial lung disease (ILD) is complex and can be very challenging. In addition, information about the prognostic factors is limited.AIMS:We aimed to determine the clinical characteristics and prognostic factors that impact pSS-ILD survival.METHODS:This retrospective review included 84 consecutive patients diagnosed with pSS-ILD. The information analyzed included the clinical characteristics, laboratory findings, and physiological and hemodynamic data. Prognostic factors were identified using a Cox proportional hazards regression model.RESULTS:The mean age was 60.5 years, and 61.9% were females. The mean time between the onset of symptoms and diagnosis was 21 months (range, 1–98 months). Minor salivary gland biopsy (MSGB) was positive for pSS in 92.3% of the cohort. Fifty percent of the patients had negative autoimmune serology related to pSS. Based on the available hemodynamic data, 40% had pulmonary hypertension (PH), and 20% had severe PH. During follow-up, acute exacerbation was noted in 38% of the cohort. The 5-year survival rate for all patients was 56%. Male sex, usual interstitial pneumonia pattern, and a reduced forced vital capacity were independent predictors of mortality in the pSS-ILD patients.CONCLUSIONS:A significant delay between the onset of symptoms and diagnosis was noted in our cohort. Importantly, our study highlights the importance of MSGB and emphasizes that clinicians should not rely solely on serological tests to diagnose pSS in ILD patients. The overall survival was poor, and more efforts are needed to diagnose pSS-ILD at an early stage and refer patients to experienced centers.

Update in Diagnosis of Idiopathic Pulmonary Fibrosis and Interstitial Lung Abnormality

최신 국제 임상진료지침을 기반으로 한 특발폐섬유증의 진단은 부합하는 임상 소견과 함께 고해상 CT에서 전형적인 상용간질폐렴 소견을 보일 때 조직학적 폐 생검 없이 진단 가능하다. 영상 검사는 특발폐섬유증의 평가 및 진단에 중추적인 역할을 하며, 정확한 진단을 위하여 임상적, 영상의학적 및 병리학적 소견에 대한 다학제 검토의 중요성이 강조된다. 간질성 폐이상(interstitial lung abnormality)은 우연히 발견된 영상의학적 이상 소견을 지칭하며, 간질성폐이상과 임상적으로 의미 있는 간질폐질환에 대한 구분은 적절한 임상 평가를 기반으로 이루어져야 한다. 저자들은 이번 종설을 통하여 특발폐섬유증 진단의 최신 지견 및 간질성폐이상에 대한 이해를 도움으로써 미만성 간질폐섬유증 환자의 정확한 진단과 치료 및 예후 증진에 도움이 되고자 한다.

DPO: Diffuse Pulmonary Ossification - A Diagnostic Challenge.

Diffuse pulmonary ossification (DPO) is a rare condition of DLD (diffuse lung disease) characterized by the presence of metaplastic ectopic bone in the lungs and is less frequent in patients without a clear background of lung diseases. DPO is characterized by very small calcific nodules, often with bone mature located in both lungs and often in peripheral areas of the lungs. Two patterns of DPO have been recognized dendriform and nodular. The dendriform type is less common and is characterized by a coral-like network of bone spiculae along the alveolar septa and is often related to interstitial fibrosis or chronic obstructive lung disease [1]. Recent literature papers indicate that DPO may be a predictor of pulmonary fibrosis, is related to Usual Interstitial Pneumonia (UIP) pattern, and has a higher correlation with Idiopathic Pulmonary Fibrosis (IPF). We present a case of a 41-years-old male with persistent bronchitis who underwent a chest X-ray (CXR) that showed multiple pulmonary small calcified nodules in both lungs. These findings were then defined with a high-resolution computed tomography of the chest (HRCT) that showed multiple small nodules spread in both lungs with a "tree-like pattern". A lung biopsy was performed to confirm the radiological diagnostic hypothesis of DPO, and further pathological examination showed multifocal areas of mature bone tissue within the lung parenchyma.

