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BackgroundNo data on healthcare utilisation and associated costs for the many rare entities of children’s interstitial lung diseases (chILD) exist. This paper portrays healthcare utilisation structures among individuals with chILD, provides a pan-European estimate of a 3-month interval per-capita costs and delineates crucial cost drivers.MethodsBased on longitudinal healthcare resource utilisation pattern of 445 children included in the Kids Lung Register diagnosed with chILD across 10 European countries, we delineated direct medical and non-medical costs of care per 3-month interval. Country-specific utilisation patterns were assessed with a children-tailored modification of the validated FIMA questionnaire and valued by German unit costs. Costs of care and their drivers were subsequently identified via gamma-distributed generalised linear regression models.ResultsDuring the 3 months prior to inclusion into the registry (baseline), the rate of hospital admissions and inpatient days was high. Unadjusted direct medical per capita costs (€19 818) exceeded indirect (€1 907) and direct non-medical costs (€1 125) by far. Country-specific total costs ranged from €8 713 in Italy to €28 788 in Poland. Highest expenses were caused by the disease categories ‘diffuse parenchymal lung disease (DPLD)-diffuse developmental disorders’ (€45 536) and ‘DPLD-unclear in the non-neonate’ (€47 011). During a follow-up time of up to 5 years, direct medical costs dropped, whereas indirect costs and non-medical costs remained stable.ConclusionsThis is the first prospective, longitudinal study analysing healthcare resource utilisation and costs for chILD across different European countries. Our results indicate that chILD is associated with high utilisation of healthcare services, placing a substantial economic burden on health systems.

Interstitial lung abnormalities (ILA) can be incidentally detected in patients undergoing low-dose CT screening for lung cancer. In this retrospective study, we explore the downstream impact of ILA detection on interstitial lung disease (ILD) diagnosis and treatment. Using a targeted approach in a lung cancer screening programme, the rate of de novo ILD diagnosis was 1.5%. The extent of abnormality on CT and severity of lung function impairment, but not symptoms were the most important factors in differentiating ILA from ILD. Disease modifying therapies were commenced in 39% of ILD cases, the majority being antifibrotic therapy for idiopathic pulmonary fibrosis.

BackgroundThe impact of pulmonary rehabilitation (PR) on survival in patients with fibrotic interstitial lung disease (ILD) is unknown. Given the challenges conducting a large randomised controlled trial, we aimed to determine whether improvement in 6-minute walk distance (6MWD) was associated with better survival.MethodsThis retrospective, international cohort study included patients with fibrotic ILD participating in either inpatient or outpatient PR at 12 sites in 5 countries. Multivariable models were used to estimate the association between change in 6MWD and time to death or lung transplantation accounting for clustering by centre and other confounders.Results701 participants (445 men and 256 women) with fibrotic ILD were included. The mean±SD ages of the 196 inpatients and 505 outpatients were 70±11 and 69±12 years, respectively. Baseline/changes in 6MWD were 262±128/55±83 m for inpatients and 358±125/34±65 m for outpatients. Improvement in 6MWD during PR was associated with lower hazard rates for death or lung transplant on adjusted analysis for both inpatient (HR per 10 m 0.94, 95% CI 0.91 to 0.97, pConclusionsPatients with fibrotic ILD who improved physical performance during PR had better survival compared with those who did not improve performance. Confirmation of these hypothesis-generating findings in a randomised controlled trial would be required to definitely change clinical practice, and would further support efforts to improve availability of PR for patients with fibrotic ILD.

BackgroundSome patients with idiopathic interstitial pneumonia (IIP) show autoimmune features. Interstitial pneumonia with autoimmune features (IPAF) was recently proposed as a research concept in these patients. However, retrospective studies reported conflicting results of its prognosis. Therefore, this study was conducted to prospectively evaluate the clinical significance of autoimmune features in patients with IIP.MethodsThis nationwide multicentre study prospectively enrolled consecutive patients with IIP. At the diagnosis, we systematically evaluated 63 features suggestive of connective tissue diseases using a checklist including symptoms/signs and autoantibodies, which contained most items of the IPAF criteria and followed up with the patients. Clinical phenotypes were included in a cluster analysis.ResultsIn 376 patients with IIP enrolled, 70 patients (18.6%) met the IPAF criteria. The proportion of patients with IPAF was significantly lower in idiopathic pulmonary fibrosis (IPF) than in non-IPF (6.0% vs 24.3%, respectively). During a median observation period of 35 months, patients with IPAF more frequently developed systemic autoimmune diseases and had less frequent acute exacerbation of IIPs than patients with non-IPAF. IPAF diagnosis was significantly associated with better survival and was an independent positive prognostic factor in total and patients with non-IPF. Cluster analysis by similarity of clinical phenotypes identified a cluster in which there was a higher number of women, and patients had more autoimmune features and a better prognosis than other clusters.InterpretationThese observations suggest that some patients with IIP show autoimmune features with distinct characteristics and favourable prognosis. However, we were not able to determine the appropriate therapies for these patients.

BackgroundAcute exacerbations of interstitial lung diseases (AE-ILD) have a high mortality rate with no effective medical therapies. Lung transplantation is a potentially life-saving option for patients with AE-ILD, but its role is not well established. The aim of this study is to determine if this therapy during AE-ILD significantly affects post-transplant outcomes in comparison to those transplanted with stable disease.MethodsWe conducted a retrospective study of consecutive patients with AE-ILD admitted to our institution from 2015 to 2018. The comparison group included patients with stable ILD listed for lung transplant during the same period. The primary end-points were in-hospital mortality for patients admitted with AE-ILD and 1-year survival for the transplanted patients.ResultsOf 53 patients admitted for AE-ILD, 28 were treated with medical therapy alone and 25 underwent transplantation. All patients with AE-ILD who underwent transplantation survived to hospital discharge, whereas only 43% of the AE-ILD medically treated did. During the same period, 67 patients with stable ILD underwent transplantation. Survival at 1 year for the transplanted patients was not different for the AE-ILD group versus stable ILD group (96% vs 92.5%). The rates of primary graft dysfunction, post-transplant hospital length-of-stay and acute cellular rejection were similar between the groups.ConclusionPatients with ILD transplanted during AE-ILD had no meaningful difference in overall survival, rate of primary graft dysfunction or acute rejection compared with those transplanted with stable disease. Our results suggest that lung transplantation can be considered as a therapeutic option for selected patients with AE-ILD.

