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.

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.

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.

The role of PET/CT in connective tissue disorders: systemic sclerosis, Sjögren's syndrome and systemic lupus erythematosus.

Advanced imaging techniques are needed to help clinicians in the diagnosis, in the choice of the right time for therapeutic interventions or for modifications and monitoring of treatment response in patients with autoimmune connective tissue diseases. Nuclear medicine imaging, especially PET/CT and PET/MRI, may play an important role in detecting disease activity, assessing early treatment response as well as in clarifying the complex mechanisms underlying systemic sclerosis, Sjögren's syndrome or systemic lupus erythematosus. In addition, [18F]FDG PET/CT may help in excluding or detecting coexisting malignancies. Other more specific radiopharmaceuticals are being developed and investigated, targeting specific cells and molecules involved in connective tissue diseases. Further larger studies with standardized imaging protocol and image interpretation are strongly required before including PET/CT in the diagnostic work-up of subsets of patients with autoimmune connective tissue diseases.

Chest high-resolution computed tomography in patients with connective tissue disease: pulmonary conditions beyond "the usual suspects".

The term "connective tissue diseases" (CTDs) refers to a heterogeneous group of autoimmune disorders, including systemic sclerosis, rheumatoid arthritis, Sjögren's syndrome, systemic lupus erythematosus, polymyositis, dermatomyositis, antisynthetase syndrome, and mixed connective tissue disease. Chest high-resolution computed tomography (HRCT) is the imaging method of choice for evaluating patients with known or suspected CTD-related interstitial lung disease (CTD-ILD), a complication accounting for substantial morbidity and mortality. While specific HRCT patterns and signs of CTD-ILD have been extensively described (hence the designation "the usual suspects"), the knowledge of various, less frequent conditions involving the lungs in patients with CTD would help the radiologist produce a clinically valuable report, thus potentially influencing patient management. This paper aims to provide an up-to-date review of various unusual pulmonary CTD-related conditions the radiologist should be aware of; namely, acute exacerbation of CTD-ILD, CTD-related interstitial lung abnormalities, lung amyloidosis, MALT lymphoma, antisynthetase syndrome, pleuroparenchymal fibroelastosis-like lesion, drug-induced ILD, combined pulmonary fibrosis and emphysema, and pulmonary hypertension. For each condition, the chest HRCT appearance and the key histopathological and clinical features are resumed, helping the radiologist participate actively in the multidisciplinary discussion of complex clinical cases.

Computed Tomography Findings Suggestive of Connective Tissue Disease in the Setting of Usual Interstitial Pneumonia.

PURPOSE: A usual interstitial pneumonia (UIP) pattern is common in idiopathic pulmonary fibrosis (IPF) and connective tissue disease-related interstitial lung disease (CTD-ILD). The purpose of the study was to validate imaging findings differentiating CTD-ILD from IPF in UIP. METHODS: Patients with a multidisciplinary diagnosis of CTD-ILD or IPF and a UIP pattern on computed tomography and/or pathology were included in this study. Prevalence of 3 computed tomography findings shown to be associated with CTD-ILD (the straight edge sign [SES], the exuberant honeycombing sign, and the anterior upper lobe sign [AULS]) were tabulated in CTD-ILD and IPF subjects. The ability of each of these signs to discriminate between CTD-ILD and IPF was evaluated. Survival analysis was also performed using log-rank analysis. RESULTS: The study cohort included 50 CTD-ILD and 100 IPF subjects with UIP. The SES and the AULS were more common in CTD-ILD than IPF (prevalence, 36.0% and 34.9% in CTD-ILD vs 8.3% and 17.2% in IPF, respectively [P = 0.0105 - <0.001]). The highest specificity (95.7%) of CTD-ILD diagnosis was seen with bilateral SES. Moreover, the SES was associated with improved survival (P = 0.0383), which appeared to be largely because of improvement in survival in IPF subjects. The presence of AULS was associated with pulmonary functional abnormalities. CONCLUSIONS: A radiographic UIP pattern with evidence of SES or the AULS should raise suspicion for CTD-ILD rather than IPF. Patients with IPF and SES have an attenuated disease course and might represent a different phenotype than those without the SES.

Expanding the phenotypic spectrum of Mendelian connective tissue disorders to include prominent kidney phenotypes.

Heritable connective tissue disorders are a group of diseases, each rare, characterized by various combinations of skin, joint, musculoskeletal, organ, and vascular involvement. Although kidney abnormalities have been reported in some connective tissue disorders, they are rarely a presenting feature. Here we present three patients with prominent kidney phenotypes who were found by whole exome sequencing to have variants in established connective tissue genes associated with Loeys-Dietz syndrome and congenital contractural arachnodactyly. These cases highlight the importance of considering connective tissue disease in children presenting with structural kidney disease and also serves to expand the phenotype of Loeys-Dietz syndrome and possibly congenital contractural arachnodactyly to include cystic kidney disease and cystic kidney dysplasia, respectively.

Evaluation of serum interleukin-6 (IL-6), IL-13, and IL-17 levels and computed tomography finding in interstitial lung disease associated with connective tissue disease patients.

OBJECTIVE: Interstitial lung disease (ILD) is one of the most severe complications which is associated with connective tissue disease (CTD) and causes to morbidity and mortality. So, we aimed to determine serum levels of interleukin-6 (IL-6), IL-13, and IL-17, to investigate whether these cytokines are related to CTD-ILD, and to find their possible contribution to determining the prognosis of the disease. METHODS: A total of 150 participants, 80 patients diagnosed with CTD-ILD (mean age, 58.21 ± 12.36) and 70 healthy controls (mean age, 57.07 ± 9.60) were recruited from the rheumatology department between January 2016 and June 2019 in the study. High-resolution computed tomography (HRCT) findings were scored as similarly to previous studies. Serum IL-6, IL 13, and IL-17 levels were measured by ELISA test kits. RESULTS: The levels of IL-6, IL-13, and IL-17 in CTD patients were significantly higher than the healthy individuals (p < 001), but the HRCT score's relation were not determined. IL-6 was associated with disease duration and disease activity scores of DAS28, ESDAII, and dSSc. There was a significant relation between dSSc, HCRT fibrosis, and total score.CRP, hemoglobin, and platelets were associated with the HRCT inflammation pattern. CONCLUSION: At the study, it has been observed that serum IL-13, IL-6 and IL-17 levels are increased in patients with CTD-ILD. Besides, IL-6 was associated with disease activity scores of DAS28, ESDAII, and dSSc. Also, HRCT fibrosis score is associated with dSSc. Further and comprehensive studies are needed to understand better the complex intersection of lung disease with systemic autoimmunity. Key Points • Serum IL-13, IL-6, and IL-17 levels are increased in patients with CTD-ILD. • IL-6 was associated with disease activity scores of DAS28, ESDAII, and diffuse skin involvement. • HRCT fibrosis score is associated with diffuse skin involvement in patients with SSc-ILD. • HRCT inflammation score is associated with PAH.

Confirming the involvement of PIEZO2 in the etiology of Marden-Walker syndrome.

Pathogenic heterozygous variants in PIEZO2 typically cause distal arthrogryposis type 5 (DA5) and the closely related Gordon syndrome (GS). Only one case of PIEZO2-related Marden-Walker syndrome (MWS) has been reported to date. We report the phenotypic features of a Saudi female patient with features consistent with MWS in whom we identified a novel de novo likely pathogenic variant in PIEZO2. Our case lends support to the link between PIEZO2 and MWS.

[Lung cancer combined with connective tissue disease-related interstitial lung disease: CT features].

Objective: To investigate the CT features and dynamic changes of new developed lung cancer in patients with connective tissue disease-related interstitial lung disease (CTD-ILD). Methods: A series of chest CT images of 58 CTD-ILD patients during follow-up were collected. The CT features of interstitial lung disease, the initial appearance time of lung cancer, the time of diagnosis of lung cancer, the morphological characteristics (location, shape, size) of lung cancer lesions and the dynamic changes of CT features were analyzed. Results: Among 58 patients, rheumatoid arthritis was the most common (31 cases). Chest CT images showed coexistence of two or more interstitial CT signs. During the follow-up, a total of 59 lung cancer lesions were found. The median time of lung cancer lesion occurred was 289 days. The median delay in diagnosis was 43 days. There were 44 cases of non-small cell lung cancer (including 23 cases of squamous cell carcinoma and 19 cases of adenocarcinoma), 12 cases of small cell lung cancer. Forty-three (72.9%) lesions were located in the lower lobes and 41 (69.5%) lesions were located in the area of pulmonary interstitial fibrosis. According to CT morphological characteristics of lung cancer, nodular type (37 cases), inflammatory consolidation (12 cases) and intra-honeycomb type (10 cases) were identified. Conclusions: The chest CT features of patients with CTD-ILD are complex. New developed lung cancer is easily missed or misdiagnosed in the early stage. Pay attention to the special CT characteristics of CTD-ILD with lung cancer is helpful for early diagnosis.

Tumor Necrosis Factor Induces Obliterative Pulmonary Vascular Disease in a Novel Model of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.

OBJECTIVE: Connective tissue disease (CTD)-associated pulmonary arterial hypertension (PAH) is the second most common etiology of PAH and carries a poor prognosis. Recently, it has been shown that female human tumor necrosis factor (TNF)-transgenic (Tg) mice die of cardiopulmonary disease by 6 months of age. This study was undertaken to characterize this pathophysiology and assess its potential as a novel model of CTD-PAH. METHODS: Histologic analysis was performed on TNF-Tg and wild-type (WT) mice to characterize pulmonary vascular and right ventricular (RV) pathology (n = 40 [4-5 mice per group per time point]). Mice underwent right-sided heart catheterization (n = 29) and micro-computed tomographic angiography (n = 8) to assess vascular disease. Bone marrow chimeric mice (n = 12), and anti-TNF-treated mice versus placebo-treated mice (n = 12), were assessed. RNA sequencing was performed on mouse lung tissue (n = 6). RESULTS: TNF-Tg mice displayed a pulmonary vasculopathy marked by collagen deposition (P < 0.001) and vascular occlusion (P < 0.001) with associated RV hypertrophy (P < 0.001) and severely increased RV systolic pressure (mean ± SD 75.1 ± 19.3 mm Hg versus 26.7 ± 1.7 mm Hg in WT animals; P < 0.0001). TNF-Tg mice had increased α-smooth muscle actin (α-SMA) staining, which corresponded to proliferation and loss of von Willebrand factor (vWF)-positive endothelial cells (P < 0.01). There was an increase in α-SMA-positive, vWF-positive cells (P < 0.01), implicating endothelial-mesenchymal transition. Bone marrow chimera experiments revealed that mesenchymal but not bone marrow-derived cells are necessary to drive this process. Treatment with anti-TNF therapy halted the progression of disease. This pathology closely mimics human CTD-PAH, in which patient lungs demonstrate increased TNF signaling and significant similarities in genomic pathway dysregulation. CONCLUSION: The TNF-Tg mouse represents a novel model of CTD-PAH, recapitulates key disease features, and can serve as a valuable tool for discovery and assessment of therapeutics.

The utility of magnetic resonance imaging lesion combinations in the sacroiliac joints for diagnosing patients with axial spondyloarthritis. A prospective study of 204 participants including post-partum women, patients with disc herniation, cleaning staff, runners and healthy persons.

OBJECTIVES: To investigate the diagnostic utility of different combinations of SI joint MRI lesions for differentiating patients with axial SpA (axSpA) from other conditions with and without buttock/pelvic pain. METHODS: A prospective cross-sectional study included patients with axSpA (n = 41), patients with lumbar disc herniation (n = 25), women with (n = 46) and without (n = 14) post-partum (birth within 4-16 months) buttock/pelvic pain and cleaning assistants (n = 26), long-distance runners (n = 23) and healthy men (n = 29) without pain. Two independent readers assessed SI joint MRI lesions according to the Spondyloarthritis Research Consortium of Canada MRI definitions and pre-defined MRI lesion combinations with bone marrow oedema (BME) and fat lesions (FAT), respectively. Statistical analyses included the proportion of participants with scores above certain thresholds, sensitivity, specificity, positive and negative predictive values and likelihood ratios. RESULTS: BME adjacent to the joint space (BME@joint space) was most frequent in axSpA (63.4%), followed by women with post-partum pain (43.5%), but was present in nearly all groups. BME adjacent to fat lesions (BME@FAT) and BME adjacent to erosions (BME@erosion) were only present in axSpA patients and in women with post-partum pain, but scores ≥3 and ≥4, respectively, were only seen in axSpA patients. FAT@erosion was exclusively recorded in axSpA patients. FAT@joint space and FAT@sclerosis were present in most groups, but with higher scores in the axSpA group. CONCLUSION: BME@joint space and FAT@joint space were frequent in axSpA but also in other conditions, reducing the diagnostic utility. FAT@erosion, and BME@FAT, BME@erosion and FAT@sclerosis above certain thresholds, were exclusively seen in axSpA patients and may thus have diagnostic utility in the differentiation of axSpA from other conditions.

Prevalence and Clinical Significance of Incidental Vertebral Marrow Signal Abnormality in Thoracolumbar Spine MRI.

STUDY DESIGN: A cross-sectional study. OBJECTIVE: This study investigates the prevalence of incidental vertebral marrow signal abnormality (VMSA) in thoracolumbar spine magnetic resonance imaging (MRI) ordered for the evaluation of back and/or leg pain and assess the clinical work-up for VMSAs. SUMMARY OF BACKGROUND DATA: Patients presenting with back pain are often referred for spine MRI for diagnostic evaluation. VMSA is most frequently found in the lumbar spine and is of clinical concern because it can represent malignancy. Standardized procedures for reporting and managing VMSAs do not exist. METHODS: The radiology database at the Oregon Health & Science University health system was queried to identify patients with thoracolumbar spine MRI scans performed between January 2014 and June 2016. Patients 16 years or older with MRIs ordered by providers at a multidisciplinary spine specialty clinic for the diagnostic evaluation of back and/or leg pain were included. Radiology reports were searched for keywords pertaining to VMSAs, such as "malignancy." Medical records of these patients were further reviewed for the clinical work-up and final diagnoses pertaining to the VMSA. RESULTS: The study sample included 1503 individual patients, of whom 65 (4%) had MRI radiology reports that described a VMSA. Thirty-one (48%) of the 65 patients with VMSAs had further evaluation recommended by radiology. Ten (32%) of these 31 patients were followed clinically without further diagnostic testing for the VMSA. Of the 65 patients with VMSAs, only one was diagnosed with malignancy (multiple myeloma). CONCLUSION: While VMSAs are not frequently found on thoracolumbar MRIs ordered to evaluate back and/or leg pain, there is a large amount of heterogeneity in how these abnormalities are documented and managed. This may indicate the need for clinical guidelines for the reporting and management of VMSAs detected on spine MRI and for improvement in communication between radiologists and ordering providers. LEVEL OF EVIDENCE: 3.

Comparative analysis of connective tissue disease-associated interstitial lung disease and interstitial pneumonia with autoimmune features.

OBJECTIVE: This retrospective clinical study aimed to examine the similarities and differences between connective tissue disease-associated interstitial lung disease (CTD-ILD) and interstitial pneumonia with autoimmune features (IPAF) and to identify the influencing factors of CTD-ILD, with a goal of early detection and active treatment of the disease. METHODS: We conducted a retrospective study of 480 patients: 412 with CTD-ILD and 68 with IPAF. Demographic features, clinical characteristics, laboratory indicators, and chest high-resolution computed tomography (HRCT) imaging data were analyzed. RESULTS: Compared with the IPAF group, the CTD-ILD group contained more women, and the incidences of joint pain, dry mouth/dry eyes, and Raynaud's phenomenon were higher; erythrocyte sedimentation rate (ESR) and D-dimer levels were higher; red blood cell (RBC) and hemoglobin (Hb) levels were lower; a high rheumatoid factor (RF) titer (> 2 times the normal upper limit) was observed, and anti-cyclic citrullinated peptide antibody (anti-CCP), anti-keratin antibody (AKA), antinuclear antibody (ANA), and anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5) levels were higher. Compared with CTD-ILD patients, IPAF patients were more likely to present initially with respiratory symptoms, with higher rates of fever, cough and expectoration, dyspnea, and Velcro crackles; anti-Ro52 titers were higher; incidences of honeycombing opacity, reticulate opacity, patchy opacity, and pleural thickening were greater. Female sex, a high RF titer (> 2 times the normal upper limit), anti-CCP positivity, ANA positivity, and anti-MDA5 positivity were risk factors for CTD-ILD when the odds ratios were adjusted. CONCLUSION: CTD-ILD and IPAF patients differed in demographic features, clinical characteristics, laboratory indicators, and chest HRCT imaging data. Female sex, a high RF titer (> 2 times the normal upper limit), anti-CCP positivity, ANA positivity, and anti-MDA5 positivity were risk factors for CTD-ILD.Key Points• This retrospective clinical study comprehensively compared the demographic features, clinical characteristics, laboratory indicators, and chest HRCT imaging data of CTD-ILD and IPAF patients.• The evidence suggested that female sex, a high RF titer, anti-CCP positivity, ANA positivity, and anti-MDA5 positivity were risk factors for CTD-ILD.

Mechanic hands: clinical and capillaroscopy manifestations of patients with connective tissue diseases presented with and without mechanic hands.

OBJECTIVES: The condition known as 'Mechanic's Hands' is a thickened, hyperkeratotic eruption, which is bilaterally symmetric along the fingers, and often occurs in patients with some connective tissue diseases. Nail fold capillaroscopy is a non-invasive technique for evaluation of connective tissue diseases. We evaluated the prevalence of mechanic hands in patients with connective tissue diseases and compared the clinical manifestations and capillaroscopic changes in the patients with and without mechanic hands. METHODS: The clinical manifestations and capillaroscopy of 576 patients with scleroderma, dermatomyositis, systemic lupus erythematosus, Sjogren's syndrome, undifferentiated and mixed connective tissue diseases were evaluated and compared in patients with and without mechanic hands. RESULTS: A total of 576 patients were enrolled. Mechanic hands were observed in 17.2% of patients: 50% of mixed connective tissue disease, 35% of dermatomyositis, 15.4% of scleroderma, 14.9% of undifferentiated connective tissue disease, 14.3% of Sjogren's syndrome, and no patient with SLE. Among them, 80.8% had abnormal capillaroscopic findings. In dermatomyositis patients, Raynaud's phenomenon, anti-Jo-1 positivity, and some capillaroscopy findings were detected more frequently in patients with mechanic hand. In scleroderma, positive Scl70 and capillary loss were observed more frequently in patients without mechanic hands. CONCLUSIONS: Mechanic hands can be a presenting sign of some systemic connective tissue diseases. Probably, finding this sign on examination, especially together with Raynaud's phenomenon or abnormal capillaroscopy, can be helpful in the early diagnosis of the connective tissue diseases and can be used as a predictive and prognostic tool in future studies.