Relationship between nailfold capillaroscopy findings and the etiology and prognosis of interstitial lung disease.

OBJECTIVES: Connective tissue-associated interstitial lung diseases (CTD-ILD) are believed to be caused by microvascular damage. The objective of this study was to assess the nailfold capillaroscopy (NFC) pattern in patients diagnosed with both CTD-ILD and non-CTD-ILD to identify microvascular changes and determine the relation between capillaroscopic parameters, clinical variables, and disease-related measurements. PATIENTS AND METHODS: This cross-sectional study included 95 patients with interstitial lung disease who applied to our Rheumatology and Chest Clinics between September 2021 and July 2023. The patients were divided into two groups based on their diagnosis: non-CTD-ILD (group 1) and CTD-ILD (group 2). Nailfold capillaroscopy was performed. RESULTS: Ninety-five patients, 49 (51% female, mean age 62.31 ± 11.027 years) in group 1 and 46 (69.6% female, mean age 62.09 ± 10.887 years) in group 2, were included in the study. Abnormal capillary morphologies were both detected in the CTD-ILD group and the non-CTD-ILD groups. In patients with a usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT), tortuosity was higher than in patients with non-specific interstitial pneumonia (NSIP) (P = 0.041), and the proportion of tortuosity increased significantly as the duration of the disease increased (P = 0.016). CONCLUSION: Our study highlights capillaroscopic abnormalities alone may not be sufficient to differentiate CTD-ILD (other than systemic sclerosis) from non-CTD-ILD. The presence of NFC abnormalities in non-CTD-ILD may suggest that fibrotic lung disease could potentially play a role in the deterioration of the microvascular structure or abnormal angiogenesis. Our study demonstrated that a multidisciplinary approach, incorporating clinical, morphological, pathological, and serological evaluations, is necessary for interpreting ILD. Key Points • Capillaroscopic abnormalities can also be seen in non-CTD-ILD. • Capillaroscopy findings do not distinguish the non-Ssc etiology of ILD. • Nailfold capillaroscopy may have the potential to serve as a useful tool in predicting prognosis and monitoring the disease progression in patients with idiopathic pulmonary fibrosis (IPF).

Connective tissue disease-associated lung disease in children.

Connective tissue diseases are a heterogeneous group of autoimmune diseases that can affect a variety of organ systems. Lung parenchymal involvement is an important contributor to morbidity and mortality in children with connective tissue disease. Connective tissue disease-associated lung disease in children often manifests as one of several radiologic-pathologic patterns of disease, with certain patterns having a propensity to occur in association with certain connective tissue diseases. In this article, key clinical, histopathologic, and computed tomography (CT) features of typical patterns of connective tissue disease-associated lung disease in children are reviewed, with an emphasis on radiologic-pathologic correlation, to improve recognition of these patterns of lung disease at CT and to empower the pediatric radiologist to more fully contribute to the care of pediatric patients with these conditions.

Screening value of lung ultrasound and pleural shear wave elastography in connective tissue disease-related interstitial lung disease: a preliminary study.

OBJECTIVE: To explore the diagnostic value of lung ultrasound (LUS) and pleural shear wave elastography (SWE) for connective tissue disease-interstitial lung disease (CTD-ILD). METHODS: We selected 104 patients diagnosed with connective tissue disease (CTD) at our hospital. All patients underwent LUS, SWE, and high-resolution computed tomography (HRCT). With HRCT as the imaging gold standard for diagnosis, patients were categorized into CTD-ILD and CTD-non-ILD groups. We employed paired chi-square tests to compare the diagnostic differences between HRCT and LUS for ILD. Receiver operating characteristic (ROC) curves were used to assess the diagnostic value of pleural SWE for ILD. Correlation analysis was performed between pleural elasticity values and lung ultrasound scores. RESULTS: The sensitivity, specificity, positive likelihood ratio, and negative likelihood ratio of LUS for diagnosing CTD-ILD were 93.3%, 86.2%, 6.761, and 0.078, respectively. There was no statistically significant difference in the results between HRCT and LUS (P = 1.000), with a kappa value of 0.720 (P < 0.001). There was a statistically significant difference in the pleural elasticity in the bilateral lower back region between the case and control groups (P < 0.001). The area under the receiver operating characteristic (ROC) curve (AUC) for pleural SWE in diagnosing CTD-ILD was 0.685. In CTD-ILD patients, there was no significant correlation between pleural elasticity values and LUS scores (P > 0.05). CONCLUSION: The LUS can serve as an important imaging method for screening for CTD-ILD and assessing the severity of the disease. However, pleural SWE has been shown to demonstrate lower diagnostic efficacy for CTD-ILD, and its ability to assess disease severity is limited.