BackgroundPatients with interstitial lung disease (ILD) are at risk of developing nocturnal hypoxaemia due to ventilatory restriction and impaired gas exchange that worsen with supine posture and reduced ventilatory drive during sleep. This systematic review synthesised literature on the diagnostic evaluation, epidemiology, associations, management and prognosis of nocturnal hypoxaemia in ILD.MethodsOvid MEDLINE, Embase and CENTRAL databases were searched for eligible studies. Meta-analyses with subgroup analyses were conducted, where possible.ResultsFifty-three studies were included (total participant number=2590). The most common definition for clinically significant nocturnal hypoxaemia was ≥10% of total sleep time with oxyhaemoglobin saturation ConclusionNocturnal hypoxaemia is common, associated with DLCO impairment and markers suggestive of pulmonary hypertension, and a potential prognostic factor in patients in ILD. There is a need to establish a consensus definition of nocturnal hypoxaemia and evaluate long-term effects of nocturnal supplemental oxygen in ILD.

Learning points A 16-year-old boy with persisting fixed obstructive spirometry is approaching transition to adult services. He has been under long-term follow-up, initially referred aged 2 months by his local team for tachypnoea and mild respiratory distress since birth. He was born at term by vaginal delivery with meconium suctioned from his oropharynx and admitted to a special care baby unit for observation and intravenous antibiotics. Chest X-ray (CXR) at birth was reportedly consistent with possible meconium aspiration, although there was no neonatal resuscitation or ventilation required and he was discharged home after 4 days without an oxygen requirement. Flexible bronchoscopy was arranged that was unremarkable, there was no evidence of aspiration or infection, he had a negative congenital infection screen and normal echocardiogram. CT chest showed linear atelectasis, occasional thickened interlobular septa and areas of secondary pulmonary lobular hyperinflation. Neither bronchopulmonary dysplasia (BPD) nor meconium aspiration syndrome really fits his neonatal history or imaging appearance. He was well in himself and so was discharged with a plan for regular outpatient review. Follow-up CT chest age 3 years (figure 1A) demonstrated persistent abnormality with an …

Herd immunity through extensive and rapid vaccination rather than natural immunity acquired by infection is necessary to control a global pandemic like COVID-19. The development of COVID-19 vaccines has been accelerated through government funding and the collaborative efforts of the medical–scientific institutions and the pharmaceutical industry.1 2 In South Korea, the ChAdOx1 nCoV-19 (Oxford/AstraZeneca) and BNT162b2 (Pfizer/BioNTech) vaccines have received emergency approval and are being used. Although the safety and efficacy of these vaccines were established through interim analysis in global clinical trials, long-term data and reports of rare adverse reactions remain inadequate.1 2 We report a case of interstitial lung disease (ILD) after COVID-19 vaccination and review the literature on influenza vaccine-related ILDs. This review is favourable as influenza vaccines are widely administered annually among the elderly, and several vaccine-related ILDs have been reported. In early April 2021, an 86-year-old man presented to the emergency department with a 1-day history of weakness, dyspnoea and fever. He had no cough, expectoration, nasal discharge or sore throat. One day before symptom onset, he had received a COVID-19 mRNA vaccine. He had hypertension, diabetes and chronic renal disease, which were well controlled with medications (atorvastatin, amlodipine, furosemide, linagliptin, metformin and clopidogrel). He was a non-smoker with no history of cardiovascular, pulmonary, allergic or connective tissue disease (CTD). He took an influenza vaccine annually with no adverse events and had no history of adverse events with other vaccines or drugs. He denied any recent changes in his living environment and exposure to chemicals or organic particles. On admission, his body temperature was 38.2°C, and peripheral oxygen saturation was 80% on room air. He had no rash, oedema or clubbing, but bilateral crackles were found on auscultation. The partial pressure of oxygen in arterial blood (PaO2)/fraction of inspired oxygen ratio (FiO …

The aim of this review is to explain why the term ‘desquamative interstitial pneumonia’ (DIP) should be discarded and replaced with modern terminology. Reason 1: DIP is a misnomer. Within a few years after the term was coined, it was shown that the airspace cells in DIP are macrophages not desquamated pneumocytes. Reason 2: As a result of overly simplistic and poorly defined histologic criteria, DIP is currently a mixed bag of smoking-related diseases and unrelated processes in never-smokers. Reason 3: DIP obfuscates the modern concept that smoking causes some forms of parenchymal lung disease. Despite the fact that >80% of cases of DIP are caused by smoking, it is currently classified as a ‘smoking-related idiopathic interstitial pneumonia’, an oxymoron. Reason 4: The premise that the presence of numerous macrophages within airspaces defines an entity creates problematic histologic overlap with other lung diseases that may feature prominent airspace macrophages. Reason 5: DIP is outdated. It was coined in 1965, when many entities in interstitial lung disease had not been described, smoking-related interstitial lung disease was an unknown concept, computed tomograms of the chest had not been introduced and immunohistochemistry was unavailable. We suggest a way forward, which includes eliminating the term DIP and separating smoking-related lung abnormalities (including accumulation of pigmented airspace macrophages) from cases characterised by numerous non-pigmented macrophages in never-smokers. The laudable goal of smoking cessation is not served well by muddying the relationship between smoking and lung disease with inaccurate, outdated terminology.

A 29-year-old African woman with active adult-onset Still’s disease (AOSD) that developed at the end of 2018 presented to our hospital in September 2019 with pleuritic right-sided pain and respiratory insufficiency of almost a month’s duration, which had failed to respond to high-dose corticosteroid and antibiotic treatment. A thoracic CT revealed right pleural effusion, multiple consolidations and pulmonary collapse. Besides pulmonary symptoms, the patient had arthralgia, fever, a salmon-coloured rash, leucocytosis and rising inflammatory markers. After an AOSD flare diagnosis was established, intravenous methylprednisolone and painkillers were administered. Due to the severity of the pleural effusion and the lack of response to previous treatments, subcutaneous anakinra was started. Two days after the first dose the patient had almost fully recovered. The success of this case points out the potential effectiveness of anakinra in the prevention of a pulmonary failure and the treatment of a severe intermittent polycyclic pattern of AOSD.

