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Seminars in Respiratory and Critical... Jun 2024Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad... (Review)
Review
Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad of myositis, arthritis, and interstitial lung disease (ILD), its presentation can be diverse. Additional common symptoms experienced by patients with ASyS include Raynaud's phenomenon, mechanic's hand, and fever. Although there is a significant overlap with polymyositis and dermatomyositis, the key distinction lies in the presence of antisynthetase antibodies (ASAs). Up to 10 ASAs have been identified to correlate with a presentation of ASyS, each having manifestations that may slightly differ from others. Despite the proposal of three classification criteria to aid diagnosis, the heterogeneous nature of patient presentations poses challenges. ILD confers a significant burden in patients with ASyS, sometimes manifesting in isolation. Notably, ILD is also often the initial presentation of ASyS, requiring pulmonologists to remain vigilant for an accurate diagnosis. This article will comprehensively review the various aspects of ASyS, including disease presentation, diagnosis, management, and clinical course, with a primary focus on its pulmonary manifestations.
Topics: Humans; Myositis; Lung Diseases, Interstitial; Autoantibodies; Diagnosis, Differential
PubMed: 38710221
DOI: 10.1055/s-0044-1785536 -
Rheumatology International Jul 2024Patients with rheumatic diseases (RDs) are prone to a number of comorbidities, particularly those affecting the respiratory system due to inflammatory and autoimmune... (Review)
Review
Patients with rheumatic diseases (RDs) are prone to a number of comorbidities, particularly those affecting the respiratory system due to inflammatory and autoimmune mechanisms. Rheumatoid arthritis (RA), systemic sclerosis (SSc), and inflammatory idiopathic myopathies (IIMs) often present with progressive interstitial lung disease (ILD). The prevalence of ILD varies among patients with RDs, with 11% in RA, 47% in SSc, and 41% in IIMs. Some diagnostic markers, including KL-6, cytokines TNF-α and IL-6, and autoantibodies (anti-CCP), play a crucial role in assessing and predicting the course of pulmonary involvement in RDs. Lung fibrosis is a progressive disorder in SSc and RA, limiting the effiency of therapeutic interventions. Re-evaluating treatment approaches with disease-modifying anti-rheumatic drugs (DMARDs) is crucial for understanding their impact on the risk of lung affections. Despite initial concerns surrounding methotrexate, recent evidence points to its benefits in RA-associated interstitial lung disease (RA-ILD). Recognizing the intricate relationship between autoimmune RDs and lung affections is crucial for formulating effective treatment strategies. Emphasis is placed on collaborative efforts of rheumatologists and pulmonologists for early diagnosis, comprehensive care, and optimal patient outcomes in RA-ILD.
Topics: Humans; Antirheumatic Agents; Arthritis, Rheumatoid; Comorbidity; Lung; Lung Diseases, Interstitial; Rheumatic Diseases; Scleroderma, Systemic
PubMed: 38509351
DOI: 10.1007/s00296-024-05565-w -
Pediatric Pulmonology Mar 2024This review highlights both the longstanding impact of bronchopulmonary dysplasia (BPD) on the health of adult survivors of prematurity and the pressing need for... (Review)
Review
This review highlights both the longstanding impact of bronchopulmonary dysplasia (BPD) on the health of adult survivors of prematurity and the pressing need for prospective, longitudinal studies of this population. Conservatively, there are an estimated 1,000,000 survivors of BPD in the United States alone. Unfortunately, most of the available literature regarding outcomes of lung disease due to prematurity naturally focuses on pediatric patients in early or middle childhood, and the relative amount of literature on adult survivors is scant. As the number of adult survivors of BPD continues to increase, it is essential that both adult and pediatric pulmonologists have a comprehensive understanding of the pathophysiology and underlying disease process, including the molecular signaling pathways and pro-inflammatory modulators that contribute to the pathogenesis of BPD. We summarize the most common presenting symptoms for adults with BPD and identify the critical challenges adult pulmonologists face in managing the care of survivors of prematurity. Specifically, these challenges include the wide variability of the clinical presentation of adult patients, comorbid cardiopulmonary complications, and the paucity of longitudinal data available on these patients. Adult survivors of BPD have even required lung transplantation, indicating the high burden of morbidity that can result from premature birth and subsequent lung injury. In addition, we analyze the disparate symptoms and management approach to adults with "old" BPD versus "new" BPD. The aim of this review is to assist pulmonologists in understanding the underlying pathophysiology of BPD and to improve clinical recognition of this increasingly common pulmonary disease.
Topics: Infant, Newborn; Adult; Female; Child; Humans; Bronchopulmonary Dysplasia; Prospective Studies; Lung; Infant, Premature; Premature Birth; Phenotype
PubMed: 38050796
DOI: 10.1002/ppul.26795 -
Pediatrics Sep 2023Optimizing pulmonary health across the lifespan begins from the earliest stages of childhood and requires a partnership between the family, pulmonologist, and...