Nail involvement in connective tissue diseases: an epidemiological, clinical, and dermoscopic study.

BACKGROUND: The assessment of nail changes in connective tissue diseases (CTD) has been rarely explored in previous studies. The use of dermoscopy to study vascular changes in nailfolds is an interesting diagnostic technique. The aim of the study was to describe the epidemiological, clinical, and dermoscopic features of nail lesions in CTD. METHODS: A prospective study was performed at the Dermatology Department of Habib Thameur Hospital (Tunis, Tunisia) in collaboration with the Internal Medicine Department over a period of 15 months, from July 2020 to September 2021, including patients diagnosed with systemic sclerosis (SS), systemic lupus erythematosus (SLE) and dermatomyositis (DM). RESULTS: Our study included 48 patients. Nail involvement was found in 44 cases. Dermoscopic nailfold abnormalities were identified in 37 cases. The most common clinical features were ragged cuticle, nailfold erythema, and onycholysis. Additionally, splinter hemorrhage, longitudinal ridging, lunula abnormalities, melanonychia, trachyonychia, leukonychia, increase in transverse curvature, parrot beak nail, half and half nails, and onychorrhexis were described. Nailfold dermoscopy showed a normal pattern in 10 cases, a nonspecific pattern in nine cases (SLE), and a scleroderma pattern in 29 cases (SS and DM). The scleroderma pattern was further categorized into an early pattern (6), an active pattern (14), and a late pattern (9). Normal pattern was observed solely in patients in remission. The late scleroderma pattern was associated with disease duration and systemic involvement. In SLE, disease activity correlated with onycholysis, nailfold erythema, and pathologic pattern in dermoscopy. However, patients with DM displayed a positive correlation between pulmonary involvement and scleroderma pattern. CONCLUSION: Nail involvement in CTD includes a diverse range of abnormalities. Despite being nonspecific, it can provide crucial clues for establishing a diagnosis. Nailfold dermoscopy serves as a mirror for microangiopathy, enabling the detection of changes at an initial stage, and thus, it becomes a diagnostic and prognostic tool.

Radiological and histopathological features and treatment response by subtypes of interstitial pneumonia with autoimmune features: A prospective, multicentre cohort study.

BACKGROUND: Patients with idiopathic interstitial pneumonia (IIP) have a favourable prognosis when they have interstitial pneumonia with autoimmune features (IPAF). However, precise IPAF-related findings from high-resolution computed tomography (HRCT) and lung histopathological specimens and the treatment response have not been fully determined. Therefore, this study was conducted to evaluate the relationship between findings on HRCT or lung histopathological specimens and the progression of interstitial pneumonia in patients with IPAF. METHODS: This multicentre cohort study prospectively enrolled consecutive patients with IIP. At the diagnosis of IIP, we systematically evaluated 74 features suggestive of connective tissue diseases and followed them up. HRCT, lung specimens, serum antibodies, and the clinical course were also evaluated. RESULTS: Among 222 patients with IIP, 26 (11.7%) fulfilled the IPAF criteria. During a median observation period of 36 months, patients with IPAF showed better survival than those without IPAF (p = 0.034). While histopathological findings were not related to IPAF, nonspecific interstitial pneumonia (NSIP) with organizing pneumonia (OP) overlap was the most prevalent HRCT pattern (p < 0.001) and the consolidation opacity was the most common radiological finding in IPAF (p = 0.017). Furthermore, in patients with IPAF, the diagnosis of COP or NSIP with OP overlap was associated with a higher increase in %FVC in 1 year than in those with idiopathic pulmonary fibrosis, NSIP, or unclassifiable IIP (p = 0.002). CONCLUSIONS: This study shows the presence of consolidation opacity on HRCT and the diagnosis of COP or NSIP with OP overlap are associated with IPAF and its favourable treatment response in patients with IPAF.

