Interstitial Lung Disease Mortality Disparities Along the US-Mexico Border, 1999-2020.

BACKGROUND: Optimal diagnosis and management of interstitial lung diseases (ILDs) needs access to specialized centers, frequent monitoring, and complex therapeutic options. In underprivileged areas, these necessities can often lead to barriers in delivering care. RESEARCH QUESTION: What are the ILD mortality disparities in the regions along the US-Mexico (US-MX) border? STUDY DESIGN AND METHODS: We obtained ILD mortality information through death certificate queries from the Centers for Disease Control and Prevention repository. Death data were adjusted for age and stratified by US-MX border regions and nonborder regions in the United States. Log-linear regression models were used to analyze mortality trends in the period from 1999 to 2020 followed by calculation of annual percentage changes (APCs). Age-adjusted mortality rates (AAMRs) were compared across cumulative and subdemographic populations. RESULTS: ILD-related mortality among border regions (AAMR, 5.31) was higher than nonborder regions (AAMR, 4.86). Mortality within border regions remained unchanged from 1999 to 2020 (APC, 0.3; P = .269). Nonborder regions experienced a significant rise in mortality rates (APC, 2.6; P = .017) from 1999 to 2005 and remained unchanged from 2005 to 2020. Mortality was higher within both men (AAMR, 6.57) and women (AAMR, 4.36) populations among border regions compared with their nonborder counterparts (AAMR, 6.27 and 3.87, respectively). Hispanic populations among the border regions experienced higher mortality rates (AAMR, 6.15) than Hispanic populations within nonborder regions (AAMR, 5.44). Non-Hispanic populations encountered similar mortality rates between the two regions. Mortality rates among Hispanic (APC, 0.0; P = .938) and non-Hispanic (APC, 0.2; P = .531) populations in the border regions remained unchanged from 1999 to 2020. INTERPRETATION: These results revealed ILD-related mortality disparities among the US-MX border regions, emphasizing the importance of public health measures to increase access to equitable medical care and implement targeted interventions among these vulnerable populations.

Case Report: Common variable immunodeficiency phenotype and granulomatous-lymphocytic interstitial lung disease with a novel SOCS1 variant.

Common variable immunodeficiency is a heterogeneous symptomatic group of inborn errors of immunity that mainly affects antibodies production and/or function, predisposing patients to recurrent and severe infections. More than half of them usually develop autoimmunity, lymphoproliferation, enteropathy, and malignancies. Among these conditions, chronic lung disease such as granulomatous-lymphocytic interstitial lung disease is one of the leading causes of death in these patients. Recently, many genes that play a key role in B and T cells' development, maintenance, and/or cytokines signaling pathways have been implicated in the pathogenesis of the disease. Here, we describe the first Argentinian patient presenting with common variable immunodeficiency and granulomatous-lymphocytic interstitial lung disease, harboring two in cis heterozygous variants in the SOCS1 gene.

2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc. METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed. RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud's phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found. CONCLUSION: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.

Approach to interstitial lung disease associated with systemic sclerosis-A survey to pulmonologists and rheumatologists in Colombia.

INTRODUCTION: Interstitial lung disease is a leading cause of mortality in patients with systemic sclerosis. Currently, there is a lack of consensus regarding screening, rescreening, diagnosis, and follow-up practices in interstitial lung disease associated with systemic sclerosis (SSc-ILD) in Colombia. METHODS: A structured survey focused on clinical practices in patients with SSc-ILD was conducted. Members of the Asociación Colombiana de Neumología y Cirugía de Tórax (Asoneumocito) and the Asociación Colombiana de Reumatología (Asoreuma) were invited to participate from March 2023 to May 2023. RESULTS: We surveyed 51 pulmonologists and 44 rheumatologists. Overall, 51.6% reported having access to multidisciplinary team discussion in ILD. Among the 95 participants, 78.9% would routinely perform a high-resolution computed tomography scan of the chest once a diagnosis of systemic sclerosis was established. This practice is more frequent among rheumatologists (84.1%) than among pulmonologists (74.5%). Approximately half of the participants would rescreen patients annually with computed tomography scan (56.8%) if baseline images were negative. Spirometry (81.1%), diffusing capacity of the lung for carbon monoxide (80.0%), and 6-min walk test (55.8%) were the most frequently performed tests upon diagnosis of systemic sclerosis. During follow-up, participants would consider repeating pulmonary function tests mostly every 6 months. CONCLUSIONS: Screening of SSc-ILD is high among pulmonologists and rheumatologists. Decision-making on diagnosis and follow-up is similar between specialties, but there are variations in their frequency and indications. Further research is needed to evaluate how to adapt recommendations for assessing SSc-ILD in different settings.