IntroductionPulmonary diseases are significant contributors to morbidity and mortality in patients with rheumatoid arthritis (RA). RA-associated interstitial lung disease (RA-ILD) may be prevalent in up to 30% and clinically evident in 10% of patients with RA. Feasible methods to detect concomitant ILD in RA are warranted. Our objective is to determine the diagnostic accuracy of thoracic ultrasound (TUS) for ILD in patients with RA with respiratory symptoms, by using chest high-resolution CT (HRCT) as the reference standard. Further, we aim to evaluate the diagnostic accuracy for the promising blood biomarkers surfactant protein-D and microfibrillar-associated protein 4 in the detection of ILD in this group of patients.Methods and analysisBy use of a standardised 14 zone protocol patients suspected of having RA-ILD will undergo TUS as index test performed by a junior resident in rheumatology (BKS), who is certified by the European Respiratory Society in performing TUS assessments. Participants form a consecutive series of up to 80 individuals in total. The anonymised TUS images will be stored and scored by the junior resident as well as two senior rheumatologists, who have received training in TUS, and a TUS-experienced pulmonologist. HRCT will be used as the gold standard for ILD diagnosis (reference standard). The two basic measures for quantifying the diagnostic test accuracy of the TUS test are the sensitivity and specificity in comparison to the HRCT.Ethics and disseminationData will be collected and stored in the Research Electronic Data Capture database. The study is approved by the Committees on Health Research Ethics and the Danish Data Protection Agency. The project is registered at clinicaltrials.gov (NCT05396469, pre-results) and data will be published in peer-reviewed journals.

Congenital lobar emphysema (CLE)/congenital alveolar overdistension/congenital lobar overinflation or infantile lobar emphysema is a rare developmental anomaly of the lower respiratory tract which is characterised by hyperinflation of one or more of the pulmonary lobes. Histopathology may be variable, which may show abnormality in the cartilage, granulation tissue, mucosal folds, etc. We report a rare underlying histopathology in a preterm neonate with CLE. This entity referred to as pulmonary interstitial glycogenosis (PIG) is a group of heterogeneous lung disease affecting the lung parenchyma and is characterised by the presence of glycogen laden cells in the lung interstitium. This impairs the gas exchange and typically manifest as tachypnoea, retractions, hypoxia and increased respiratory support. Our case report highlights the association of CLE and PIG and a review of literature. One should always have a detailed histopathology in children presenting with CLE to rule out PIG.

Acute fibrinous and organising pneumonia (AFOP) is a rare form of interstitial lung disease. It is a pathological diagnosis sharing similarities to organising pneumonia, diffuse alveolar damage and eosinophilic pneumonia, however, is histologically distinct, characterised by intra-alveolar fibrin deposition (‘fibrin balls’) and associated organising pneumonia. AFOP was first described in 2002, only 150 cases have been reported since. While it has been described in association with infection, autoimmune disorders, connective tissue diseases, drugs, environmental exposures and organ transplant, it can also be idiopathic in nature. AFOP follows an acute course with potential rapid fulminant respiratory failure, or a subacute trajectory with a more favourable prognosis. Corticosteroids are commonly prescribed to induce remission. While cases of relapse of AFOP during weaning or cessation of steroids have been described, there are no published cases of remote relapse of AFOP. We describe a case of idiopathic AFOP, which recurred after 12 years of good health.

A 39-year-old woman presented with a 6-month history of breathlessness, productive cough and painful lumps on her lower legs. She had suffered with recurrent rhinosinusitis and frequent infections of the ears, throat and chest since childhood, as well as cutaneous herpes zoster in her 20s. There was no documented history of pneumonia, invasive or deep-seated infections. She had smoked lightly, was allergic to penicillin and worked as a commercial tea buyer. Examination showed a slim female with a normal breathing pattern. Chest expansion was reduced and fine inspiratory crackles were detected over the lower lung zones. Pulmonary function tests showed ventilatory restriction with decreased gas transfer. Forced expiratory volume in one second (FEV1) 2.10 L, 71% predicted; forced vital capacity (FVC) 2.22 L, 65% predicted; spirometric ratio 0.93; total lung capacity 3.04 L, 62% predicted; haemoglobin (Hb)-adjusted carbon monoxide transfer factor/TLco 45% predicted and its coefficient/Kco 81% predicted. Indices related to small airway function were normal, maximal expiratory flow (MEF)75/50/25 all above 80% predicted and air-trapping was absent. Bloods tests showed normal full blood count, C-reactive protein as well as kidney, bone and liver biochemistry. Serum ACE was elevated at 93 ACEU (ref: 12–68). Elispot assay and HIV serology were negative. A prereferral chest radiograph revealed widening of the superior mediastinum and ill-defined nodular opacities in the middle and lower zones. Despite the history of possible erythema nodosum and raised serum ACE, the account of recurrent infections suggested a differential diagnosis broader than typical sarcoidosis. Demonstration of ventilatory restriction and impaired gas transfer factor hinted towards an interstitial or diffuse lung pathology. The Kco, at nearly twice the TLco in %-predicted terms, indicated a low likelihood of major pulmonary vasculopathy and the absence of airflow limitation or small airway dysfunction steered the diagnosis away from primary bronchiolar disorders. CT with contiguous …

BackgroundFraming bias occurs when people make a decision based on the way the information is presented, as opposed to just on the facts themselves. How the diagnostician sees a problem may be strongly influenced by the way it is framed. Does framing bias result in clinically meaningful diagnostic error?MethodsWe created three hypothetical cases and asked consultants and registrars in Emergency Medicine and Internal Medicine to provide their differential diagnoses and investigations list. Two of the presentations were written two ways to frame the case towards or away from a particular diagnosis (Presentation 2 – pulmonary embolus (PE) and Presentation 3 – interstitial lung disease (ILD)) and these were randomly assigned to the participants. Both versions were however entirely identical in terms of the objective facts. Physician impressions and diagnostic plan were compared. A third presentation was identical for all and served as a control for clinician baseline ‘risk-averseness’.ResultsThere were significant differences in the differential diagnoses generated depending on the presentation’s framing. PE and ILD were considered and investigated for the majority of the time when the presentation was framed towards these diagnoses, and the minority of the time when it was not. This finding was most striking in Presentation 2, where 100%versus50% of clinicians considered PE in their diagnosis when the presentation was framed towards PE. This result remained robust when undertaking stratified analysis and logistic regression to account for differences in seniority and baseline risk-averseness— neither of the latter variables had any effect on the result.ConclusionWe demonstrate a clinically meaningful effect of framing bias on diagnostic error. The strength of our study is focus on clinically meaningful outcomes: investigations ordered. This finding has implications for the way we conduct handovers and teach juniors to communicate clinical information.