Telomere length across different UIP fibrotic-Interstitial Lung Diseases: a prospective Greek case-control study

IntroductionShort telomeres are recognized as risk factor for idiopathic pulmonary fibrosis (IPF). We aimed to assess the role of telomere length (TL) in fibrotic-Interstitial Lung Diseases (f-ILDs) associated with a usual interstitial pneumonia (UIP) pattern as well as in IPF acute exacerbation (IPF-AE). Aim and methodsTL was measured from peripheral white blood cells using a multiplex quantitative polymerase chain reaction in consecutive patients with f-ILDs, all presenting UIP pattern in the high-resolution chest-computed-tomography and compared to age-matched healthy controls. ResultsSeventy-nine individuals were included (mean age 69.77 ± 0.72 years); 24 stable IPF, 18 IPF-AE, 10 combined pulmonary fibrosis and emphysema, 7 Rheumatoid arthritis-UIP-ILDs and 20 controls. TL in all patients was significantly shorter compared to controls [mean T/S ratio (SE) 0.77 (±0.05) vs 2.26 (±0.36), p < 0.001] as well as separately in each one of f-ILD subgroups. IPF-AE patients presented significantly shorter TL compared to stable IPF (p = 0.029). Patients with IPF and shorter than the median TL (0−0.72) showed reduced overall survival (p = 0.004). T/S < 0.72 was associated with increased risk for IPF-AE (OR = 30.787, 95% CI: 2.153, 440.183, p = 0.012) independent of age, gender, smoking and lung function impairment. A protective effect of TL was observed, as it was inversely associated with risk of death both in UIP-f-ILDs (HR = 0.174, 95%CI: 0.036, 0.846, p = 0.030) and IPF patients (HR = 0.096, 95%CI: 0.011, 0.849, p = 0.035). ConclusionsShorter TL characterizes different UIP f-ILDs. Although no difference was observed in TL among diverse UIP subgroups, IPF-AE presented shorter TL compared to stable IPF. Reduced overall survival and higher hazard ratio of death are associated with shorter TL in IPF.

RETRACTED: Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): Evolving concept of CT findings, pathology and management

The connective tissue diseases (CTDs) demonstrating features of interstitial lung disease (ILD) include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), progressive systemic sclerosis (PSS), dermatomyositis (DM) and polymyositis (PM), ankylosing spondylitis (AS), Sjogren’s syndrome (SS), and mixed connective tissue disease (MCTD). In RA patients in particular, interstitial lung abnormality (ILA) (of varying degrees; severe vs. mild) is reported to occur in approximately 20–60 % of individuals and CT disease progression occurs in approximately 35–45 % of them. The ILAs have been associated with a spectrum of functional and physiologic decrement. The identification of progressive ILA may enable appropriate surveillance and the commencement of treatment with the goal of improving morbidity and mortality rates of established RA-ILD. Subpleural distribution and higher baseline ILA/ILD extent were risk factors associated with disease progression. At histopathologic analysis, connective tissue disease-related interstitial lung diseases (CTD-ILDs) are diverse and include nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), apical fibrosis, diffuse alveolar damage (DAD), and lymphoid interstitial pneumonia (LIP). Even though proportions of ILDs vary, NSIP pattern accounts for a large proportion, especially in PSS, DM/PM and MCTD, followed by UIP pattern. Evidence has been published that treatment of subclinical CT lung abnormalities showing a tendency to progress to ILD may stabilize the CT alterations. The identification of subclinical lung abnormalities can be appropriate in the management of the disease and CT appears to be the gold standard for the evaluation of lung parenchyma.