Time trends in the incidence of interstitial lung disease across Brazil, Russia, India, China and South Africa (BRICS) from 1990 to 2019: An age-period-cohort analysis.

BACKGROUND AND OBJECTIVE: The global incidence of interstitial lung disease (ILD) has risen over the past few decades. However, few studies have evaluated the status and incidence trends of ILD in Brazil, Russia, India, China and South Africa (BRICS). This study assesses the trends of ILD incidence across the BRICS with an emphasis on ILD changes from 1990 to 2019. METHODS: Incidence rates were estimated by the data obtained from the Global Burden of Disease Study 2019 (GBD 2019). Age-period-cohort modelling was used to estimate the effects on ILD from 1990 to 2019, and the net drift and local drift were calculated. RESULTS: In 2019, a total of 11.4 million cases of ILD were reported in the BRICS countries. From 1990 to 2019, the incidence rate of ILD in BRICS showed an upward trend. India consistently reported the highest incidence rate, while China showed the fastest growth rate (107.6%). Russia reported a similar incidence rates for men and women, with a lower age of peak incidence compared to the other four countries. We found the time effect was unfavourable for BRICS in the first decade, especially for Brazil; in China and Brazil, the risk of people born after 1960 has rapidly decreased. CONCLUSION: ILD shows a rising incidence in BRICS. with the trends varying based on age and other environmental factors. BRICS should strengthen specific public health approaches and policies for different stages and populations.

Controles de salud durante la pandemia e impacto clínico del COVID-19 en niños con enfermedad neuromuscular / Health checkups during the pandemic and clinical impact of COVID-19 in children with neuromuscular disease

Introducción. Los niños con enfermedad neuromuscular (ENM) requieren cuidados crónicos de salud (CCS) y podrían presentar COVID-19 grave. Objetivos. Describir CCS para niños con ENM durante la pandemia y evolución del COVID-19 en este grupo. Población y métodos. Cohorte prospectiva unicéntrica. Se incluyeron pacientes de 2-18 años, con ≥ 1 año de seguimiento previo a la pandemia. Se recolectaron variables demográficas, relativas a los CCS y al COVID-19 mediante historias clínicas y encuestas telefónicas. Resultados. Se incluyeron 226 pacientes; el 71 % varones, mediana de edad 11,3 años. Presentaban distrofias musculares (55,7 %) y atrofia muscular espinal (23 %). Comparando el primer año de pandemia con el previo, el 30 % no realizó controles médicos y el 25 % no realizó kinesioterapia. Otros disminuyeron la frecuencia. Hubo 52 casos de COVID-19. Fueron sintomáticos el 82 %: el 88,4 % leves/moderados y el 11,6 % graves. No hubo fallecidos. Conclusiones. La pandemia impactó negativamente en los CCS y los casos de COVID-19 fueron mayormente leves.

Introduction. Children with neuromuscular disease (NMD) require chronic health care (CHC) and may develop severe COVID-19. Objectives. To describe CHC for children with NMD during the pandemic and the course of COVID-19 in this group. Population and methods. Prospective, single-center cohort. Patients aged 2 to 18 years with ≥ 1 year of follow-up prior to the pandemic were included. Demographic variables in relation to CHC and COVID-19 were collected from medical records and via telephone surveys. Results. A total of 226 patients with a median age of 11.3 years were included; 71% were males. They had muscular dystrophy (55.7%) and spinal muscular atrophy (23%). When comparing the first year of the pandemic with the previous year, 30% did not have a health checkup and 25% did not receive kinesiotherapy. Others did, but with a lower frequency. A total of 52 COVID-19 cases were reported; 82% were symptomatic: 88.4% were mild/moderate and 11.6%, severe. No patient died. Conclusions. The pandemic had a negative impact on CHC, and COVID-19 cases were mostly mild.