Interstitial lung abnormalities, when present in members of undiagnosed family members recruited on the basis of familial interstitial pneumonia, or in undiagnosed research participants, have been associated with a syndrome that includes distinct sets of imaging abnormalities, restrictive physiological and exercise impairments, and an increased prevalence of histopathological findings, and genetic predictors, that have been noted in patients with idiopathic pulmonary fibrosis. Recent longitudinal studies have demonstrated that qualitative and quantitative assessments of interstitial abnormalities are associated with accelerated lung function decline, an increased rate of clinical diagnoses of interstitial lung disease and an increased rate of mortality. In this perspective, in addition to reviewing the prior information, four major efforts that could help the field of early pulmonary fibrosis detection move forward are discussed. These efforts include: (1) developing standards for characterising and reporting imaging findings from patients with existing CTs; (2) developing consensus statements on when undiagnosed and asymptomatic imaging abnormalities should be considered a disease; (3) identifying populations for which screening efforts might be beneficial; and (4) considering approaches to developing effective secondary prevention trials.

A man in his 60s exhibited persistent dry cough and dyspnoea, which persisted even after smoking cessation. Chest high-resolution CT showed diffuse ground-glass opacities in the subpleural areas of both lungs. He underwent bronchoscopy, but no definitive diagnosis could be made. Histopathological analysis of the specimen obtained by surgical lung biopsy showed a desquamative interstitial pneumonia (DIP) pattern, with lymphocyte and plasma cell infiltrates in the alveolar septa; the ratio of IgG and IgG4-positive cells was more than 90%. He quit smoking, but the radiological findings worsened. Based on the pathological findings, we diagnosed the patient with DIP due to IgG4-related lung disease. Prednisolone was initiated, and the symptoms and radiological findings improved.

A middle-aged man was referred to our respiratory department with dyspnoea progressively deteriorating and non-productive cough over the past 8 months. High-resolution CT revealed multiple bilateral consolidations, traction bronchiectasis, reticular pattern and honeycombing with basal and peripheral predominance. Serology tests were negative. Pulmonary function tests showed moderate restrictive functional impairment and severe reduction in diffusing capacity for carbon monoxide. Meticulous evaluation of patient’s medical history revealed recent administration of oral corticosteroid due to pulmonary fibrosis potentially in the context of Fanconi syndrome diagnosed at childhood. The working diagnosis of interstitial lung disease (ILD) as a rare complication of Fanconi syndrome was proposed following multidisciplinary discussion. Despite combination treatment with low doses of corticosteroids and antifibrotic compound, the patient exhibited clinical, radiological and functional deterioration, was admitted to intensive care unit due to respiratory failure following infection-driven progression of fibrotic ILD and finally died.

A man in his 70s presents with 12 months of progressive dyspnoea, sicca symptoms and Raynaud’s phenomenon. Serological testing and tear duct biopsy confirm Sjögren’s syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on high-resolution computed tomography (HRCT), supported by a 40% lymphocytosis on bronchoalveolar lavage.Biopsy of a non-characteristic additional pulmonary nodule diagnoses light chain deposition disease (LCDD). Extrapulmonary organ involvement is excluded. Pulmonary function tests are well-preserved, and the patient is kept under active surveillance without requiring immunomodulatory treatment.LIP and LCDD both have a strong association with SS. Identification of these disease associations is crucial as they may result in multiorgan involvement or progression to haematological malignancy. This is the first case published in the literature and highlights that a pragmatic approach to investigations can avoid unnecessary procedures, and that treatment may be guided by symptomology.

BackgroundInterstitial lung abnormalities (ILA) occur in around 10% of subjects over 60 years, and are associated with a higher rate of all-cause mortality. The pathogenic mechanisms are unclear, and the putative contribution of alterations in the immune response has not been explored. Normal ageing is associated with immune deficiencies, including Naïve T-cell decrease and greater expression of the proliferative-limiting, co-inhibitory receptor killer-cell lectin-like receptor G1 (KLRG1).ObjectiveTo evaluate the frequency and activation state of different T-cell subpopulations in ILA subjects.MethodsPeripheral blood mononuclear cells were obtained from 15 individuals with ILA, 21 age-matched controls and 28 healthy young subjects. T-cells phenotype was characterised by flow cytometry, and proliferation and activation by stimulation with anti-CD3/anti-CD28 or phorbol myristate acetate/ionomycin; KLRG1 isoforms were evaluated by western blot and cytokines were quantified by ELISA and Multiplex.ResultsA significant increase of Naïve CD4+T cells together with a decrease of central and effector memory CD4+T cells was observed in ILA compared with age-matched controls. CD4+T cells from ILA subjects exhibited greater basal proliferation, which raised after anti-CD3/anti-CD28 stimulation. Additionally, a significant increase in the levels of interleukin-6 and interferon gamma was observed in isolated CD4+T cells and plasma of ILA subjects. They also displayed fewer KLRG1+/CD4+T cells with an increase of circulating E-cadherin, the ligand of KLRG1+. No changes were observed with CD8+T cell subsets.ConclusionCD4+T cells from ILA subjects are highly proliferative and show an excessive functional activity, likely related to the loss of KLRG1 expression, which may contribute to an inflammatory state and the development of ILA.

IntroductionRheumatoid arthritis (RA) affects approximately 0.5%–1% of the general population. Clinically significant interstitial lung diseases (ILD) develops in just under 10% of people with RA, and subclinical disease is more common. Little is known about RA-ILD in New Zealand (NZ), or the number of persons with RA in Canterbury, NZ. This study aims to determine: (1) incidence and prevalence of RA, (2) incidence and prevalence of RA-ILD, (3) clinical characteristics and risk factors for the development of RA-ILD, (4) long-term outcomes of RA-ILD, in the population resident within the Canterbury District Health Board (CDHB) catchment area.Methods and analysisPersons aged 18 years of age and older, and resident in the region covered by the CDHB with RA as well as RA-ILD will be identified by retrospective review of medical records. Prevalent as well as incident cases of RA between 1 January 2006 and 31 December 2008 and between 1 January 2011 and 31 December 2013 will be identified, and followed until 30 June 2019. Existing as well as incident cases of RA-ILD during this time will be identified. The association between the development of ILD and clinical characteristics and environmental exposures will be examined using Cox-proportional hazard models. Kaplan-Meier methods will be used to estimate survival rates for patients with RA-ILD. Mortality for people with RA and RA-ILD will also be compared with the general population of the CDHB.Ethics and disseminationData will be obtained by retrospective review of medical records. Deidentified patient data will be stored in a secure online database. Data on individual patients will not be released, and all results will only be published in aggregate. Ethical approval has been obtained from the University of Otago Human Research Ethics Committee (REF HD18/079). Results will be published in peer-reviewed medical journals and presented at conferences.Trial registration numberACTRN12619001310156; Pre-results.