Histologic features suggesting connective tissue disease in idiopathic pulmonary fibrosis

Some patients with idiopathic pulmonary fibrosis (IPF) have histopathologic features suggesting connective tissue disease (CTD); however, their clinical course and prognosis remain unclear. Thus, we aimed to investigate the clinical course and prognosis of these patients with histologic autoimmune features. Among 114 patients with biopsy-proven IPF, the histologic features were semi-quantitatively graded, and CTD scores (range: 0–9) were calculated as the sum of each score of plasma cell infiltration, lymphoid aggregates, and germinal centres. Patients with high CTD scores (≥ 4) were classified into the interstitial pneumonia with histologic autoimmune features (IP-hAF) group. The mean age of the patients was 60.0 years; 74.6% were men, 69.3% were ever-smokers, and 35.1% had IP-hAF. During follow-up, the IP-hAF group showed slower decline in lung function, and better prognosis (median survival, 48.7 vs. 40.4 months; p = 0.015) than the no-IP-hAF group. On multivariate Cox analysis, IP-hAF was an independent prognostic factor (hazard ratio, 0.522; p = 0.016), along with the lower diffusing capacity for carbon monoxide, higher scores of reticulation and honeycombing, and usual interstitial pneumonia pattern on high-resolution computed tomography. Patients with IPF having histologic autoimmune features show distinct clinical characteristics and better outcome than those without histologic autoimmune features.

Acute exacerbation of rheumatoid arthritis-associated interstitial lung disease: clinical features and prognosis.

RA-associated interstitial lung disease (RA-ILD) is commonly associated with acute exacerbations (ILD-AE). This study examined the clinical characteristics and risk factors of ILD-AE and mortality of RA-ILD. We retrospectively collected data on 165 RA-ILD patients who visited or were admitted to our hospital between January 2007 and December 2019. We compared the clinical characteristics of patients who did and did not develop ILD-AE and identified variables significantly associated with ILD-AE. We also compared the admission characteristics of those who survived and those who died after admission for ILD-AE. ILD-AE was defined using previously proposed criteria, modified slightly for application to RA-ILD. The mean patient age was 73.6 years (s.d. 9.7) and 97 (71.9%) patients were female. Thirty (22.2%) patients developed ILD-AE, 13 (43.3%) of whom died. In univariate analyses, neither the usual interstitial pneumonia (UIP) pattern nor MTX was associated with ILD-AE. In multivariate analyses, the UIP pattern was significantly associated with ILD-AE [odds ratio (OR) 2.55 (95% CI 1.05, 6.20), P = 0.038]. In the Cox proportional hazards model, the UIP pattern [hazard ratio (HR) 4.67 (95% CI 1.02, 21.45), P = 0.048] was significantly associated with death, while MTX use [HR 0.16 (95% CI 0.04, 0.72), P = 0.016] was significantly associated with survival. Our data suggest that the UIP pattern is related to ILD-AE. Furthermore, both the UIP pattern and non-use of MTX might be related to death from ILD-AE in RA-ILD.

Serial changes of CT findings in patients with chronic hypersensitivity pneumonitis: imaging trajectories and predictors of fibrotic progression and acute exacerbation.

To evaluate the longitudinal changes of chest CT findings in patients with chronic hypersensitivity pneumonitis (HP) and identify risk factors for fibrotic progression and acute exacerbation (AE). This retrospective study included patients with chronic HP with follow-up CT. Baseline and serial follow-up CT were evaluated semi-quantitatively. Fibrosis score was defined as the sum of the area with reticulation and honeycombing. The modified CT pattern of Fleischner Society idiopathic pulmonary fibrosis diagnostic guidelines was evaluated. Cox proportional hazards regression was performed to determine significant variables associated with fibrotic progression and AEs. Of 91 patients, mean age was 59.1 years and 61.5% were women. The median follow-up period was 4.9 years. Seventy-nine patients (86.8%) showed fibrotic progression with persistent areas of mosaic attenuation, finally replaced by fibrosis, and 20 (22.0%) developed AE. Baseline fibrosis score and CT pattern of usual interstitial pneumonia (UIP)/probable UIP were independent risk factors for predicting fibrotic progression (hazard ratio [HR] = 1.05, 95% confidence interval [CI] = 1.02-1.09, p < 0.001, for fibrosis score; HR = 2.50, CI = 1.50-4.16, p < 0.001, for CT pattern) and AEs (HR = 1.07, CI = 1.01-1.13, p = 0.019, for fibrosis score; HR = 5.47, CI = 1.23-24.45, p = 0.026, for CT pattern) after adjusting clinical covariables. Fibrotic progression and AE were identified in 86.8% and 22.0% of patients with chronic HP. Fibrosis score and CT pattern of UIP/probable UIP on baseline chest CT may predict fibrotic progression and AE. • Most patients (87%) showed fibrotic progression on long-term follow-up with persistent areas of mosaic attenuation that were finally replaced by fibrosis at a later stage. • One-fifth of patients (22%) experienced acute exacerbation associated with worse prognosis. • Fibrosis score (sum of reticulation and honeycombing) and CT pattern of UIP/probable UIP on baseline CT were independent predictors for predicting fibrotic progression and acute exacerbation.

