Features Of Interstitial Lung Disease Research Articles

Radiological features of interstitial lung disease associated with collagen vascular disease in Fayoum University Hospital

Objective Collagen vascular diseases (CVD) are a varied group of immune-mediated disorders that affect multiple organs, such as affection of interstitium in the form of interstitial lung disease (ILD). High-resolution computed tomography is the cornerstone for appropriate diagnosis of ILD. Aim This study aimed to study the radiological features of ILD associated with CVD in Fayoum University Hospital. Design This was a cross-sectional study. Setting The study was conducted at Fayoum University Hospital in Egypt during January 2018 to June 2018. Patients and methods This study included 100 patients with diagnosis of CVD who were referred from Rheumatological Department of Fayoum University Hospital complaining of chest symptoms. For the diagnosis of these cases, we needed a full medical history, a detailed clinical examination, spirometry, 6-min walk test, and high-resolution computed tomography. Results The most important radiologic pattern for ILD-CVD was the nonspecific interstitial pneumonia pattern, representing 50% of cases, followed by the usual interstitial pneumonia pattern, representing 13% of cases. Conclusion Nonspecific interstitial pneumonia is the most common radiological pattern among patients of CVD in Fayoum University Hospital.

Transthoracic ultrasonography in patients with interstitial lung disease.

Background:Transthoracic ultrasonography (TUS) is suggested as a noninvasive, radiation-free method for the assessment of interstitial lung disease (ILD). This study was designed to study TUS features of ILD. Furthermore, possible correlations of these features with parameters of spirometry, arterial blood gas (ABG) analysis and 6-min walk test (6MWT) were assessed.Materials and Methods:Fifty patients with ILD were diagnosed based on history, examination, chest X-ray/high-resolution computed tomography, and spirometry. Each patient underwent 6MWT, ABG analysis, and TUS. TUS was also performed on 20 healthy volunteering controls.Results:The TUS features among patients were B pattern in 40 patients (80.0%, P < 0.001), decreased lung sliding in 22 patients (44.0%, P < 0.001), pleural line thickening in 28 patients (56.0%, P < 0.001), pleural line irregularity in 39 patients (78.0%, P < 0.001) and subpleural changes in 22 patients (44.0%, P < 0.01). Increasing pleural line thickness was inversely correlated with forced vital capacity (FVC) percent predicted (r = −0.345, P < 0.05), pO2 (r = −0.335, P < 0.01), SpO2 at rest (r = −0.444, P < 0.01), 6-min walk distance (6MWD) (r = −0.554, P < 0.001) and distance-saturation product (DSP) (r = −0.572, P < 0.001). Increasing distance between B lines also correlated inversely with FVC percent predicted (r = −0.278), pO2 (r = −0.207), SpO2 at rest (r = −0.170), 6MWD (r = −0.209), and DSP (r = −0.214); however these correlations were not statistically significant (P > 0.05).Conclusion:TUS seems to be a useful imaging method for the diagnosis of ILD. It can be used to estimate the severity of ILD. It is simple, bedside, cost-effective, and radiation-free. It may be especially useful in the follow up of patients in low resource settings, pregnant females, and bed-ridden or unstable patients who cannot be shifted to radiology suite.

Diagnostic test interpretation and referral delay in patients with interstitial lung disease