Non-contrast computed tomography-based radiomics for staging of connective tissue disease-associated interstitial lung disease.

Rationale and introduction: It is of significance to assess the severity and predict the mortality of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). In this double-center retrospective study, we developed and validated a radiomics nomogram for clinical management by using the ILD-GAP (gender, age, and pulmonary physiology) index system. Materials and methods: Patients with CTD-ILD were staged using the ILD-GAP index system. A clinical factor model was built by demographics and CT features, and a radiomics signature was developed using radiomics features extracted from CT images. Combined with the radiomics signature and independent clinical factors, a radiomics nomogram was constructed and evaluated by the area under the curve (AUC) from receiver operating characteristic (ROC) analyses. The models were externally validated in dataset 2 to evaluate the model generalization ability using ROC analysis. Results: A total of 245 patients from two clinical centers (dataset 1, n = 202; dataset 2, n = 43) were screened. Pack-years of smoking, traction bronchiectasis, and nine radiomics features were used to build the radiomics nomogram, which showed favorable calibration and discrimination in the training cohort {AUC, 0.887 [95% confidence interval (CI): 0.827-0.940]}, the internal validation cohort [AUC, 0.885 (95% CI: 0.816-0.922)], and the external validation cohort [AUC, 0.85 (95% CI: 0.720-0.919)]. Decision curve analysis demonstrated that the nomogram outperformed the clinical factor model and radiomics signature in terms of clinical usefulness. Conclusion: The CT-based radiomics nomogram showed favorable efficacy in predicting individual ILD-GAP stages.

Computed Tomography-Based Deep Learning Model for Assessing the Severity of Patients With Connective Tissue Disease-Associated Interstitial Lung Disease.

OBJECTIVES: This study aimed to develop a computed tomography (CT)-based deep learning model for assessing the severity of patients with connective tissue disease (CTD)-associated interstitial lung disease (ILD). METHODS: The retrospective study included 298 CTD-ILD patients between January 2018 and May 2022. A deep learning-based RDNet model was established (1610 fully annotated CT images for training and 402 images for validation). The model was used to automatically classify and quantify 3 radiologic features (ground glass opacities [GGOs], reticulation, and honeycombing), along with a volumetric sum of 3 areas (ILD%). As a control, we used 4 previously defined CT threshold methods to calculate the ILD assessment index. The Spearman rank correlation coefficient ( r ) evaluated the correlation between various indicators and the lung function index in the remaining 184 CTD-ILD patients who were staged according to the gender-age-physiology (GAP) system. RESULTS: The RDNet model accurately identified GGOs, reticulation, and honeycombing, with corresponding Dice indexes of 0.784, 0.782, and 0.747, respectively. A total of 137 patients were at GAP1 (73.9%), 36 patients at GAP2 (19.6%), and 11 patients at GAP3 (6.0%). The percentages of reticulation and honeycombing at GAP2 and GAP3 were markedly elevated compared with those at GAP1 ( P < 0.001). The percentage of GGOs was not significantly different among the GAP stages ( P = 0.62). As the GAP stage increased, all lung function indicators tended to decrease, and the composite physiologic index (CPI) indicated an upward tendency. The percentage of honeycombs moderately correlated with the percentage of diffusing capacity of the lung for carbon monoxide (DLco%) ( r = -0.58, P < 0.001) and CPI ( r = 0.63, P < 0.001). The ILD assessment index calculated by the CT threshold method (-260 to -600 Hounsfield units) had a low correlation with DLco% and CPI (DLco%: r = -0.42, P < 0.001; CPI: r = 0.45, P < 0.001). CONCLUSIONS: The RDNet model can quantify GGOs, reticulation, and honeycombing of chest CT images in CTD-ILD patients, among which honeycombing had the most significant effect on lung function indicators. In addition, this model provided good clinical utility for evaluating the severity of CTD-ILD.