Disfunciones del metabolismo del surfactante pulmonar: caso clínico pediátrico / Pulmonary surfactant metabolism dysfunction: A pediatric clinical case report

Las enfermedades pulmonares intersticiales son patologías poco frecuentes en pediatría. Dentro de ellas, se incluyen las disfunciones del metabolismo del surfactante pulmonar, molécula anfipática cuya función es disminuir la tensión superficial y evitar el colapso alveolar. Se presenta el caso de un lactante de 6 meses, en seguimiento por bajo peso, que presentó dificultad respiratoria aguda y cianosis; la radiografía de tórax evidenció infiltrado intersticial, neumomediastino y neumotórax bilateral. Al interrogatorio, surgió antecedente materno de internación al año de vida, con requerimiento de oxigenoterapia prolongada y diagnóstico desconocido; presenta signos de hipoxia crónica. El paciente cursó internación con requerimiento de oxigenoterapia. Se realizaron estudios complementarios en búsqueda de etiología, sin resultados positivos. La tomografía de tórax evidenció opacidades en vidrio esmerilado, engrosamiento del intersticio septal y áreas de atrapamiento aéreo; con resultado de biopsia pulmonar y estudio genético se llegó al diagnóstico de disfunción del metabolismo del surfactante pulmonar.

Interstitial lung diseases are rare in pediatrics. They include dysfunctions in the metabolism of pulmonary surfactant, an amphipathic molecule that reduces surface tension and prevents alveolar collapse. Here we describe the case of a 6-month-old infant controlled for low weight, who presented with acute respiratory distress and cyanosis; his chest X-ray showed interstitial infiltrate, pneumomediastinum, and bilateral pneumothorax. During history-taking, it was noted that his mother had a history of hospitalization at 1 year old with unknown diagnosis, requiring prolonged oxygen therapy; she now shows signs of chronic hypoxia. The patient was hospitalized and required oxygen therapy. Ancillary tests were done to look for the etiology of the condition, with no positive results. A chest computed tomography showed groundglass opacities, thickening of the septal interstitium, and areas of air trapping; based on the results of a lung biopsy and a genetic study, pulmonary surfactant metabolism dysfunction was diagnosed.

Changes in Lung Function Among Infants Born Extremely Preterm over a 20-Year Period in the Post-Surfactant Era: A Retrospective Study.

Lung function was assessed at 8 years in 308 infants born extremely preterm between 1994 and 2013. Although lung function of those infants born at 22 through 25 weeks remained unchanged, those who were born at 26-27 weeks showed a significant improvement over the past 2 decades.

Association between anti-PL7 antibodies and increased fibrotic component in patients with antisynthetase syndrome and interstitial lung disease: a cross-sectional study.

OBJECTIVE: To evaluate whether anti-PL7 and anti-PL12 autoantibodies are associated with a greater extent of the fibrotic component of ILD in ASSD patients. METHODS: Patients with ILD-ASSD who were positive for one of the following autoantibodies: anti-Jo1, anti-PL7, anti-PL12, and anti-EJ were included. Clinical manifestations, CPK levels, pulmonary function tests, and HCRT assessments were prospectively collected according to the Goh index. The fibrotic, inflammatory, and overall extension of the Goh index and DLCO were assessed by multiple linear analyses and compared between ASSD antibody subgroups. RESULTS: Sixty-six patients were included; 17 were positive for anti-Jo1 (26%), 17 for anti-PL7 (26%), 20 for anti-PL12 (30%), and 9 (14%) for anti-EJ. Patients with anti-PL7 and anti-PL12 had a more extensive fibrotic component than anti-Jo1. Anti-PL7 patients had a 7.9% increase in the fibrotic extension (cß = 7.9; 95% CI 1.863, 13.918), and the strength of the association was not modified after controlling for sex, age, and time of disease evolution (aß = 7.9; 95% CI 0.677, 15.076) and also was associated with an increase in ILD severity after adjusting for the same variables, denoted by a lower DLCO (aß = - 4.47; 95% CI - 8.919 to - 0.015). CONCLUSIONS: Anti-PL7-positive ASSD patients had more extensive fibrosis and severe ILD than the anti-Jo1 subgroup. This information is clinically useful and has significant implications for managing these patients, suggesting the need for early consideration of concurrent immunosuppressive and antifibrotic therapy.

The Value of Ultrasound for Detecting and Following Subclinical Interstitial Lung Disease in Systemic Sclerosis.