Chest CT Imaging Features of COVID-19-Related Pulmonary Fibrosis: A Case Report

: Shortly after being identified in China, 2019 coronavirus disease (COVID-19) caused by SARS-CoV-2 has spread rapidly and millions of people were infected as of May 2020; the outbreak resulting in more than 250,000 deaths was declared a pandemic. In this critical stage of the ongoing pandemic of COVID-19, information regarding the long term effects of the disease remains limited. While most of the patients recover completely, some patients may develop complications. In this report, we aimed to present the serial chest high-resolution computed tomography (HRCT) findings in a patient who developed pulmonary fibrosis (PF) similar to usual interstitial pneumonia (UIP) pattern during recovery from severe novel coronavirus pneumonia.

Risk factors for progression and prognosis of primary Sjögren's syndrome-associated interstitial lung disease in a Chinese population.

This study explored differences between primary Sjögren's syndrome-associated interstitial lung disease (pSS-ILD) patients with and without ILD progression, and analyzed the factors affecting the progression and prognosis of pSS-ILD. This study is a retrospective cohort study which enrolled 113 pSS-ILD patients hospitalized between 2011 and 2017. The 3-year survival rate of the pSS-ILD patients was 91.15%, and the 5-year survival rate was 84.07%. Univariate analysis showed that Raynaud's syndrome, hypoproteinemia, extensive lung involvement, possible usual interstitial pneumonia pattern were risk factors for the progression of ILD in patients with pSS-ILD, and cyclophosphamide was a protective factor for the progression of ILD in patients with pSS-ILD. Multiple logistic regression analysis showed that extensive lung involvement (odds ratio 4.143, 95% CI: 1.203-14.267, P<.05) was an independent risk factor for the progression of pSS-ILD. Cox hazard analysis showed that pSS-ILD with hypoproteinemia (hazard ratio [HR] 17.758, 95% CI: 4.753-66.340, P<-.05) and extensive lung involvement (HR 3.450, 95% CI: 1.419-8.390, P<.05) were associated with worse survival of patients. Extensive lung involvement is an independent risk factor for the progression of ILD in patients with pSS-ILD. Hypoproteinemia and extensive lung involvement are independent risk factors for mortality in patients with pSS-ILD, after controlling for potentially influential variables.

Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia.