A computed tomography-based radiomics nomogram for predicting overall survival in patients with connective tissue disease-associated interstitial lung disease.

OBJECTIVES: Accurate prognostic prediction is beneficial for the management of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). The purpose of the present study was to develop and validate a nomogram using clinical features and computed tomography (CT) based radiomics features to predict overall survival (OS) in patients with CTD-ILD, and to assess the incremental prognostic value the radiomics might add to clinical risk factors. MATERIALS & METHODS: Patients from two clinical centers with CTD-ILD were enrolled in the present retrospective study. A radiomics signature, a clinical model and a combined nomogram were developed and assessed in the cohorts. The incremental value of radiomics signature to the clinical independent risk factors in survival prediction was evaluated. The models were externally validated to evaluate the model generalization ability. RESULTS: A total of 215 patients (mean age, 53 years ± 14 [standard deviation], 45 men) were evaluated. Patients with higher radiomics scores had higher mortality risk than those with lower radiomics scores (Hazard ratio, 12.396; 95% CI, 3.364-45.680; P < 0.001). The combined nomogram showed better predictive capability than the clinical model did with higher C-indices (0.800, 0.738, 0.742 vs. 0.747, 0.631, 0.587 in the training, internal- and external-validation cohort, respectively), time-AUCs and overall net-benefit. CONCLUSION: The radiomics signature is a potential prognostic biomarker of CTD-ILD and add incremental value to the clinical independent risk factors. The combined nomogram can provide a more accurate estimation of OS than the clinical model for CTD-ILD patients. CLINICAL RELEVANCE STATEMENT: The developed combined nomogram showed accurate prognostic prediction performance, which is beneficial for the management of CTD-ILD patients. It also proved radiomics could extract prognostic information from CT images.

Nailfold capillaroscopy findings of interstitial pneumonia with autoimmune features.

BACKGROUND/AIMS: We evaluated nailfold capillaroscopy (NFC) of interstitial pneumonia with autoimmune features (IPAF) and compared it with that of patients with connective tissue disease-interstitial lung disease (CTD-ILD) and idiopathic interstitial pneumonia (IIP). METHODS: Patients with newly diagnosed as ILD were evaluated using NFC. Baseline demographic, clinical, serological, and high-resolution CT findings were collected. NFC was semi-quantitatively scored with six domains ranging from 0 to 18. In addition, the overall patterns (scleroderma/non-scleroderma patterns) were determined. RESULTS: A total of 81 patients (31 with CTD-ILD, 18 with IPAF, and 32 with IIP) were included. The non-specific interstitial pneumonia pattern was the most common ILD pattern in the CTD-ILD and IPAF groups, whereas the usual interstitial pneumonia pattern was the most common in the IIP group. The semi-quantitative score of the CTD-ILD group was higher than that of the IPAF or IIP groups (5.8 vs 4.2 vs 3.0, p < 0.001, respectively). Giant capillaries and haemorrhages were more frequently present in the CTD-ILD and IPAF groups than in the IIP group. A scleroderma pattern was present in 27.8% of the IPAF group, whereas none of the IIP patients showed a scleroderma pattern. CONCLUSION: NFC findings may be useful in classifying patients with ILD into CTD-ILD/IPAF/IIP.

Spectrum of high-resolution computed tomography pattern in lungs in patients with connective tissue disorders.