BACKGROUND: Interstitial lung disease (ILD) is a complication in patients with systemic sclerosis (SSc). Accurate strategies to identify its presence in early phases are essential. We conducted the study aiming to determine the validity of ultrasound (US) in detecting subclinical ILD in SSc, and to ascertain its potential in determining the disease progression. METHODS: 133 patients without respiratory symptoms and 133 healthy controls were included. Borg scale, Rodnan skin score (RSS), auscultation, chest radiographs, and respiratory function tests (RFT) were performed. A rheumatologist performed the lung US. High-resolution CT (HRCT) was also performed. The patients were followed every 12 weeks for 48 weeks. RESULTS: A total of 79 of 133 patients (59.4%) showed US signs of ILD in contrast to healthy controls (4.8%) (p = 0.0001). Anti-centromere antibodies (p = 0.005) and RSS (p = 0.004) showed an association with ILD. A positive correlation was demonstrated between the US and HRCT findings (p = 0.001). The sensitivity and specificity of US in detecting ILD were 91.2% and 88.6%, respectively. In the follow-up, a total of 30 patients out of 79 (37.9%) who demonstrated US signs of ILD at baseline, showed changes in the ILD score by US. CONCLUSIONS: US showed a high prevalence of subclinical ILD in SSc patients. It proved to be a valid, reliable, and feasible tool to detect ILD in SSc and to monitor disease progression.

Exercise-Induced Pulmonary Hypertension in Long-Term Survivors of Congenital Diaphragmatic Hernia.

OBJECTIVE: To determine the prevalence of exercise-induced pulmonary hypertension (PH) among long-survivors of congenital diaphragmatic hernia repair. STUDY DESIGN: This is a single-center, retrospective cohort study of CDH survivors who underwent exercise stress echocardiography (ESE) at Boston Children's Hospital from January 2006 to June 2020. PH severity was assessed by echocardiogram at baseline and after exercise. Patients were categorized by right ventricular systolic pressure (RVSP) after exercise: Group 1 - no or mild PH; and Group 2 - moderate or severe PH (RVSP ≥ 60 mmHg or ≥ ½ systemic blood pressure). RESULTS: Eighty-four patients with CDH underwent 173 ESE with median age 8.1 (4.8 - 19.1) years at first ESE. Sixty-four patients were classified as Group 1, 11 as Group 2, and 9 had indeterminate RVSP with ESE. Moderate to severe PH after exercise was found in 8 (10%) patients with no or mild PH at rest. Exercise-induced PH was associated with larger CDH defect size, patch repair, use of ECMO, supplemental oxygen at discharge, and higher WHO functional class. Higher VE/VCO2 slope, lower peak oxygen saturation, and lower percent predicted FEV1, and FEV1/FVC ratio were associated with Group 2 classification. ESE changed management in 9/11 Group 2 patients. PH was confirmed in all 5 Group 2 patients undergoing cardiac catheterization after ESE. CONCLUSIONS: Among long-term CDH survivors, 10% had moderate-severe exercise-induced PH on ESE, indicating ongoing pulmonary vascular abnormalities. Further studies are needed to optimally define PH screening and treatment for patients with repaired CDH.

Adaptive multi-interventional trial platform to improve patient care for fibrotic interstitial lung diseases.

BACKGROUND: Fibrotic interstitial lung diseases (fILDs) are a heterogeneous group of lung diseases associated with significant morbidity and mortality. Despite a large increase in the number of clinical trials in the last 10 years, current regulatory-approved management approaches are limited to two therapies that prevent the progression of fibrosis. The drug development pipeline is long and there is an urgent need to accelerate this process. This manuscript introduces the concept and design of an innovative research approach to drug development in fILD: a global Randomised Embedded Multifactorial Adaptive Platform in fILD (REMAP-ILD). METHODS: Description of the REMAP-ILD concept and design: the specific terminology, design characteristics (multifactorial, adaptive features, statistical approach), target population, interventions, outcomes, mission and values, and organisational structure. RESULTS: The target population will be adult patients with fILD, and the primary outcome will be a disease progression model incorporating forced vital capacity and mortality over 12 months. Responsive adaptive randomisation, prespecified thresholds for success and futility will be used to assess the effectiveness and safety of interventions. REMAP-ILD embraces the core values of diversity, equity, and inclusion for patients and researchers, and prioritises an open-science approach to data sharing and dissemination of results. CONCLUSION: By using an innovative and efficient adaptive multi-interventional trial platform design, we aim to accelerate and improve care for patients with fILD. Through worldwide collaboration, novel analytical methodology and pragmatic trial delivery, REMAP-ILD aims to overcome major limitations associated with conventional randomised controlled trial approaches to rapidly improve the care of people living with fILD.