BackgroundThere are no established therapeutic options available for idiopathic pleuroparenchymal fibroelastosis (IPPFE) apart from supportive care and lung transplantation. Furthermore, it is known that IPPFE with a usual interstitial pneumonia (UIP) pattern and lower lobe predominance is a disease entity distinct from idiopathic pulmonary fibrosis (IPF). To our knowledge, few studies are available that report on the efficacy of antifibrotic agents for IPPFE with UIP.AimThe aim of this study was to compare the efficacy of antifibrotic agents between IPPFE with UIP and typical IPF in real-world clinical practice.Patients and methodsA retrospective analysis was performed on the medical records of all patients at two interstitial lung disease centres. Sixty-four patients were diagnosed as having IPPFE with UIP and 195 patients were diagnosed with typical IPF. We compared the efficacy of antifibrotic agents between these two groups.ResultsSurvival time was significantly shorter in the patients with IPPFE with UIP. Some 125 patients were administered antifibrotic agents for over 6 months (34 with IPPFE with UIP and 91 with typical IPF). Reduced forced vital capacity (FVC) 6 months after treatment with antifibrotic agents was significantly greater in the IPPFE with UIP group than in those in the typical IPF group. Moreover, the change in % predicted FVC was significantly greater during the follow-up in patients with IPPFE with UIP compared with those with typical IPF.ConclusionsThe efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease.

Clinical diagnosis of patients subjected to surgical lung biopsy with a probable usual interstitial pneumonia pattern on high-resolution computed tomography

BackgroundUsual interstitial pneumonia can present with a probable pattern on high-resolution computed tomography (HRCT), but the probability of identifying usual interstitial pneumonia by surgical lung biopsy in such cases remains controversial. We aimed to determine the final clinical diagnosis in patients with a probable usual interstitial pneumonia pattern on HRCT who were subjected to surgical lung biopsy.MethodsHRCT images were assessed and categorized by three radiologists, and tissue slides were evaluated by two pathologists, all of whom were blinded to the clinical findings. The final clinical diagnosis was accomplished via a multidisciplinary discussion. Patients with a single layer of honeycombing located outside of the lower lobes on HRCT were not excluded.ResultsA total of 50 patients were evaluated. The most common final clinical diagnosis was fibrotic hypersensitivity pneumonitis (38.0%) followed by idiopathic pulmonary fibrosis (24.0%), interstitial lung disease ascribed to gastroesophageal reflux disease (12.0%) and familial interstitial lung disease (10.0%). In the group without environmental exposure (n = 22), 10 patients had a final clinical diagnosis of idiopathic pulmonary fibrosis (45.5%). Irrespective of the final clinical diagnosis, by multivariate Cox analysis, patients with honeycombing, dyspnoea and fibroblastic foci on surgical lung biopsy had a high risk of death.ConclusionsThe most common disease associated with a probable usual interstitial pneumonia pattern on HRCT is fibrotic hypersensitivity pneumonitis followed by idiopathic pulmonary fibrosis and interstitial lung disease ascribed to gastroesophageal reflux disease. In patients without environmental exposure, the frequencies of usual interstitial pneumonia and a final clinical diagnosis of idiopathic pulmonary fibrosis are not sufficiently high to obviate the indications for surgical lung biopsy.

Computed tomography patterns predict clinical course of idiopathic pulmonary fibrosis

BackgroundA new clinical guideline for idiopathic pulmonary fibrosis (IPF) uses high-resolution computed tomography (HRCT) patterns for diagnostic purposes. However, it is unknown how they relate to the IPF clinical course. We aimed to investigate whether HRCT patterns could be used to predict lung function changes and survival in patients with IPF.MethodsClinical data were retrospectively reviewed in 337 patients with IPF (all biopsy-proven cases). HRCT patterns were classified according to the 2018 IPF diagnostic criteria.ResultsThe median follow-up was 46.9 months. The mean age was 62.5 years, and 74.2% were men. Among the HRCT patterns, usual interstitial pneumonia (UIP), probable UIP, indeterminate for UIP, and an alternative diagnosis were identified in 163 (48.4%), 110 (32.6%), 33 (9.8%), and 31 (9.2%) patients, respectively. The indeterminate for UIP group showed higher lung function and exercise capacity and better prognosis than the other groups. They also had a lesser decline in lung function than the other groups during follow-up. In the multivariate Cox analysis, which was adjusted by age, smoking status, lung function, exercise capacity, and use of antifibrotic agents, indeterminate for UIP pattern was found to be an independent prognostic factor (hazard ratio 0.559, 95% confidence interval 0.335–0.933, P = 0.026). However, the probable UIP group had similar lung function changes and prognosis when compared the UIP group.ConclusionsOur results suggest that indeterminate for UIP pattern on HRCT may predict a more favorable clinical course in patients with IPF, supporting the validity of the new IPF diagnostic guidelines.

Radiological diagnosis of fibrosing interstitial lung diseases: innovations and controversies.

Following the introduction of new effective antifibrotic drugs, interest in fibrosing interstitial lung diseases (FILD) has been renewed. In this context, radiological evaluation of FILD plays a cardinal role. Radiological diagnosis is possible in about 50% of the cases, which allows the initiation of effective therapy, thereby avoiding invasive procedures such as surgical lung biopsy. Usual interstitial pneumonia (UIP) pattern may be diagnosed based on clinical, radiological, and pathological data. High-resolution computed tomography features of UIP have been widely described in literature; however, interpreting them remains challenging, even with specific expertise on the subject. Diagnostic difficulties are understandable given the continuous evolution of FILD classifications and their complexity. Both early-stage diseases and advanced or combined patterns are not easily classifiable, and many end up being labelled 'indeterminate´ or 'unclassifiable´. Especially in these cases, optimal patient management involves collaboration and communication between different specialists. Here, we discuss the most critical aspects of radiological interpretation in FILD diagnosis based on the most recent classifications. We believe that the clinicians´ awareness of radiological diagnostic issues of FILD would improve comprehension and dialogue between physicians and radiologists, leading to better clinical practice.

Risk of Radiation Pneumonitis in Locally Advanced Lung Cancer Patients: Role of Interstitial Lung Disease Subtypes and Severity of Pulmonary Emphysema

Interstitial lung disease (ILD) is a known risk factor for radiation pneumonitis (RP). In this study, the Fleischner Society new diagnostic criteria for idiopathic pulmonary fibrosis (IPF), a subtype of ILD, are applied to pretreatment diagnostic CT scans of lung cancer patients to assess their predictive value for grade 3+ RP. In addition, the severity of pulmonary emphysema (PE) is radiographically graded and correlated to RP as well. The pretreatment diagnostic CT chest scans of 121 locally advanced lung cancer patients with definitive chemoradiotherapy between 2009 and 2018 were retrospectively analyzed by a thoracic radiologist. CTs were classified into typical usual interstitial pneumonia (UIP), probable UIP, indeterminate for UIP, and non-IPF. Typical and probable UIP patterns support the diagnosis of IPF in a typical clinical context. In addition, the location (upper lobe, lower lobe, or both), severity (no PE (score 0), ⩽25% of lung (score 1), 26–75% (score 2), ⩾75% (score 3)), and morphology (predominantly centrilobular vs. predominantly paraseptal) of PE were recorded. RP was recorded as RTOG grade 3+ pneumonitis requiring steroid treatment. Univariate Cox (UVA) and logistic regression were applied to predict for an effect of ILD and PE on overall survival (OS) and RP, respectively. Median dose prescribed was 60Gy (33-78Gy), median lung was V20Gy of 27.7% (20.1-30.0). Twenty patients (16%) experienced RP≥G3 and 17 (13.6%) had radiographic evidence of ILD. Most (13/17, 76%) were classified as non-IPF. The four patients with UIP diagnoses were classified as indeterminate for UIP (4/17, 24%). On logistic regression, ILD was associated with RP (p = 0.08). No significant difference in RP between UIP and non-IPF diagnoses was seen (p = 0.36). On univariate analysis, the presence of ILD or subtype of ILD (UIP vs. non-IPF) did not predict for OS (p = 0.19 and 0.85 respectively). 113 patients (93.4%) had radiographic PE, 96 (84.2%) of which had upper lobe emphysema and 110 (89.4%) of which had PE in a predominantly centrilobular pattern. Eight patients (6.4%) were assigned score 0, 52 (41.6%) score 1, 42 (33.6%) score 2, 19 (15.2%) score 3. The diagnosis, location, morphologic subtype, and severity of PE did not predict for RP≥G3 (p = 0.75, 0.29, 0.93, and 0.36, respectively) and were not significantly related to OS (p = 0.5, 0.85, 0.97, and 0.7, respectively). As a control, known dosimetric predictors of RP were significantly correlated (lung V5Gy/V10Gy/V20Gy; p = 0.005, 0.003, and 0.014, respectively). In the current study, there were no significant differences between UIP and non-IPF diagnoses in regard to clinical RP risk, potentially because none of our patients had UIP features more strongly associated with IPF. There was also a trend towards presence of radiographic ILD and development of RP. In addition, our data indicate no increased pneumonitis risk for patients with emphysema regardless of location or severity.