Background: Connective tissue disease associated with interstitial lung disease, or CT-ILD, is a lung condition that affects a large number of patients with a connective tissue disease. Objective: Our aim in this study is to correlation between images of high-resolution computed tomography (HRCT) of different connective tissue diseases associated interstitial lung diseases (CTD-ILDs). Methods: We shall be aiming to investigate the feasibility of HRCT imaging and thereby avoid lung biopsy in such patients. Results: Rheumatoid arthritis predominantly presented with usual interstitial pneumonia (UIP) (47.8%), followed by nonspecific interstitial pneumonia (NSIP) (30.4%). Mixed connective tissue disorder predominantly presented with NSIP and UIP (42.8%), followed by organizing pneumonia (OP) (14.2%). Systemic lupus erythematosus predominantly presented with UIP (38.8%), followed by NSIP (27.7%). Sjogren's syndrome predominantly presented with lymphocytic interstitial pneumonia (40%), followed by UIP (26.6%). Scleroderma predominantly presented with UIP (45.4%), followed by NSIP (36.4%). Sarcoidosis predominantly presented with UIP (75%), followed by NSIP (25%). Dermatomyositis predominantly presented with NSIP (50%), followed by UIP and OP each (25%). Conclusion: Both clinicians and radiologists should be aware of the expected evolution of HRCT changes in a variety of CT-ILDs.

Résumé Contexte: La maladie du tissu conjonctif associée à la maladie pulmonaire interstitielle, ou CT ILD, est une affection pulmonaire qui affecte un grand nombre de patients atteints d'une maladie du tissu conjonctif. Objectif: Notre objectif dans cette étude est de mettre en corrélation des images de tomodensitométrie à haute résolution (HRCT) de différentes maladies du tissu conjonctif associées à des maladies pulmonaires interstitielles (CTD ILDs). Méthodes: Notre objectif sera d'étudier la faisabilité de l'imagerie HRCT et d'éviter ainsi la biopsie pulmonaire chez ces patients. Résultats: La polyarthrite rhumatoïde se présentait principalement avec une pneumonie interstitielle habituelle (PUI) (47,8 %), suivie d'une pneumonie interstitielle non spécifique (NSIP) (30,4 %). Trouble mixte du tissu conjonctif présenté principalement avec NSIP et UIP (42,8 %), suivi d'une pneumonie organisée (OP) (14,2 %). Le lupus érythémateux disséminé présentait principalement une UIP (38,8 %), suivie d'une NSIP (27,7 %). Le syndrome de Sjogren présentait principalement une pneumonie interstitielle lymphocytaire (40 %), suivie d'une PUI (26,6 %). La sclérodermie se présentait principalement avec l'UIP (45,4 %), suivi du NSIP (36,4 %). La sarcoïdose se présentait principalement avec l'UIP (75 %), suivi du NSIP (25 %). La dermatomyosite se présentait principalement avec NSIP (50 %), suivi par UIP et OP chacun (25 %). Conclusion: Les cliniciens et les radiologues doivent être conscients de l'évolution attendue des changements HRCT dans une variété d'ILD CT. Mots-clés: Tissu conjonctif, CT ILDs, tomodensitométrie haute résolution, poumon interstitiel.

Classification of pulmonary sounds through deep learning for the diagnosis of interstitial lung diseases secondary to connective tissue diseases.

Early diagnosis of interstitial lung diseases secondary to connective tissue diseases is critical for the treatment and survival of patients. The symptoms, like dry cough and dyspnea, appear late in the clinical history and are not specific, moreover, the current approach to confirm the diagnosis of interstitial lung disease is based on high resolution computer tomography. However, computer tomography involves x-ray exposure for patients and high costs for the Health System, therefore preventing its use for a massive screening campaign in elder people. In this work we investigate the use of deep learning techniques for the classification of pulmonary sounds acquired from patients affected by connective tissue diseases. The novelty of the work consists of a suitably developed pre-processing pipeline for de-noising and data augmentation. The proposed approach is combined with a clinical study where the ground truth is represented by high resolution computer tomography. Various convolutional neural networks have provided an overall accuracy as high as 91% in the classification of lung sounds and have led to an overwhelming diagnostic accuracy in the range 91%-93%. Modern high performance hardware for edge computing can easily support our algorithms. This solution paves the way for a vast screening campaign of interstitial lung diseases in elder people on the basis of a non-invasive and cheap thoracic auscultation.

Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis.