BackgroundDiagnostic delays are common in patients with interstitial lung disease (ILD). A substantial percentage of patients experience a diagnostic delay in the primary care setting, but the factors underpinning this observation remain unclear. In this multi-center investigation, we assessed ILD reporting on diagnostic test interpretation and its association with subsequent pulmonology referral by a primary care physician (PCP).MethodsA retrospective cohort analysis of patients referred to the ILD programs at UC-Davis and University of Chicago by a PCP within each institution was performed. Computed tomography (CT) of the chest and abdomen and pulmonary function test (PFT) were reviewed to identify the date ILD features were first present and determine the time from diagnostic test to pulmonology referral. The association between ILD reporting on diagnostic test interpretation and pulmonology referral was assessed, as was the association between years of diagnostic delay and changes in fibrotic features on longitudinal chest CT.ResultsOne hundred and forty-six patients were included in the final analysis. Prior to pulmonology referral, 66% (n = 97) of patients underwent chest CT, 15% (n = 21) underwent PFT and 15% (n = 21) underwent abdominal CT. ILD features were reported on 84, 62 and 33% of chest CT, PFT and abdominal CT interpretations, respectively. ILD reporting was associated with shorter time to pulmonology referral when undergoing chest CT (1.3 vs 15.1 months, respectively; p = 0.02), but not PFT or abdominal CT. ILD reporting was associated with increased likelihood of pulmonology referral within 6 months of diagnostic test when undergoing chest CT (rate ratio 2.17, 95% CI 1.03–4.56; p = 0.04), but not PFT or abdominal CT. Each year of diagnostic delay was associated with a 1.8% increase in percent fibrosis on chest CT. Patients with documented dyspnea had shorter time to chest CT acquisition and pulmonology referral than patients with documented cough and lung crackles.ConclusionsDeterminants of ILD diagnostic delays in the primary care setting include underreporting of ILD features on diagnostic testing and prolonged time to pulmonology referral even when ILD is reported. Interventions to modulate these factors may reduce ILD diagnostic delays in the primary care setting.

IBS14.02 Best Management of Early Stage NSCLC in ILD Patients

IBS14.02 Best Management of Early Stage NSCLC in ILD Patients

4. A case of SAE-1 dermatomyositis

IntroductionDermatomyositis is a rare, acquired inflammatory myopathy. The panel of myositis specific antibodies (MSAs) continues to grow and inclusivity of MSAs in myositis autoantibody testing can vary among trusts. Our patient had serology and clinical features in keeping inflammatory myopathy associated with one of the more recently found MSAs- SAE-1.Case descriptionA 63-year-old man developed a purple rash over his scalp in July 2018. He was previously fit and well apart from childhood rheumatic fever. He was a non-smoker. The rash spread over his face, forearms and feet.He developed discomfort and weakness in his neck, upper arms and thighs in September 2018 with increasing difficulty walking up stairs and lifting boxes and driving. In October 2018 he developed progressive dysphagia and was struggling to swallow semi-solid food and then liquids too.A skin biopsy showed mild papillary dermal inflammatory infiltrate in keeping with possible dermatomyositis or systemic lupus. The patient was admitted to hospital in December 2018 with progression of the rash, proximal weakness and swallowing difficulty and referred to rheumatology.He lost over 7kgs in weight between October and December 2018. He denied any fever or symptoms suggestive of a connective tissue disease. On examination he had a purple rash on his scalp, over the malar distribution and upper back. He had a heliotrope rash over his eyelids and gottrens papules. He had 4/5 weakness in hip and shoulder flexors. He also had bibasal crepitations from his lungs on auscultation.Investigations revealed a serial normal creatine kinase (CK) and transaminases. A myositis panel revealed the SAE-1 autoantibody. CT thorax, abdomen and pelvis revealed some inflammatory changes at the bases of both lungs in keeping with organising pneumonia. Upper and lower GI endoscopy was unremarkable. An MRI revealed inflammation of multiple muscles including gluteus maximus and minimus, iliopsoas, paraspinal muscles and posterior abdominal wall in keeping with polymyositis.He was commenced on prednsiolone 40mg for 3 months after a pulse intravenous methylprednisolone. He noticed great improvement with proximal myopathy, dysphagia and rash within a week of commencing treatment. He was started on azathioprine at 2mg/kg in December 2018.DiscussionThe SAE-1 autoantibody targets the auto-antigen SUMO-1 facilitating protein sumoylation. Previous cohort studies have shown skin manifestations and dysphagia as predominant clinical characteristics in SAE-1 dermatomyositis. Our patient had features of interstitial lung disease which has previously been reported as rare in such patients. Our patient also had a normal serial CK and transaminases despite having proximal clinical weakness and on activity on radiological modality.Our patient had SAE-1 positive dermatomyositis which has responded quickly to corticosteroids. Azathioprine was chosen ahead of other immunosuppressive therapy after discussion with the dermatology team as cutaneous manifestations were predominant. The patient remains in remission on azathioprine.Key learning pointsSAE-1 dermatomyositis is very steroid responsive and remission can be maintained on azathioprine when there are widespread cutaneous manifestations, dysphagia and mild ILD.The case serves as an important reminder that CK levels can be normal in active myositis. A normal CK should not stop further investigations where clinical suspicion for inflammatory myopathy remains high.SAE-1 positive dermatomyositis is a heterogeneous inflammatory myopathy. Our patient had skin manifestations and dysphagia as reported in many previous case reports however they also had features in keeping with organising pneumonia which has been reported as a rarer finding in SAE-1 dermatomyositis.A multi-disciplinary approach to such patients is paramount. This case involved team work between rheumatology, dermatology, respiratory medicine and radiology.Conflicts of interestThe authors have declared no conflicts of interest.