Clinical significance of minor salivary gland biopsy in patients with idiopathic interstitial pneumonia

BackgroundSignificant overlap may occur between idiopathic interstitial pneumonia (IIP) and connective tissue diseases (CTDs) that do not meet the established classification criteria for any known CTDs (i.e., occult CTD). Performing minor salivary gland biopsy (MSGB) to detect occult primary Sjogren's syndrome (pSS) in IIP patients is not well studied. MethodsConsecutive IIP patients underwent MSGB to determine the prevalence of positive MSGB findings. Furthermore, we characterised the clinical, physiological and serological profiles of the MSGB-positive patients. Cox regression models were used to identify independent predictors of survival. ResultsThe data of 155 patients with IIP were available for analysis. Sixty patients (38.7%) had positive MSGB findings. Of them, the mean age was 63.3 years, 51.6% were women, usual interstitial pneumonia (UIP) was the predominant pattern (63.3%), and seronegative antibodies (61.6%) were likely. Patients with positive MSGB findings had significantly greater survival than those with negative MSGB findings (p = 0.041). After stratifying the MSGB cohort based on the presence of a UIP pattern, no significant difference in survival was noted between those with positive MSGB-UIP pattern and those with a negative MSGB-UIP pattern (p = 0.231). Multivariate analysis on all UIP patients showed that higher forced vital capacity (p = 0.010) and smoking status (p = 0.035) were independently associated with survival. ConclusionsA substantial number of IIP patients had underlying occult CTD, highlighting the importance of performing MSGB to identify the salivary component of pSS when evaluating patients with interstitial lung disease of undetermined aetiology.

Idiopathic pulmonary fibrosis in the UK: analysis of the British Thoracic Society electronic registry between 2013 and 2019.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and terminal interstitial lung disease (ILD) with a median survival of 3–5 years. The British Thoracic Society (BTS) established the UK IPF Registry in 2013 as a platform to collect data on clinical characteristics, treatments and outcomes for this cohort in the UK.Between 1 January 2013 and 31 October 2019, 2474 cases were registered. Most patients were male (79%) with a mean±sd age of 74±8.3 years and 66% were ex-smokers. Over time we observed an increase in the number of patients aged over 70 years. However, we have not seen a trend towards earlier presentation as symptoms of breathless and/or cough were present for >12 months in 63% of the cohort. At presentation, mean±sd % predicted forced vital capacity (FVC) was 78.2±18.3%, median 76.2% (interquartile range (IQR) 22.4%) and transfer factor of the lung for carbon monoxide (TLCO) 48.4±16.0, median 47.5 (IQR 20.1). Most cases were discussed at an ILD multidisciplinary meeting, with an increase over this time in the number of cases reported as having possible usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) thorax. We noted a reduction in the number of patients undergoing surgical lung biopsy or bronchoalveolar lavage. Although more patients were prescribed anti-fibrotic therapies from 2013 to 2019, 43% were ineligible for treatment based upon National Institute for Health and Care Excellence (NICE) prescribing criteria. Hypertension, ischaemic heart disease, diabetes mellitus and gastro-oesophageal reflux were the most common comorbidities.In conclusion, we have presented baseline demographics as well as diagnostic and treatment strategies from the largest single-country IPF registry, reflecting changes in UK practices over this period.