INTRODUCTION: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue disease (CTD) with substantial variability in prevalence and outcomes reported across CTD subtypes. This systematic review summarises the prevalence, risk factors and ILD patterns on chest computed tomography of CTD-ILD. METHODS: A comprehensive search was performed in Medline and Embase to identify eligible studies. Meta-analyses were completed using a random effects model to determine the pooled prevalence of CTD-ILD and ILD patterns. RESULTS: 11 582 unique citations were identified with 237 articles included. Pooled prevalence of ILD was 11% in rheumatoid arthritis (95% CI 7-15%), 47% in systemic sclerosis (44-50%), 41% in idiopathic inflammatory myositis (33-50%), 17% in primary Sjögren's syndrome (12-21%), 56% in mixed connective tissue disease (39-72%) and 6% in systemic lupus erythematosus (3-10%). Usual interstitial pneumonia was the most prevalent ILD pattern in rheumatoid arthritis (pooled prevalence of 46%), while nonspecific interstitial pneumonia was the most common ILD pattern in all other CTD subtypes (pooled prevalence range 27-76%). Across all CTDs with available data, positive serology and higher inflammatory markers were risk factors for development of ILD. DISCUSSION: We identified substantial variability in ILD across CTD subtypes suggesting that CTD-ILD is too heterogenous to be considered a single entity.

Case report of severe coronary artery tortuosity with coexisting connective tissue disease.

Coronary artery tortuosity (CAT) is frequently detected during coronary angiography or coronary electron-beam computed tomography angiography by cardiovascular interventionalists. In this article, we described the case of a 69-year-old female patient with recurrent chest discomfort for 1 month and recurrence 1 week ago, accompanied by emaciation, gastrointestinal discomfort, and low skin temperature at the extremities. After a series of tests, the patient was finally diagnosed with severe CAT and coexisting connective tissue disease. Accordingly, she was treated with conventional medications, and diet and lifestyle modifications. The symptoms of the patient resolved gradually after 1 year of follow-up. Although there is no unanimous conclusion on the pathogenesis and clinical characteristics of CAT, this disease may provide a clue to the diagnosis of connective tissue disease, and warrants exploration through further research.

CT predictors of outcomes in patients with connective tissue disease and progressive lung fibrosis.

PURPOSE: Progressive fibrosing interstitial lung disease (PF-ILD) is a phenotype defined by rapid clinical progression towards respiratory failure. While idiopathic pulmonary fibrosis is the archetype of PF-ILD, connective tissue disease associated interstitial lung disease (CTD-ILD) can also manifest as PF-ILD. Few studies have described the value of serial computed tomography (CT) in predicting clinical progression of ILD. We explore which single and serial clinical and radiographic variables, in particular serial CT variables and a novel variable, the right lower lobe anterior bronchial angle (RLL-ABA), best predict mortality, oxygen requirement, hospital admissions, and lung transplant in CTD-ILD. METHODS: This is a single-center retrospective study of 84 patients with a history of CTD-ILD. Cox survival analysis was used to predict two endpoints, all-cause mortality and composite negative outcomes (CNO): new oxygen requirement, respiratory admission, lung transplant, and death. RESULTS: On serial CT, change in pulmonary artery (PA) size and RLL-ABA were predictive of mortality and CNO, and change in fibrosis was predictive of mortality alone. On single CT, the extent of fibrosis, PA size, and PA to aorta ratio were predictive of mortality and CNO. Among clinical variables, oxygen requirement, forced vital capacity (FVC), change in FVC, and worsening shortness of breath were predictive of mortality and CNO, and diffusing capacity for carbon monoxide was predictive of mortality alone. CONCLUSIONS: In addition to clinical and single CT variables, serial CT measurements such as change in extent of fibrosis, PA size, PA to aorta ratio, and RLL-ABA were predictive of mortality and CNO.

Progression and prognosis of interstitial pneumonia with autoimmune features: a longitudinal, prospective, multi-centre study.