Chronic Obstructive Pulmonary Disease in Heart Failure: Challenges in Diagnosis and Treatment for HFpEF and HFrEF.

PURPOSE OF REVIEW: Chronic obstructive pulmonary disease (COPD) is common in heart failure (HF), and it has a significant impact on the prognosis and quality of life of patients. Additionally, COPD is independently associated with lower adherence to first-line HF therapies. In this review, we outline the challenges of identifying and managing HF with preserved (HFpEF) and reduced (HFrEF) ejection fraction with coexisting COPD. RECENT FINDINGS: Spirometry is necessary for COPD diagnosis and prognosis but is underused in HF. Therefore, misdiagnosis is a concern. Also, disease-modifying drugs for HF and COPD are usually safe but underprescribed when HF and COPD coexist. Patients with HF-COPD are poorly enrolled in clinical trials. Guidelines recommend that HF treatment should be offered regardless of COPD presence, but modern registries show that undertreatment persists. Treatment gaps could be attenuated by ensuring an accurate and earlier COPD diagnosis in patients with HF, clarifying the concerns related to pharmacotherapy safety, and increasing the use of non-pharmacologic treatments. Acknowledging the uncertainties, this review aims to provide key clinical resources to support better physician-patient co-decision-making and improve collaboration between health professionals.

A new proposal for phenotypic classification and outcome assessment of dermatomyositis based on clinical manifestations and serological testing.

BACKGROUND: Dermatomyositis (DM) is an infrequent disease subgroup of idiopathic inflammatory myopathies characterized by distinct skin lesions. However, high heterogeneity makes clinical diagnosis and treatment of DM very challenging. OBJECTIVES: Unsupervised classification in DM patients and analysis of key factors related to clinical outcomes. METHODS: This retrospective study was conducted between 2017 and 2022 at the Department of Rheumatology, Xiangya Hospital, Central South University. 162 DM patients were enrolled for unsupervised hierarchical cluster analysis. In addition, we divided the clinical outcomes of DM patients into four subgroups: withdrawal, stabilization, aggravation, and death, and compared the clinical profiles amongst the subgroups. RESULTS: Out of 162 DM patients, three clusters were defined. Cluster 1 (n = 40) was mainly grouped by patients with prominent muscular involvement and mild Interstitial Lung Disease (ILD). Cluster 2 (n = 72) grouped patients with skin rash, anti-Melanoma Differentiation Associated protein 5 positive (anti-MDA5+), and Rapid Progressive Interstitial Lung Disease (RP-ILD). Cluster 3 (n = 50) grouped patients with the mildest symptoms. The proportion of death increased across the three clusters (cluster 3 < cluster 1 < cluster 2). STUDY LIMITATIONS: The number of cases was limited for the subsequent construction and validation of predictive models. We did not review all skin symptoms or pathological changes in detail. CONCLUSIONS: We reclassified DM into three clusters with different risks for poor outcome based on diverse clinical profiles. Clinical serological testing and cluster analysis are necessary to help clinicians evaluate patients during follow-up and conduct phenotype-based personalized care in DM.

Heart failure worsens leg muscle strength and endurance in coexistence patients with COPD and heart failure reduced ejection fraction.

PURPOSE: Exercise intolerance and dyspnoea are clinical symptoms in both heart failure (HF) reduced ejection fraction (HFrEF) and chronic obstructive pulmonary disease (COPD), which are suggested to be associated with musculoskeletal dysfunction. We tested the hypothesis that HFrEF + COPD patients would present lower muscle strength and greater fatigue compared to compared to the COPD group. METHODS: We included 25 patients with HFrEF + COPD (100% male, age 67.8 ± 6.9) and 25 patients with COPD alone (100% male, age 66.1 ± 9.1). In both groups, COPD severity was determined as moderate-to-severe according to the GOLD classification (FEV1/FVC < 0.7 and predicted post-bronchodilator FEV1 between 30%-80%). Knee flexor-extensor muscle performance (torque, work, power and fatigue) were measured by isokinetic dynamometry in age and sex-matched patients with HFrEF + COPD and COPD alone; Functional capacity was assessed by the cardiopulmonary exercise test, the 6-min walk test (6MWT) and the four-minute step test. RESULTS: The COPD group exhibited reduced lung function compared to the HFrEF + COPD group, as evidenced by lower FEV1/FVC (58.0 ± 4.0 vs. 65.5 ± 13.9; p < 0.0001, respectively) and FEV1 (51.3 ± 17.0 vs. 62.5 ± 17.4; p = 0.026, respectively) values. Regarding musculoskeletal function, the HFrEF + COPD group showed a knee flexor muscles impairment, however this fact was not observed in the knee extensors muscles. Power peak of the knee flexor corrected by muscle mass was significantly correlated with the 6MWT (r = 0.40; p < 0.05), number of steps (r = 0.30; p < 0.05) and work ratepeak (r = 0.40; p < 0.05) in the HFrEF + COPD and COPD groups. CONCLUSION: The presence of HFrEF in patients with COPD worsens muscular weakness when compared to isolated COPD.