COMBING IMAGING TO DIFFERENTIATE CONNECTIVE TISSUE DISEASE-ASSOCIATED INTERSTITIAL LUNG DISEASE FROM IDIOPATHIC PULMONARY FIBROSIS

SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 Introduction: Parenchymal lung involvement in Sjogren's syndrome has generally been associated with a nonspecific interstitial pneumonia pattern (NSIP)1,2. We present a patient with chest CT findings compatible with usual interstitial pneumonia (UIP) pattern including exuberant honeycombing, straight edge sign, and the upper lobe sign. After thorough evaluation, the patient was diagnosed with Sjogren's syndrome with exuberant honeycombing, a rare finding in this specific connective tissue disease. Case Presentation: A 36-year-old non-Spanish speaking Guatemalan female presented with progressive chest pain, subacute on chronic dyspnea, and a dry cough. On admission, she had hypoxemic respiratory failure which responded to supplemental oxygen. CT angiogram was negative for pulmonary emboli but showed extensive bibasilar “exuberant” honeycombing. She immigrated to the US 2 years before and had no significant past medical conditions or medication use - prescription or herbal. Exposure history was notable for exposure to indoor smoke from firewood burning stove 10 years ago and she is currently employed on a food production line. Otherwise, infectious and social histories were negative. On examination, she had poor dentition with several capped teeth, cracked lips, and dry Velcro crackles on lung auscultation. Autoimmune serology was significant for positive SSA/SSB and ANA. Her QuantiFERON gold test was likewise positive. Echocardiogram and right heart catheterization showed severe pulmonary hypertension with normal PCWP with a positive vasodilator response. In the context of the UIP pattern on CT chest, examination, and autoimmune serology, she was diagnosed with Sjogren's related interstitial lung disease (ILD). She gradually improved in the hospital with conservative management including supplemental oxygen. A twelve-week course of isoniazid and rifapentine was initiated for LTBI. Sjogren's related ILD therapy is planned after completion of LTBI treatment. She is undergoing evaluation in the Pulmonary Hypertension Clinic and the Transplant Clinic. Discussion: Although mild basilar and reticular honeycombing is a well-described pattern in Sjogren's related ILD, extensive honeycombing is extremely rare1,2. Connective Tissue Disease-related-UIP (CTD-UIP) is differentiated from Idiopathic Pulmonary Fibrosis-associated-UIP (IPF-UIP) in this patient by the presence of the anterior upper lobe sign, straight-edge sign, and exuberant honeycombing sign. (See figure 1) Individually, these signs have low sensitivity (<25%) albeit high specificity (87-94%) for CTD-IPF3. When present together, the sensitivity increases to 95.5% with a likelihood ratio LR(+) of 5.28 for CTD-UIP3. Conclusions: We report an unusual case with extensive “exuberant” honeycombing due to Sjogren's disease. When present, these radiographic signs are valuable aids in differentiating CTD-related-UIP from IPF UIP. Reference #1: Kim, E. J., Collard, H. R. & King, T. E. Rheumatoid arthritis-associated interstitial lung disease: the relevance of histopathologic and radiographic pattern. Chest 136, 1397 -1405 (2009). Reference #2: Mueller-Mang, C., Grosse, C., Schmid, K., Stiebellehner, L. & Bankier, A. A. What Every Radiologist Should Know about Idiopathic Interstitial Pneumonias. RadioGraphics 27, 595 -615 (2007) Reference #3: Chung, J. H. et al. CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease -Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. Am. J. Roentgenol. 210, 307 -313 (2017) Disclosures: No relevant relationships by Timothy Dempsey, source=Web Response No relevant relationships by Samuel Falde, source=Web Response No relevant relationships by Kaiser Lim, source=Web Response