OBJECTIVES: To evaluate the rate of progression towards specific autoimmune diseases (SADs) of a prospective, multi-centre cohort of patients classifiable as interstitial pneumonia with autoimmune features (IPAF). METHODS: IPAF patients were enrolled based on specific research criteria, and jointly followed by rheumatologists and pulmonologists for at least one year with clinical check-ups, serological exams including autoimmunity, capillaroscopy and high-resolution computed tomography (HRCT). Diagnostic assessment was repeated at least once a year, or earlier when deemed useful. RESULTS: We enrolled 191 IPAF patients through 95 different combinations of IPAF criteria. Of these, 24.1% progressed towards SAD, mainly in connective tissue diseases but also in microscopic polyangiitis. The IPAF patients who progressed were younger than stable IPAF patients (63±10 years vs. 68±9 years, p=0.002) and had a longer follow-up (36.9±18.7 vs. 29.3±15.7 months, p=0.007), but similar severity. No parameters were associated with overall progression, but some parameters were associated with the development of specific diagnoses: Sjögren's syndrome with positivity for SSA (p=0.007, χ2 7.4); idiopathic inflammatory myopathy with mechanic's hands (p=<0.0001, χ2 12.6), organizing pneumonia pattern (p=0.01, χ2 6.1), positivity for anti-Pm/scl (p=0.04 χ2 4.1) and anti-MDA5 (p=0.04, χ2 4.2); systemic sclerosis with palmar telangiectasias (p=<0.0001 2 18.3), positivity for anti-Scl70 (p=<0.0001 χ2 12.5) and anti-PM/Scl (p=0.001 χ2 10.1). CONCLUSIONS: IPAF patients had a rate of progression towards SAD similar to that reported in previous studies on undifferentiated connective tissue diseases, thus including some patients in which lung involvement could represent the first or even the sole clinical manifestation of a SAD.

Screening value of lung ultrasound in connective tissue disease related interstitial lung disease.

BACKGROUND: Interstitial lung disease (ILD) is a common pulmonary complication of connective tissue disease (CTD) that can lead to poor quality of life and prognosis. OBJECTIVES: To explore the screening value of lung ultrasound (LUS) for connective tissue disease-associated interstitial lung disease (CTD-ILD). METHODS: Data of patients with CTD were collected, and each patient underwent LUS, high-resolution computed tomography (HRCT), and pulmonary function tests. Considering HRCT is the gold standard for diagnosing CTD-ILD, patients were divided into CTD-ILD and CTD-non-ILD groups. The LUS and HRCT results were assessed using semiquantitative and Warrick scores, respectively. Pulmonary function results were also collected. Receiver operating characteristic (ROC) curves were used to evaluate the accuracy of LUS diagnosis. Spearman correlation analysis was used to analyze the correlation between LUS, HRCT, and lung function indices. RESULTS: A total of 88 patients (65 with CTD-ILD and 23 with CTD-non-ILD) were included in this study. The sensitivity and specificity of LUS for the diagnosis of CTD-ILD were 86.60% and 82.60%, respectively, which was consistent with the HRCT results (P < 0.05). The LUS results (total number of B-lines, frequency of B-line, pleural thickness, and pleural-line irregularity) were positively correlated with the HRCT Warrick score (r = 0.77, 0.76, 0.65 and 0.71, P < 0.05). CONCLUSIONS: LUS may be a promising tool for screening patients with CTD-ILD.

Clinical and HRCT features of amyopathic dermatomyositis associated with interstitial lung disease: A retrospective study of 128 patients with connective tissue disease-related interstitial lung disease.