Characterization of Interstitial Lung Disease Associated With Anti-Ribonucleoprotein Antibodies.

Interstitial lung disease (ILD) is a common feature of mixed connective tissue disease. However, many patients do not meet the criteria for mixed connective tissue disease and thus may be diagnosed as interstitial pneumonia with autoimmune features. The aim of this study was to characterize ILD associated with anti-ribonucleoprotein (RNP) antibodies. Chest computed tomography scans of patients with anti-RNP antibody who were seen between January 2011 and October 2015 were reviewed. The underlying disease was classified with international criteria using clinical and serological features. Among 544 patients with anti-RNP antibodies, 188 had a chest computed tomography scan, and 48 (26%) of them had radiological features of ILD. The presence of ILD was significantly associated with dyspnea, crackles, arthritis, Raynaud phenomenon, myositis, and sicca syndrome. The most frequent pattern was nonspecific interstitial pneumonia in 39 patients (81%). Among patients with ILD, 17 (35%) had a radiological pattern consisting of cysts and ground-glass attenuation not fulfilling the lymphoid interstitial pneumonia criteria. In 3 patients, cysts were related to fibrosis; in 14 patients, cysts corresponded to an original ILD pattern. Interstitial lung disease was found in 26% of patients with anti-RNP antibodies independently of the underlying disease. Anti-RNP-associated ILD mainly corresponds to nonspecific interstitial pneumonia or an original pattern consisting of cysts and ground-glass attenuation.

Interstitial Lung Disease in Anti-Synthetase Syndrome

Anti-synthetase syndrome is an autoimmune disorder characterized by the presence of autoantibodies against aminoacyl transfer RNA (tRNA) synthetases, and myositis, interstitial lung disease (ILD), arthritis, fever and Raynaud’s phenomenon (RP). We present a 54-year-old woman, who complained of fatigue, low-grade fever, myalgias, arthralgias, RP and dyspnoea on exertion. Chest CT scan revealed features of interstitial lung disease. Due to rapid deterioration of her lung function, she required oxygen support. The patient did not respond to empiric treatment with antibiotics. Autoantibody testing was remarkable for ANA positivity (1/160) and high-titre anti-Jo1 positivity. A diagnosis of anti-synthetase syndrome was made and the patient was placed on high-dose corticosteroids and rituximab with significant improvement. At 1-year follow up, she remains in good condition, without the need for oxygen supplementation.

Dysregulation of the Wnt signaling pathway in South African patients with diffuse systemic sclerosis.

The objectivewas to explore changes in gene expression in Wnt pathway genes in skin samples of black South Africans with diffuse cutaneous systemic sclerosis (dcSSc). Affected (forearm) and unaffected (upper back) skin samples of eight Black South Africans with active early dcSSc were compared to skin samples from seven ethnically matched control subjects. The Wnt Pathway Plus RT2 Profiler qPCR Array was used to determine gene expression and analyzed for differential expression between cases and controls. Selective validation was done using single-gene TaqMan assays. Several genes were similarly upregulated in both affected and unaffected skin of the dcSSc patients compared to controls. These included the Wnt ligands WNT7A and WNT10A, the frizzled receptors FZD8 and FZD9, intracellular signaling proteins AXIN1 and AXIN2, and the pathway target genes FGF4 and MMP7. Principal component analysis revealed patients clustering into two groups, which co-segregated with clinical features of interstitial lung disease and/or inflammatory myopathy, or the absence of an inflammation phenotype. These two groups showed paradoxical gene expression of the genes TCF7, SOX17, and FRZB in affected and unaffected skin. This study provides further evidence of dysregulation of gene expression at various levels of the Wnt signaling pathway in dcSSc. Moreover, principal component analysis showed two distinct patient clusters of gene expression, which co-segregated with the presence or absence of clinical inflammatory features, and may reflect different pathological pathways in dcSSc.