BackgroundThe Living with Pulmonary Fibrosis (L-PF) questionnaire assesses symptoms and quality of life in patients with fibrosing interstitial lung diseases (ILDs). Its Dyspnoea and Cough domains, whose items’ responses are based on a 24-hour recall, have scores ranging from 0 to 100, with higher scores indicating greater symptom severity. We evaluated the ability of these domain scores to detect change and estimated their meaningful change thresholds in patients with progressive fibrosing ILDs.MethodsThe INBUILD trial enrolled subjects with progressive fibrosing ILDs other than idiopathic pulmonary fibrosis. The L-PF questionnaire was completed at baseline and week 52. The responsiveness of the Dyspnoea and Cough scores was evaluated by comparing changes in these scores with 52-week changes in three anchors: forced vital capacity % predicted and two self-reported items, one for global physical health and one for global quality of life. We used a triangulation approach including anchor-based and distribution-based methods to estimate meaningful change thresholds.ResultsThe analyses included 542 subjects with an L-PF Dyspnoea score at baseline and week 52, and 538 subjects with an L-PF Cough score at baseline and week 52. The L-PF Dyspnoea and Cough scores were responsive to change over 52 weeks. Triangulation of anchor-based and distribution-based estimates resulted in meaningful change thresholds of 6 to 7 points for the L-PF Dyspnoea score and 4 to 5 points for the L-PF Cough score to differentiate subjects who were stable or improved from those who deteriorated.ConclusionThese analyses support the responsiveness, one aspect of validity, of the L-PF Dyspnoea and Cough domains scores as measures of symptom severity in patients with progressive fibrosing ILDs. Estimates for meaningful change thresholds in these domain scores may be of value in interpreting the effects of interventions in these patients.Trial registration numberNCT02999178.

BACKGROUND: Peripheral blood leukocyte telomere length (PBL-TL) is associated with outcomes in idiopathic pulmonary fibrosis patients. Whether PBL-TL is associated with progression of systemic sclerosis-associated interstitial lung disease (SSc-ILD) is unknown. METHODS: A retrospective observational cohort study was performed using prospectively collected data from 213 SSc patients followed at the University of California San Francisco (UCSF) Scleroderma Center. PBL-TL was measured by qPCR of DNA isolated from peripheral blood. Associations between PBL-TL and PFT trends in patients with SSc-ILD were assessed by longitudinal analysis using Generalized Linear Mixed Models. Findings were validated in a cohort of 61 SSc-ILD patients enrolled in the Stanford University (SU) Scleroderma Center database. RESULTS: UCSF SSc patients with ILD were found to have shorter PBL-TL compared to those without ILD (6554± 671 base pairs (bp) vs 6782 ± 698 bp, p=0.01). Shorter PBL-TL was associated with the presence of ILD (adjusted odds ratio [OR] 2.1 per 1000 bp TL decrease, 95%CI [1.25–3.70], p=0.006). PBL-TL was shorter in SSc-ILD patients lacking SSc-specific autoantibodies compared to seropositive subjects (6237± 647 bp vs 6651± 653 bp, p=0.004). Shorter PBL-TL was associated with increased risk for lung function deterioration with an average of 67 ml greater loss in FVC per year for every 1000 bp decrease in PBL-TL in the combined SSc-ILD cohorts (longitudinal analysis, adjusted model: 95% CI −104ml to −33ml, p

Drug-induced interstitial lung disease (DILD) with an estimated incidence of approximately 4.1–12.4 cases/million/year is implicated in ~5% of ILD cases.1 Data vary by country, with DILD more commonly diagnosed in Japan, perhaps due to higher reporting.2 The Common Terminology Criteria for Adverse Events (CTCAE) scale3 (table 1) helps quantify severity and treatment includes cessation of the drug and, in more severe cases, corticosteroid use. At least 350 drugs have been implicated in causing lung toxicity, or pneumonitis, across a spectrum from mild radiological infiltrates to life-threatening respiratory failure.4 This heterogeneity of presentation and lack of diagnostic standards, with rechallenge to confirm toxicity rarely justified, makes DILD difficult to identify, even at individual patient level.4 The result is a heavy reliance on databases such as pneumotox5 that collate evidence from the literature, often case reports or small series, but with no large-scale assessment. View this table: Table 1 Common Terminology Criteria for Adverse Events (CTCAE), V.5.0 Jo et al 6 take a different approach using a large, nationally representative dataset of hospitalised patients in Japan to retrospectively identify patients that had developed DILD, severe enough to warrant corticosteroid therapy, after receiving a ‘risk drug’ from one of 42 categories associated with lung toxicity. We applaud the methodology used in this study which identified 2342 cases of DILD out of ~42 million hospital admissions (0.0056%). For each case the authors selected four controls matched for known DILD risk factors (primary diagnosis, …

ObjectiveTo assess survival and identify predictors of survival in patients with systemic sclerosis-interstitial lung disease (SSc-ILD) who participated in the Scleroderma Lung Studies (SLS) I and II.MethodsSLS I randomised 158 patients with SSc-ILD to 1 year of oral cyclophosphamide (CYC) vs placebo. SLS II randomised 142 patients to 1 year of oral CYC followed by 1 year of placebo vs 2 years of mycophenolate mofetil. Counting process Cox proportional hazard modelling identified variables associated with long-term mortality in SLS I and II. Internal validation was performed using joint modelling.ResultsAfter a median follow-up of 8 years, 42% of SLS I patients died, and when known the cause of death was most often attributable to SSc. There was no significant difference in the time to death between treatment arms in SLS I or II. Higher baseline skin score, older age, and a decline in the forced vital capacity (FVC) and the diffusing capacity for carbon monoxide (DLCO) over 2 years were independently associated with an increased risk of mortality in SLS I. The Cox model identified the same mortality predictor variables using the SLS II data.ConclusionIn addition to identifying traditional mortality risk factors in SSc (skin score, age), this study demonstrated that a decline in FVC and DLCO over 2 years was a better predictor of mortality than baseline FVC and DLCO. These findings suggest that short-term changes in surrogate measures of SSc-ILD progression may have important effects on long-term outcomes.

Clinical introductionA 76-year-old man with 50 years of smoking history was diagnosed in 2012 with diffuse interstitial lung disease, with radiological data of usual interstitial pneumonia. He came to the emergency room presenting with progression of dyspnoea for 1 week, concomitant with loss of 5 kg of weight, anorexia and poor general condition. He had tachypnoea at rest (30 breaths/min), peripheral cyanosis, speech interfered by cough and breathlessness, baseline oxygen saturation 90%, heart rate 40 beats/min and blood pressure 130/70 mm Hg. Chest X-ray was performed and there was basal atelectasia of the right lung. ECG and urgent echocardiogram (transthoracic echocardiogram, TTE) were also performed (figure 1).QuestionWhich of the following best explains the patient’s situation?Figure 1(A) ECG. (B) Long-axis parasternal view from the transthoracic echocardiogram (TTE). (C) Four-chamber view from the TTE.Acute pulmonary embolismNon-ST elevation myocardial infarctionCardiac lymphomaCardiac myxomaMobitz type II AV block

Dr Andrew W Creamer (AWC), Respiratory Registrar : A 70-year-old retired teacher was referred to the respiratory clinic with a 12-month history of progressive exertional dyspnoea and dry cough. He denied haemoptysis, constitutional symptoms or diurnal variation. He took omeprazole, amlodipine and ezetimibe for a long-standing history of dyspepsia, hypertension and hypercholesterolaemia. He was an ex-smoker with a 30 pack-year history. He reported monophasic colour change in his fingers in the cold, but denied any other connective tissue disease (CTD) symptoms. He denied any significant environmental exposures such as organic dusts or moulds. His vital signs were normal. There was evidence of mechanic’s hands (a hyperkeratotic eruption with fissuring and cracking on the palmar and radial aspects of the fingers, strongly associated with idiopathic inflammatory myopathies) but no other features of CTD or clubbing. Bibasal fine inspiratory crepitations were audible on chest auscultation. Cardiovascular examination was normal. Initial investigations revealed pancytopenia (figure 1) with unremarkable liver and renal function, prompting referral to local haematology services for further assessment. Figure 1 Baseline blood tests at presentation. Abnormal values are highlighted in bold. Pancytopenia was evident, in the context of normal B12, folate and ferritin. Lung function testing revealed a restrictive pattern with significantly reduced gas transfer (figure 2), while 6 min walk test demonstrated baseline saturations of 95% on air dropping to 77% on exertion, achieving 260 m (48% of theoretical distance). Figure 2 Lung function tests at baseline and 4-month follow-up. Spirometry demonstrated a restrictive pattern with reduction in gas transfer factor. KCO, carbon monoxide transfer coefficient; TLCO, transfer factor for carbon monoxide. Chest X-ray showed prominent interstitial lung markings in the mid and lower zones (figure 3). High-resolution CT thorax (HRCT) demonstrated centrilobular emphysema throughout the lungs and a basally predominant interstitial process with features suggestive of non-specific interstitial pneumonia (NSIP) (figure …

IntroductionClinicians and policymakers are promoting widespread use of home technology including spirometry to detect disease progression for patients with interstitial lung disease (ILD); the COVID-19 pandemic has accelerated this. Data collating clinicians’ views on the potential utility of telehealth in ILD are limited.AimThis survey investigated clinicians’ opinions about contemporary methods and practices used to monitor disease progression in patients with ILD using telehealth.MethodsClinicians were invited to participate in a cross-sectional survey (SurveyMonkey) of 13 questions designed by an expert panel. Telehealth was defined as home monitoring of symptoms and physiological parameters with regular automatic transmission of data from the patient’s home to the clinician. Data are presented as percentages of respondents.ResultsA total of 207 clinicians from 23 countries participated in the survey. A minority (81, 39%) reported using telehealth. 50% (n=41) of these respondents completed a further question about the effectiveness of telehealth. A majority of respondents (32, 70%) rated it to be quite or more effective than face-to-face visit. There were a greater number of respondents using telehealth from Europe (94, 45%) than Asia (51, 25%) and America (24%). Clinicians reported the most useful telehealth monitoring technologies as smartphone apps (59%) and wearable sensors (30%). Telehealth was most frequently used for monitoring disease progression (70%), quality of life (63%), medication use (63%) and reducing the need for in-person visits (63%). Clinicians most often monitored symptoms (93%), oxygen saturation (74%) and physical activity (72%). The equipment perceived to be most effective were spirometers (43%) and pulse oximeters (33%). The primary barriers to clinicians’ participation in telehealth were organisational structure (80%), technical challenges (63%) and lack of time and/or workload (63%). Clinicians considered patients’ barriers to participation might include lack of awareness (76%), lack of knowledge using smartphones (60%) and lack of confidence in telehealth (56%).ConclusionThe ILD clinicians completing this survey who used telehealth to monitor patients (n=81) supported its’ clinical utility. Our findings emphasise the need for robust research in telehealth as a mode for the delivery of cost-effective healthcare services in ILD and highlight the need to assess patients’ perspectives to improve telehealth utility in patients with ILD.

Face masks are a first-line defence against the COVID-19 pandemic.1 Concerns about face masks reducing oxygen saturation (SpO2) have been negated by controlled studies in healthy individuals.2 3 Universal masking must be followed without exceptions. However, persons with pre-existing cardiorespiratory diseases like interstitial lung diseases (ILD) have limited functional reserves. We explored the effects of face masks in patients with connective tissue disease (CTD)-associated ILD (CTD-ILD). Patients between 18 and 75 years of age with high-resolution CT (HRCT)-proven CTD-ILD and with Medical Research Council scale grades 1–3 dyspnoea and forced vital capacity (FVC) of less than 70% were included in this crossover trial. If FVC was >70%, patients were included only if the extent of involvement on HRCT was more than 20% of the lung. Patients requiring oxygen supplementation and with myositis or lower limb pain were excluded. Initial SpO2 was recorded and a standard 6 min walk test (6MWT) was carried out with and then without a standard three-layer surgical face mask. Adequate precautions were taken to prevent the spread of COVID-19 during the tests. Distance covered during the 6MWT was the primary outcome measure. The secondary outcome measures …

A 70-year-old man was referred to our respiratory department with non-productive cough over the past 6 months. High-resolution CT revealed reticular pattern with basal and peripheral predominance, centrilobular nodules and mild ground glass opacities. Serology tests were normal and bronchoalveolar lavage revealed lymphocytosis. Pulmonary function tests showed functional impairment and reduced diffusing capacity for carbon monoxide. Meticulous evaluation of patient’s medical history unveiled longitudinal administration of infliximab due to diagnosis of psoriasis. The working diagnosis of drug-induced interstitial lung disease was proposed following multidisciplinary discussion. Considerable radiological and functional improvement was determined 6 months following infliximab discontinuation without implementation of corticosteroids. To this end, the patient has reported remission of cough and functional improvement.

A 75-year-old man was admitted to our hospital with progressive dyspnoea on effort and cough. Three months before presentation, he had undergone percutaneous coronary intervention for unstable angina. At that time, he began taking aspirin (100 mg daily) and clopidogrel (75 mg daily). He had no

The echocardiogram of a 62-year-old woman with a medical history of anti-synthetase syndrome associated with interstitial lung disease (ILD) suggested pulmonary hypertension. An etiological evaluation of pulmonary hypertension was performed. The ventilation/perfusion scan had the aspect of perfusion defects with dissociation. The CT scan showed features of active ILD (basal and subpleural pulmonary infiltration with regular septal line thickening and ground glass-like opacities). After multidisciplinary discussion, the pulmonary hypertension was considered to be secondary to either chronic thromboembolic pulmonary hypertension (CTEPH) and/or ILD. It was decided (i) to initiate an oral anticoagulant (rivaroxaban) and (ii) to initiate intravenous cyclophosphamide plus oral corticosteroids. One year later, while the patient was still taking rivaroxaban, right heart catheterisation showed the persistence of pre-capillary pulmonary hypertension with …

We report a complex case of a 66-year-old female patient with a diagnosis of interstitial lung disease (ILD) that was later correctly identified as an antisynthetase syndrome. This was only diagnosed after an episode of sustained ventricular tachycardia secondary to myocarditis. In this case report, we focus on the clinical features of this rare autoimmune condition and aim to provide useful tips to both general medical professionals and cardiologists to achieve correct differential diagnosis according to the updated international guidelines and recommendations. Early diagnosis is especially important due to the possible arrhythmogenic complications and the high mortality and morbidity associated with ILD and cardiac abnormalities.

ObjectiveTo clarify the risk of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) related to anti-cyclic citrullinated peptide (CCP) antibody.Eligibility criteriaPatients with RA with and without ILD were eligible. The primary outcome was the prevalence or incidence of ILD. Primary studies of any design aside from a case report were eligible.Information sourcesMedline, EMBASE, Science Citation Index Expanded and Cochrane Central Register of Controlled Trials were searched from the inception through 12 November 2019.Data extraction and risk of biasTwo reviewers independently selected eligible reports, extracted relevant data and assessed risk of bias using a modified Quality in Prognostic Studies tool.Data synthesisMeta-analysis was conducted using a random-effects model.Quality of evidenceThe Grades of Recommendation, Assessment, Development and Evaluation system was applied.ResultsAmong 29 out of 827 records retrieved through electronic databases and four additional reports identified from other sources, 29 studies were focused for the review. A total of 10158 subjects were included and the mean age at inclusion was between 45.8 and 63.9 years. The mean RA duration was between 4.3 and 14.9 years. The positivity of anti-CCP antibody ranged from 50.7% to 95.8%. All studies except for two were deemed as high risk of bias. A pooled analysis of univariate results demonstrated that the presence of anti-CCP antibody was significantly associated with RA-ILD with an OR of 2.10 (95% CI: 1.59 to 2.78). Similarly, the titre of anti-CCP antibody was significantly higher for RA-ILD with a standardised mean difference of 0.42 (95% CI: 0.20 to 0.65). These results were confirmed by multivariate analysis in the majority of studies and consistent by any subgroup and sensitivity analyses.ConclusionThe presence and higher titres of anti-CCP antibody were suggested to be significantly associated with an increased risk of RA-ILD. However, the quality of evidence was rated as low or very low.

Organising pneumonia (OP) is an interstitial lung disease characterised by granulation tissues in alveoli and alveolar ducts. Typical imaging findings are migratory airspace opacities with peripheral or peribronchovascular distribution. Diffuse micronodular OP (MNOP) is a rare imaging manifestation, which has imaging differential diagnosis of endobronchial infection such as tuberculosis, hypersensitivity pneumonitis and respiratory bronchiolitis. Although clinical and ancillary radiological findings may aid in refining the differential diagnosis, histopathological assessment is frequently required for this rare presentation due to implications of treatment and prognosis. We report a case of MNOP and performed a review of the literature.

Polyangiitis overlap syndrome (POS) is a diagnostic term coined by Leavitt and Fauci that characterises patients with overlapping features of more than one vasculitis. Prior case studies of antineutrophil cytoplasmic antibodies (ANCA)-associated POS have only been published in patients with eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis alongside proteinase-3/cytoplasmic (C)-ANCA positivity. We present a case of a 60-year-old woman with dyspnoea, hemoptysis, positive perinuclear-ANCA and renal biopsy demonstrating evidence of microscopic polyangiitis. In addition, our patient also had asthma, mononeuritis multiplex, eosinophilia and migratory pulmonary infiltrates, thus fulfilling the criteria for EGPA. This novel case report suggests that POS is not limited to C-ANCA positivity and has variable presentations.

About 10% of term neonates present with respiratory distress at birth. The most common aetiologies include transient tachypnoea of the newborn, pneumonia and meconium aspiration syndrome (MAS). Hyaline membrane disease (HMD) in a term infant occurs either as primary HMD, secondary surfactant deficiency or congenital surfactant dysfunction. A detailed history supported with appropriate radiological and laboratory investigations can help a clinician reach a diagnosis. We report a case of surfactant dysfunction disorder which presented as severe MAS and persistent pulmonary hypertension of the newborn. In the infant described, the significant history of a sibling death with severe neonatal respiratory disease led us to think of diffuse developmental lung diseases especially surfactant dysfunction syndromes. Exome sequencing detected a heterozygous missense variation in exon 21 of the ATP binding cassette protein member 3 (ABCA3) gene. Based on the clinical picture supported with the exome sequencing, a diagnosis of surfactant dysfunction disorder (ABCA3 deficiency) was confirmed.

Background Adults with interstitial lung disease (ILD) often have serologic evidence of autoimmunity of uncertain significance without overt autoimmune disease. We examined associations of rheumatoid arthritis (RA)-associated antibodies with subclinical ILD in community-dwelling adults. Methods We measured serum rheumatoid factor (RF) and anticyclic citrullinated peptide antibody (anti-CCP) and high attenuation areas (HAAs; CT attenuation values between −600 and −250 Hounsfield units) on cardiac CT in 6736 community-dwelling US adults enrolled in the Multi-Ethnic Study of Atherosclerosis. We measured interstitial lung abnormalities (ILAs) in 2907 full-lung CTs at 9.5-year median follow-up. We used generalised linear and additive models to examine associations between autoantibodies and both HAA and ILA, and tested for effect modification by smoking. Results In adjusted models, HAA increased by 0.49% (95% CI 0.11% to 0.86%) per doubling of RF IgM and by 0.95% (95% CI 0.50% to 1.40%) per RF IgA doubling. ILA prevalence increased by 11% (95% CI 3% to 20%) per RF IgA doubling. Smoking modified the associations of both RF IgM and anti-CCP with both HAA and ILA (interaction p values varied from 0.01 to 0.09). Among ever smokers, HAA increased by 0.81% (95% CI 0.33% to 1.30%) and ILA prevalence increased by 14% (95% CI 5% to 24%,) per RF IgM doubling; and HAA increased by 1.31% (95% CI 0.45% to 2.18%) and ILA prevalence increased by 13% (95% CI 2% to 24%) per anti-CCP doubling. Among never smokers, no meaningful associations were detected. Conclusions RA-related autoimmunity is associated with both quantitative and qualitative subclinical ILD phenotypes on CT, particularly among ever smokers.

Rationale Advanced fibrosing interstitial lung disease (ILD) is often progressive and associated with a high burden of symptoms and poor prognosis. Little is known about the symptom prevalence and access to palliative care services at end of life (EOL). Objectives Compare prevalence of symptoms and palliative treatments between patients dying with oxygen-dependent ILD and patients dying of lung cancer. Methods Nationwide registry-based cohort study of patients with oxygen-dependent ILD and patients with lung cancer who died between 1 January 2011 and 14 October 2013. Prevalence of symptoms and treatments during the last seven days of life were compared using data in Swedish Registry of Palliative Care. Measurements and main results 285 patients with ILD and 10 822 with lung cancer were included. In ILD, death was more likely to be 'unexpected' (15% vs 4%), less likely to occur in a palliative care setting (17% vs 40%) and EOL discussions with the patients (41% vs 59%) were less common than in lung cancer. Patients with ILD suffered more from breathlessness (75% vs 42%) while patients with lung cancer had more pain (51% vs 73%) (p

Melanoma differentiation-associated gene 5 (MDA5) antibody, also known as anti-CADM140 antibody is recognised to be associated with rapidly progressive interstitial lung disease, which can be fatal within 3 months. It is also known to be associated with amyopathic dermatomyositis. We report a case of MDA5 antibody-associated interstitial pneumonia with autoimmune features, without cutaneous features of dermatomyositis, in a Sudanese patient with dual positive antibodies to Ro52. The patient notably had several features associated with poor prognosis, including age, high serum ferritin level, anti-Ro52 antibodies and progressive lung infiltrates during treatment.

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin with high rates of local recurrence and distant metastases despite treatment with traditional cytotoxic chemotherapies. The recent advances in immunotherapy, including the use of immune checkpoint blockade (ICB) has revolutionized treatment for this disease and resulted in durable responses for some patients. However, many patients, due to underlying conditions, have been insufficiently evaluated for potential use of immunotherapy. Here we present a case of ICB treatment with Programmed cell death protein 1 (PD-1) inhibition in a patient with underlying interstitial lung disease (ILD) and a new diagnosis of MCC. Through a multidisciplinary approach, we were able to maintain close monitoring with serial clinical and radiographical follow-up. The patient achieved a complete response though unrelated medical issues resulting in a treatment hold. At the last follow-up, the patient continued to experience a durable response without evidence of recurrence. This case describes the use of pembrolizumab, a PD-1 inhibitor, for the treatment of MCC in a patient with underlying ILD. The use of active surveillance with a multidisciplinary approach resulted in successful treatment of MCC without exacerbation of the underlying ILD.

A 59-year-old Caucasian woman presented with a 3-month history of worsening dyspnoea and productive cough with clear mucoid sputum. She also had episodic chills, night sweats and an unintentional 11 kg weight loss. She denied any fever, haemoptysis, recent travel or sick contacts. Her history was

Systemic lupus erythematosus is a multisystem autoimmune disease with wide-ranging pleuropulmonary manifestations. Acute lupus pneumonitis is one of its uncommon complications. We report a 36-year-old woman with acute lupus pneumonitis as the initial presentation of systemic lupus erythematosus. Clinical, biological, radiological and functional improvements were noticed with the administration of steroids, hydroxychloroquine and immunoglobulin.

We welcome the comments by Alunno et al on our article about the association between anticarbamylated protein antibody (anti-CarP) specificities and rheumatoid arthritis-associated interstitial lung disease (RA-ILD).1 The authors proposed a ‘mix and match approach’ consisting of the assessment of various antibody specificities at RA diagnosis with the aim of predicting the development of ILD.2 We believe this hypothesis is reasonable, considering that different antimodified protein antibodies (AMPA) including anti-CarP, anticitrullinated and antiacetylated protein antibodies (ACPA and anti-AceP, respectively) have been associated with RA-ILD.1 3 4 Furthermore, a …

We read an interesting study by Spielmann et al conducted on a single-centre large French cohort, which identified that anti-Ku autoantibodies were effective biomarkers for two distinct connective tissue diseases (CTDs): anti-Ku-positive patients with elevated serum creatine kinase (CK) levels had a high risk for developing interstitial lung diseases (ILD), while anti-Ku-positive patients with anti-double-strand DNA were at high risk for developing glomerulonephritis.1 Anti-Ku autoantibodies are associated with various CTDs, such as systemic lupus erythematosus, systemic sclerosis, idiopathic inflammatory myopathies (IIM), mixed CTDs, Sjogren’s syndrome, and rheumatoid arthritis. However, few studies have focused on the distinguishing features, especially the pathological features of IIM patients with isolated anti-Ku and anti-Ku coexistence with myositis-specific autoantibodies (MSA). Here, we retrospectively investigated the characteristics of 1214 IIM patients with anti-Ku autoantibodies, all fulfilling the Bohan & Peter criteria for IIM and admitted to the Department of Rheumatology at China-Japan Friendship Hospital from January 2008 to July 2019. Anti-Ku autoantibodies were detected by line immunoassay (EUROLINE, Germany) and ELISA assay …

We read with interest the report of a series of 61 patients with systemic juvenile idiopathic arthritis (s-JIA) or s-JIA-like associated with high-fatality lung diseases.1 Lung disease was associated with digital clubbing, a high frequency of anaphylactic reactions to tocilizumab, and macrophage activation syndrome (MAS). Because of the low 5-year survival probability of 42%, there is an urgent need to identify efficient drugs to treat such patients. Herein we report the effectiveness and safety of ruxolitinib in one patient demonstrating clinical and radiological characteristics consistent to those reported by Saper et al A 2-year-old girl born to non-consanguineous parents presented with s-JIA-like since the age of 12 months, including recurring episodes of unexplained fever, urticaria, arthralgia, poor general health status, leukocytosis and elevated serum C-reactive protein (CRP). The use of corticosteroids resulted in a complete remission but the patient relapsed when prednisone was tapered below 0.5 mg/kg/day. Owing to this corticosteroid dependence, the patient received several lines of biological agents from the …