BACKGROUND: This study retrospectively analyzed the laboratory data and chest images of patients with amyopathic dermatomyositis associated with interstitial lung disease (ADM-ILD) and patients with other connective tissue disease-related ILDs (CTD-ILDs) to find a characteristic index for the early recognition of ADM-ILD and help clinicians consider the possibility of ADM-ILD as soon as possible. METHODS: In our cohort study, the records of 128 Chinese patients with CTD-ILD, including 33 ADM-ILD patients, 37 rheumatoid arthritis (RA)-ILD patients, 33 primary Sjogren's syndrome (pSS)-ILD patients, 14 systemic sclerosis (SSc)-ILD patients and 11 systemic lupus erythematosus (SLE)-ILD patients. The patients' clinical features, laboratory parameters, and chest HRCT findings were analyzed. RESULTS: ADM-ILD patients generally had significantly higher LDH (333.52±160.21 U/L), AST (66.21±83.66 U/L), and CK-MB (18.23±8.28 U/L) levels than other CTD-ILD patients. A total of 90.91% (30/33) of ADM-ILD patients had elevated LDH. Patients with ADM-ILD were more prone to organizing pneumonia radiologic patterns on chest HRCT scans than patients with other CTD-ILDs (χ2=37.39, p < 0.001) and were found in 18 of 33 ADM-ILD patients. Anti-MDA5 (45.45%) was the most commonly detected autoantibody in ADM-ILD patients, followed by anti-PL-7 (21.21%), anti-Jo-1 (12.12%), and anti-PL-12 (9.09%), and levels of ALT (96.93±119.79 vs. 17.50±6.218 U/L), AST (113.00±106.13 vs. 23.56±6.91 U/L), LDH (415.00±198.51 vs. 261.94±67.75 U/L) and CK-MB (22.57±5.91 vs. 14.61±8.36 U/L) were significantly higher in anti-MDA5-positive patients, but these patients had significantly lower WBC counts (4.82±2.61 vs. 7.14±3.00 × 109/L), lymphocyte counts (0.72±0.20 vs. 1.23±0.53 × 109/L), and ALB levels (31.90±4.76 vs. 35.49±4.71 g/L). CONCLUSIONS: ADM-ILD patients have higher serum LDH, AST and CK-MB levels, especially serum LDH levels, and are more prone to organizing pneumonia radiologic patterns on chest HRCT scans than other CTD-ILD patients. A high level of serum LDH with ILD may be a useful characteristic index for recognizing ADM-ILD.

Structured report improves radiology residents' performance in reporting chest high-resolution computed tomography: a study in patients with connective tissue disease.

PURPOSE To evaluate the performance of radiology residents (RRs) when using a dedicated structured report (SR) template for chest HRCT in patients with suspected connective tissue disease-interstitial lung disease (CTD-ILD), compared to the traditional narrative report (NR). METHODS We retrospectively evaluated 50 HRCT exams in patients with suspected CTD-ILD. A chest-devoted radiologist reported all the HRCT exams as the reference standard, pointing out pulmonary fibrosis findings (i.e., honeycombing, traction bronchiectasis, reticulation, and volume loss), presence and pattern of ILD, and possible other diagnoses. We divided four RRs into two groups according to their expertise level. In each group, RRs reported all HRCT examinations alternatively with NR or SR, noting each report's reporting time. The Cohen's Kappa, Wilcoxon, and McNemar tests were used for statistical analysis. RESULTS Regarding the pulmonary fibrosis findings, we found higher agreement between RRs and the reference standard reader when using SR than NR, regardless of their expertise level, except for volume loss.RRs' accuracy for "other diagnosis" was higher when using SR than NR, moving from 0.48 to 0.66 in the novel group (p = 0.035) and from 0.44 to 0.80 in the expertise group (p < 0.001). No differences in accuracy were found between ILD presence and ILD pattern. The reporting time was significantly lower (p = 0.001) when using SR than NR. CONCLUSION SR is of value in increasing the reporting of critical chest HRCT findings in the complex CTD-ILD scenario and should be used early and systematically during the residency.

Imaging of Lung Disease Associated with Connective Tissue Disease.

There is a well-known association between the connective tissue disorders (CTDs) and lung disease. In addition to interstitial lung disease, the CTDs may affect the air spaces and pulmonary vasculature. Imaging tests are important not only in diagnosis but also in management of these complex disorders. In the present review, key aspects of the imaging of CTD-reated diseases are discussed.

Imaging of Pulmonary Manifestations of Connective Tissue Disease.

The majority of connective tissue diseases (CTDs) are multisystem disorders that are often heterogeneous in their presentation and do not have a single laboratory, histologic, or radiologic feature that is defined as the gold standard to support a specific diagnosis. Given this challenging situation, the diagnosis of CTD is a process that requires the synthesis of multidisciplinary data which may include patient clinical symptoms, serologic evaluation, laboratory testing, and imaging. Pulmonary manifestations of connective tissue disease include interstitial lung disease as well as multicompartmental manifestations. These CT imaging patterns and features of specific diseases will be discussed in this article.