Clinical subsets of juvenile dermatomyositis classified by myositis-specific autoantibodies: Experience at a single center in Japan

Objectives: This study investigated the association between myositis-specific autoantibodies (MSAs) and clinical subsets of juvenile dermatomyositis (JDM) in Japanese patients.Methods: Twenty-one patients at a single center who developed initial or relapsed JDM from 2011 to 2016 were analyzed. Serum concentrations of MSAs against TIF1-γ, MDA5, NXP2, Mi-2, ARS, and SAE were measured by enzyme-linked immunosorbent assays. Clinical symptoms and laboratory data were obtained from clinical records. Clinical characteristics were compared in patients with autoantibodies against TIF1-γ, MDA5, and NXP2.Results: Of the 21 patients, 20 (95.2%) were positive for one or more MSAs, including nine (42.9%), five (23.8%), six (28.6%), and one (4.8%) positive for anti-TIF1-γ, anti-MDA5, anti-NXP2, and anti-Mi-2 autoantibodies. No patient was positive for anti-ARS or anti-SAE autoantibodies. The frequency of diffuse cutaneous lesions was higher in patients with anti-TIF1-γ autoantibodies. Anti-MDA5 autoantibody-positive patients had features of interstitial lung disease on chest computed tomography. Severe muscle damage at disease onset was significantly associated with positivity for anti-NXP2 autoantibodies.Conclusion: Similar to findings in Western countries, the clinical characteristics of JDM in Japanese may differ for each type of MSAs.

Clinical and Histopathologic Features of Interstitial Lung Disease in Erdheim⁻Chester Disease.

Limited information is available regarding interstitial lung disease (ILD) in Erdheim–Chester disease (ECD), a rare multisystemic non-Langerhans cell histiocytosis. Sixty-two biopsy-confirmed ECD patients were divided into those with no ILD (19.5%), minimal ILD (32%), mild ILD (29%), and moderate/severe ILD (19.5%), based on computed tomography (CT) findings. Dyspnea affected at least half of the patients with mild or moderate/severe ILD. Diffusion capacity was significantly reduced in ECD patients with minimal ILD. Disease severity was inversely correlated with pulmonary function measurements; no correlation with BRAF V600E mutation status was seen. Reticulations and ground-glass opacities were the predominant findings on CT images. Automated CT scores were significantly higher in patients with moderate/severe ILD, compared to those in other groups. Immunostaining of lung biopsies was consistent with ECD. Histopathology findings included subpleural and septal fibrosis, with areas of interspersed normal lung, diffuse interstitial fibrosis, histiocytes with foamy cytoplasm embedded in fibrosis, lymphoid aggregates, and focal type II alveolar cell hyperplasia. In conclusion, ILD of varying severity may affect a high proportion of ECD patients. Histopathology features of ILD in ECD can mimic interstitial fibrosis patterns observed in idiopathic ILD.

Diagnosis and treatment of interstitial lung disease in scleroderma systematica

Scleroderma systemitica (SDS) is an autoimmune connective tissue disease characterized by excessive fibrosis of the skin and viscera. SDS is prone to be chronic and progressive, is accompanied by the deterioration in quality of life and working ability and has an unfavorable prognosis. Interstitial lung disease (ILD) is one of the most common causes of death due to SDS. The lecture deals with the clinical and laboratory instrumental features of ILD in SDS. SDS-associated ILD occurs in 65–80% of patients and is highly diverse in the degree of severity and the tendency to progression. In the majority of patients, the fibrous process in the lung occurs in the early years of the disease, is limited and progresses slowly. Severe lung damage with rapid progression develops only in 10–15% of cases. Evaluation of lung damage in SDS includes pulmonary function tests, lung diffusing capacity determination, Doppler echocardiography analysis, multispiral computed tomography (MSCT) of the chest, bronchoalveolar lavage, lung biopsy with morphological examination of its specimens, as well as right heart catheterization. The best technique for detecting ILD is MSCT, as chest radiography has a low sensitivity in the early stages of the disease. IPL is treated in accordance with the 2017 ACR/EULAR guidelines; the leading role is played by immunosuppressive drugs, such as cyclophosphamide and mycophenolate mofetil. Antifibrotic drugs (pirfenidone, nintedanib) are being tested now .

Does the severity of interstitial lung disease affect the gains from pulmonary rehabilitation?

Reduced exercise capacity is a main feature of Interstitial Lung Diseases (ILDs) and it is related to closely prognosis of these patients. Pulmonary Rehabilitation (PR) results to improve in peak exercise capacity, dyspnea and quality of life in ILDs. We aimed to evaluate the benefits of PR in patients with ILDs and to determine whether there are similar gains in patients with severe ILD. We recruited ILD stable patients. All patients were evaluated with pulmonary function test, exercise capacity [6-minute walking test (6MWD)], quality of life [Short Form-36 (SF-36), St. George's Respiratory Questionnare (SGRQ), Hospital anxiety and Depression (HAD)] before and after PR (8 week). Fifty-seven patients who completed PR program, 30 women and 27 men, included in this study. It was determined a significant difference in terms of quality of life and exercise capacity (P < .05) before and after PR. When these patients were divided into 2 groups (DLCO <40% predicted, severe: group-1, and group-2: other ≥40], there was no difference between 2 groups except for forced expiratory volume in 1 second, pulse oxygen saturation and partial oxygen pressure (pO2 ). When compared the differences between pre and post values of all variables, there was no difference significantly except 4 variables (SF-36; physical functioning, social functioning, role physical and pO2 ). PR led to improvement in quality of life and exercise capacity in ILDs. In addition, PR, irrespective of the severity of the disease, is particularly beneficial in patients with severe ILDs.

Clinical features of interstitial lung diseases according to data from the Irkutsk Register

The aim of the study was to investigate clinical features of interstitial lung diseases (ILD) using data from the register of Irkutsk residents with ILD. Methods. Medical history, clinical and functional data and data of high-resolution computed tomography (HRCT) of the lungs of 110 patients (67 women, 43 men, mean age, 56.2 ± 15.9 years) were analyzed. Lung biopsy was performed in 49 patients (45%). Results. Bilateral slowly resolving and/or recurrent pneumonia, unexplained pulmonary dissemination and idiopathic pulmonary fibrosis were the most frequent referral diagnoses. After thorough examination, three groups of ILDs were diagnosed most often: ILD with a known cause (32%), idiopathic interstitial pneumonias (30%), and pulmonary sarcoidosis (29%). Cough and dyspnea were the leading symptoms at ILD onset (in 48% and 39% of patients, respectively). The average duration of respiratory symptoms was 29.5 ± 33.0 months. Asymptomatic radiological abnormalities were the only reason for diagnostic work-up in 13% of the patients. Conclusion. Diagnosis of ILD is quite challenging for primary care physicians and pulmonologists. Accuracy and timeliness of diagnosis of ILD requires multidisciplinary approach as far as depends on quality of diagnostic procedures including medical history, lung auscultation, lung function testing, HRCT, and morphological examination, but also on the physician’s awareness and clinical experience.

Impact of Pretreatment Interstitial Lung Disease on Radiation Pneumonitis and Survival in Patients Treated With Lung Stereotactic Body Radiation Therapy (SBRT)

IntroductionThe purpose of this study was to determine the impact of interstitial lung disease (ILD) on radiation pneumonitis (RP) and overall survival (OS) in lung stereotactic body radiation therapy (SBRT). MethodsPatients treated with lung SBRT from 2004 to 2015 were included. Pretreatment computed tomography scans were reviewed and classified for interstitial changes by thoracic radiologists using American Thoracic Society guidelines and Washko and Kazerooni scores. RP was scored prospectively using Common Terminology Criteria for Adverse Events, version 3.0. Pretreatment imaging characteristics, clinical variables, and dosimetry were assessed by univariate (UVA) and multivariate analysis (MVA). OS was assessed by the log-rank test, and the impact of ILD on OS was assessed by Cox regression. ResultsOf the 537 patients assessed, 39 had interstitial changes (13 usual interstitial pneumonia [UIP], 24 possible UIP, and 2 inconsistent with UIP). RP was significantly higher in patients with ILD than in patients without ILD (grade ≥ 2, 20.5% vs. 5.8%; P < .01; grade ≥ 3, 10.3% vs. 1.0%; P < .01). Two of 3 grade 5 RP had imaging features of ILD. On UVA, ILD, Washko score, lung parameters performance status, and dose were significant predictors of grade ≥ 2 RP. On MVA, ILD (odds ratio, 5.81; 95% confidence interval, 2.28-14.83; P < .01) and mean lung dose (odds ratio, 1.40; 95% confidence interval, 1.14-1.71; P < .01) were predictors of RP. ILD did not significantly affect OS on UVA or MVA. Median survival was 27.4 months in the ILD cohort and 34.8 in the ILD-negative cohort (P = .17). DiscussionILD is a significant risk factor for RP in patients treated with lung SBRT. Computed tomography scans should be reviewed for evidence of ILD prior to SBRT.