Effect of Speaking Valves on Tracheostomy Decannulation.

Introduction Despite several pediatric tracheostomy decannulation protocols there remains tremendous variability in practice. The effect of tracheostomy capping on decannulation has been studied but the role of speaking valves (SVs) is unknown. Objective Given the positive benefits SVs have on rehabilitation, we hypothesized that SVs would decrease time to tracheostomy decannulation. The purpose of the present study was to evaluate this in a subset of patients with chronic lung disease of prematurity (CLD). Methods A retrospective chart review was performed at a tertiary care children's hospital. A total of 105 patients with tracheostomies and CLD were identified. Data collected included demographics, gestational age, congenital cardiac disease, airway surgeries, granulation tissue excisions, SV and capping trials, tracheitis episodes, and clinic visits. Statistics were performed with logistic and linear regression. Results A total of 75 patients were included. The mean gestational age was 27 weeks (standard deviation [SD] = 3.6) and the average birthweight was 1.1 kg (SD = 0.6). The average age at tracheostomy was 122 days (SD = 63). A total of 70.7% of the patients underwent decannulation and the mean time to decannulation (TTD) was 37 months (SD = 19). A total of 77.3% of the patients had SVs. Those with an SV had a longer TTD compared to those without (52 versus 35 months; p = 0.008). Decannulation was increased by 2 months for every increase in the number of hospital presentations for tracheitis ( p = 0.011). Conclusion The present study is the first, to our knowledge, to assess the effect of SVs on tracheostomy decannulation in patients with CLD showing a longer TTD when SVs are used.

Characteristics of Skeletal Muscle Strength in Subjects With Interstitial Lung Disease.

OBJECTIVES: To investigate muscle strength and the prevalence of muscle weakness in adults with interstitial lung diseases (ILDs) compared to healthy subjects. DESIGN: Cross-sectional (description of clinical features). SETTING: Public referral center (University Hospital). PARTICIPANTS: One hundred and twelve adults with ILD (n=48, 60±10yr, 68% female) and healthy counterparts (control group, n=64, 57±10yr, 58% female) (N=112). INTERVENTION: Not applicable. MAIN OUTCOME MEASURE(S): Muscle strength and prevalence of muscle weakness in adults with ILD. Muscle strength was assessed via maximal isometric voluntary contraction of dominant upper and lower limb muscle groups. Data from the control group were used to generate reference equations. Muscle weakness was defined as a muscle strength value below the lower limit of normal calculated using data from the control group. Data were expressed as mean ± SD or median [interquartile range] according to the data distribution. RESULTS: Compared to the control group, adults with ILD had lower muscle strength for all muscle groups assessed (values presented as %predicted: pectoralis major 75[57-86]%; quadriceps 72[58-87]%; latissimus dorsi 76[57-103]%; deltoid 74[64-98]%; biceps brachii 78[64-91]%; triceps brachii 84[62-101]%; P≤.001 for all). Prevalence of muscle weakness in people with ILD was 40% for pectoralis major, 25% for latissimus dorsi, 16% for triceps brachii, 20% for biceps brachii, 27% for deltoid and 46% for quadriceps. CONCLUSIONS: Adults with ILD present a generalised reduction in peripheral Muscle strength, ranging between 20% to 46% of people depending on the muscle group assessed. and it was more prevalent in lower limb muscles.

[Shrinking lung syndrome in systemic lupus erythematosus: A study of 9 patients]. / Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes.

INTRODUCTION: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time. METHODS: A retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected. RESULTS: Out of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable. CONCLUSIONS: SLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment.