Objective Interstitial lung disease (ILD) is a common extramuscular manifestation of the anti-synthetase syndrome (ASS). Patients with ASS-ILD are at risk in developing a progressive fibrosing phenotype despite appropriate treatments. This study investigated the risk factors and the predictive value of multiple risk factors for progressive pulmonary fibrosis (PPF) in patients with ASS-ILD. Methods Ninety patients with a diagnosis of ASS and evidence of ILD on high-resolution computed tomography (HRCT) were recruited. Among them, 72 participants completed follow-up for more than 12 months. These patients were further divided into a PPF-ASS group (n = 18) and a non-PPF-ASS group (n = 54). Logistic regression analysis was performed to investigate the risk factors for PPF. The predictive value of the combined risk factors for predicting PPF were analyzed by a ROC curve. Results The PPF-ASS group had a higher rate of positive non-Jo-1 antibodies, a significantly higher neutrophil-to-lymphocyte ratio (NLR) and serum lactate dehydrogenase (LDH), and a significantly lower PaO2/FiO2 ratio and diffusing capacity for carbon monoxide (DLCO%pred) than the non-PPF-ASS group. In addition, elevated serum Krebs von den Lungen-6 (KL-6) level and reticular opacities were significantly more common, and corticosteroid monotherapy at onset was administered more frequently in the PPF-ASS group. The median duration of follow-up was 37.4 months, survival was poorer in the PPF-ASS group, and the overall survival was 88.9%. Multivariate regression analysis further revealed that positive non-Jo-1 antibodies, NLR, and KL-6 were independent risk factors for PPF. These combined indexes had good accuracy (area under the curve = 0.874) in predicting PPF in patients with ASS-ILD. Conclusion Positive non-Jo-1 antibodies, NLR, and serum KL-6 are independent risk factors for PPF in patients with ASS-ILD. Monitoring these markers can potentially predict PPF in this group of patients. Key Points • Positive non-Jo-1 antibodies, NLR, and serum KL-6 are independent risk factors associated with PPF in patients with ASS-ILD. • Monitoring non-Jo-1 antibodies, NLR, and serum KL-6 can potentially predict PPF in patients with ASS-ILD.

Coexisting anti‐MDA5 and anti‐PL‐7 antibodies are extremely rare. Anti‐MDA5 is associated with rapidly progressive interstitial lung disease (RP‐ILD), while anti‐PL‐7 is often associated with chronic or subacute ILD and better outcomes than RP‐ILD. We report a 41‐year‐old woman diagnosed with dermatomyositis (DM)‐associated ILD positive for anti‐MDA5 and anti‐PL‐7.

BACKGROUND Mobile health (mHealth) technology is an increasingly recognized and effective way for disease control and management, which has the potential to intervene in exacerbation risk, psychology and lung function of chronic obstructive pulmonary disease (COPD) patients. OBJECTIVE This study aimed to investigate a community based closed-loop COPD management system suitable for Chinese remote area with many potential COPD patients but limited medical resources. METHODS A mHealth-based COPD management system is designed and architected and then three methods, COPD Evaluation, Psychological Intervention and Closed-Loop Care Pathway are proposed to evaluate and manage stable COPD patients. Total of 39 patients finished the whole 6 months procedure and were included in the analyses. RESULTS During the study period, 459 exacerbations were detected (Mean 11.8, SD 22.9, 95% CI 4.6 to 19.0), of which 84.5% were dealt with through our system. Thus, both hospitalization and clinic visit due to acute exacerbation per year dropped significantly (p<.001, p=.006 respectively). And psychological condition significantly improved, especially depression condition measured by PHQ-9 scale (p=.003). CONCLUSIONS It is feasible and eligible in area with limited medical resources to implement a mHealth based method for COPD management in community level. After 6-month management, the reduction of hospitalization and clinic visits was statistically significant. Pulmonary function and COPD-specific symptoms were not improved, but psychological distress was significantly reduced, where the assistant of WeChat might play an important role.

Rationale: Hyperimmunoglobulin E syndrome (HIES) is a rare and complex immunoregulatory multisystem disorder characterized by recurrent eczema, skin and sinopulmonary infections, elevated serum immunoglobulin E levels, and eosinophilia. Onset is most likely in childhood, although infrequent adult cases have been reported. Early diagnosis is important. The use of the National Institutes of Health scoring system and the HIES signal transducer and activation of transcription 3 score can standardize the diagnosis of HIES.Patient concerns: A 19-year-old woman presented with complaints of dry cough, pyrexia, dyspnea, and recurrent pneumonia. She had a history of milk allergy, recurrent eczema, suppurative otitis media, chalazia, and aphthous ulcers. Her parents had a consanguineous marriage.