Assessment of transthoracic sonography in patients with interstitial lung diseases

This study was designed to recognize the sonographic features of interstitial lung diseases (ILD). Furthermore, the possible correlations of these features with the functional and radiological parameters of the disease were assessed. Forty-two patients with ILD were included; each patient underwent spirometry, Multi Detector CT chest (MDCT) and transthoracic sonography (TS). Fifteen healthy volunteers were also studied as controls. The sonographic features among ILD patients were B lines in 73.8% , abolished lung sliding in 23.8%, irregular and thickened pleura in 47.6%and 35.7% respectively and subpleural lesions in 38.1%.Increasing distance between the B lines was negatively correlated with both of Forced Vital Capacity % predicted , ground glass opacities and positively correlated with reticular opacities patterns on MDCT chest. TS can be used as an additional imaging method for assessment of ILD and as a marker to estimate the severity of disease.

Update on the pharmacological treatment of adult myositis.

The management of patients with idiopathic inflammatory myopathy (IIM) remains a challenge given the systemic features beyond active myositis. That is, recognizing the inflammatory arthropathy, varying dermatomyositis rashes, and overt and occult features of interstitial lung disease in addition to myositis adds to the complexity of diagnosis and treatment of IIM. However, clinicians now have available many more immunosuppressive drugs as well as biologic agents for use in patients with myositis and other autoimmune diseases. Here, the use of these agents is reviewed and support based on available published literature is provided even though many studies have been small and results somewhat anecdotal. Glucocorticoids remain the initial treatment of choice in most instances and methotrexate and azathioprine are often used early in the treatment course. These agents are followed by other immunosuppressive drugs, for example mycophenolate mofetil, tacrolimus, cyclosporine and cyclophosphamide, some of which are used alone while combinations of these agents also provide an effective option. There is more rationale for the use of biologic agents such as rituximab from a mechanistic perspective and, given the incorporation of validated core set measures in assessing myositis patients, we can look forward to better designed clinical trials in the future.

Late-onset noninfectious interstitial lung disease following autologous haematopoietic stem cell transplantation in paediatric patients.

Background and objectiveHigh‐dose chemotherapy (HDCT) followed by autologous haematopoietic stem cell transplantation (HSCT) is widely used in paediatric cancer patients, but few data about noninfectious interstitial lung disease (ILD) following this treatment are available. Therefore, we aimed to evaluate the incidence, clinical features and risk factors of noninfectious ILD after HDCT in paediatric patients.MethodsThis was a retrospective cohort study of paediatric solid tumour patients who underwent HDCT and autologous HSCT between 1997 and 2012. ILD was diagnosed using clinical symptoms and radiography after excluding cardiac, renal and infectious causes. Risk factors were analysed using a Cox proportional hazard regression model.ResultsThree hundred and forty patients were enrolled, and the median age was 3 years (interquartile range 1–7). Eight patients (2.4%) were diagnosed with noninfectious ILD. The median duration of symptom onset was 30 months (range 7–74). Six (75%) of eight ILD patients died during the study period, even though steroids were administered for treatment. High‐dose cyclophosphamide use (hazard ratio = 11.37, 95% confidence interval = 1.38–93.32, P = 0.023) and sex (hazard ratio = 0.10, 95% confidence interval = 0.01–0.84, P = 0.034) were associated with late‐onset, noninfectious ILD upon multivariate analysis.ConclusionThe incidence of noninfectious ILD after HDCT and autologous HSCT was not negligible, and the clinical features of ILD showed late onset and a poor prognosis. Female gender and high‐dose cyclophosphamide treatment may be risk factors for noninfectious ILD, but further studies with a larger number of ILD patients are suggested.

Respiratory and lower limb muscle function in interstitial lung disease.

Growing evidence suggests that respiratory and limb muscle function may be impaired in patients with interstitial lung disease (ILD). Importantly, muscle dysfunction could promote dyspnoea, fatigue and functional limitation all of which are cardinal features of ILD. This article examines the risk factors for skeletal muscle dysfunction in ILD, reviews the current evidence on overall respiratory and limb muscle function and focuses on the occurrence and implications of skeletal muscle dysfunction in ILD. Research limitations and pathways to address the current knowledge gaps are highlighted.

Antisynthetase syndrome: An under-recognized cause of interstitial lung disease.

Background:Antisynthetase syndrome (AS) is an uncommon and under-recognised connective tissue disease characterized by the presence of antibodies to anti-aminoacyl t-RNA synthetase along with features of interstitial lung disease (ILD), myositis and arthritis. The aim of the current study is to describe our experience with management of AS.Materials and Methods:This was a 2-year (2013-2014) retrospective analysis of patients diagnosed with anti-Jo-1-related AS. The presence of anti-Jo-1 antibody was tested by the immunoblot assay. All patients underwent high-resolution computed tomography of the chest, transthoracic echocardiography and evaluation for inflammatory myositis. Transbronchial lung biopsies and muscle biopsies were obtained when clinically indicated.Results:Nine patients (mean age: 43.8 years) were diagnosed with anti-Jo-1-related AS. The median duration of symptoms before diagnosis of AS was 6 months. All patients were negative for antinuclear antibodies by indirect immunofluorescence. The prevalence of ILD, myositis and arthritis at presentation was 100%, 77.8% and 55.6%, respectively. The most common ILD pattern was non-specific interstitial pneumonia (n = 6) followed by organizing pneumonia (n = 2) and usual interstitial pneumonia (n = 1). ILD was the sole manifestation in two patients and was subclinical in two patients. Six patients had pleuropericardial effusions, three patients had pulmonary artery hypertension and two patients had venous thromboembolism. Eight of the nine patients improved after treatment with steroids and other immunosuppressants.Conclusion:Antisynthetase syndrome is an important and a treatable cause of ILD. Strong clinical suspicion is needed to achieve an early diagnosis.

Effect of Iterative Reconstruction on the Detection of Systemic Sclerosis-related Interstitial Lung Disease: Clinical Experience in 55 Patients.

To evaluate the effect of iterative reconstruction on the depiction of systemic sclerosis-related interstitial lung disease (ILD) when the radiation dose is reduced by 60%. This study was based on retrospective interpretation of prospectively acquired data over a 12-month period and approved by the institutional review board. The requirement to obtain informed consent was waived. Fifty-five chest computed tomographic (CT) examinations were performed in 38 women and 17 men (mean age, 55.8 years; range, 23-82 years) by using a dual-source CT unit with (a) both tubes set at similar energy (120 kVp) and (b) the total reference milliampere seconds (ie, 110 mAs) split up in a way that 40% was applied to tube A and 60% to tube B. Two series of images were generated simultaneously from the same dataset: (a) standard-dose images (generated from both tubes) reconstructed with filtered back projection (group 1, the reference standard) and (b) reduced-dose images (generated from tube A; 60% dose reduction) reconstructed with sinogram-affirmed iterative reconstruction (SAFIRE) (group 2). In both groups, the analyzed parameters comprised the image noise and the visualization and conspicuity of CT features of ILD. Two readers independently analyzed images from both groups. Results were compared by using the Wilcoxon test for paired samples; the 95% confidence interval was calculated when appropriate. The mean level of objective noise in group 2 was significantly lower than that in group 1 (22.02 HU vs 26.23 HU, respectively; P < .0001). The CT features of ILD in group 1 were always depicted in group 2, with subjective conspicuity scores (a) improved in group 2 for ground-glass opacity, reticulation, and bronchiectasis and/or bronchiolectasis and (b) identical in both groups for honeycombing. The interobserver agreement for their depiction was excellent in both groups (κ, 0.84-0.98). Despite a 60% dose reduction, images reconstructed with SAFIRE allowed similar detection of systematic sclerosis-related ILD compared with the reference standard.