Optimizing pulmonary health across the lifespan begins from the earliest stages of childhood and requires a partnership between the family, pulmonologist, and pediatrician to achieve equitable outcomes. The Community Pediatrics session of the Defining and Promoting Pediatric Pulmonary Health workshop weaved together 4 community-based pillars with 4 research principles to set an agenda for future pediatric pulmonary research in optimizing lung and sleep health for children and adolescents. To address diversity, equity, and inclusion, both research proposals and workforce must purposefully include a diverse set of participants that reflects the community served, in addition to embracing nontraditional, community-based sites of care and social determinants of health. To foster inclusive, exploratory, and innovative research, studies must be centered on community priorities, with findings applied to all members of the community, particularly those in historically marginalized and minoritized groups. Research teams should also foster meaningful partnerships with community primary care and family members from study conceptualization. To achieve these goals, implementation and dissemination science should be expanded in pediatric pulmonary research, along with the development of rapid mechanisms to disseminate best practices to community-based clinicians. To build cross-disciplinary collaboration and training, community-academic partnerships, family research partnerships, and integrated research networks are necessary. With research supported by community pillars built on authentic partnerships and guided by inclusive principles, pediatric lung and sleep health can be optimized for all children and adolescents across the full lifespan in the community in which they live and thrive.
Topics: Adolescent; Child; Humans; Family; Pediatricians; Concept Formation; Pediatrics; Lung
PubMed: 37656028
DOI: 10.1542/peds.2023-062292G -
Journal of Personalized Medicine Jul 2023In this observational cohort study, sleep quality in post-COVID-19 patients was assessed using the Pittsburgh Sleep Quality Index (PSQI) questionnaire. This study aimed...
In this observational cohort study, sleep quality in post-COVID-19 patients was assessed using the Pittsburgh Sleep Quality Index (PSQI) questionnaire. This study aimed to examine aspects of sleep quality in patients who have undergone SARS-CoV-2 infection and if there is a pattern of progression or regression over time (6 months). We also observed and analyzed the results in order to identify any possible links between the severity of COVID-19 and sleep quality as measured by the PSQI questionnaire. The study group consisted of 65 adult patients with confirmed SARS-CoV-2 infection who were referred to a pulmonologist for evaluation. Sleep quality was impacted at a high rate in post-COVID-19 patients, quantified by a PSQI score ≥5. Out of 65 patients, 51% of them had scores greater than or equal to 5. Sleep was subjectively reported as unsatisfactory predominantly in mild and moderate COVID-19 patients. According to the PSQI score and a 6-month follow-up, many patients presented persistency in poor sleep quality. Investigation and individualized treatment of sleep disorders in post-COVID-19 patients should be part of the routine pneumological control, as improvement in sleep quality has an impact not only on the health but also on the psychological state of patients. Educating patients about the importance of sleep and sleep quality impairment should be a primary concern.
PubMed: 37511738
DOI: 10.3390/jpm13071125 -
Journal of Thoracic Disease Sep 2023Lung transplantation (LTx) in Japan has taken steps toward increasing the number of donors and recipients and is at the maturity stage of development, at which point... (Review)
Review
BACKGROUND AND OBJECTIVE
Lung transplantation (LTx) in Japan has taken steps toward increasing the number of donors and recipients and is at the maturity stage of development, at which point pulmonologists (hereinafter referred to as "respirologists") become involved in transplant practice. Because of severe donor shortage and limited number of LTx surgeries, most of transplant process from candidacy evaluation to post-operative management has been handled only by thoracic surgeons, which takes away opportunities from respirologists to manage LTx recipients. Given the growth of both LTx and the number of patients with complex problems, cooperation with respirologists in transplant practice is urgently needed to achieve transplant success in Japan.
METHODS
Authors summarized current transplant circumstance in Japan from the transplant physician's standpoint. A systematic search through PubMed database and Google Scholar was performed by terms of "respirologists", "pulmonologist", "lung transplant" or "Japan" from 2000 and 2022. Thoracic surgeons working at each transplant center were asked to complete a questionnaire on physicians' intervention to LTx.
KEY CONTENT AND FINDINGS
The roles of respirologists in LTx differ with facility size and function, depending on whether they are working at a non-transplant center with other respirologists or at a transplant center with transplant physicians. LTx centers are currently devoted to educating respirologists who work at non-transplant or low-volume transplant centers in order for them to deal with patients before and after transplantation.
CONCLUSIONS
Joint efforts and training of outstanding personnel who can take care of recipients are required, this being the greatest issue for the success of transplantation in Japan.
PubMed: 37868896
DOI: 10.21037/jtd-22-1716 -
Respiratory Medicine Aug 2023Cardiopulmonary exercise testing (CPET) remains poorly understood and, consequently, largely underused in respiratory medicine. In addition to a widespread lack of... (Review)
Review
Cardiopulmonary exercise testing (CPET) remains poorly understood and, consequently, largely underused in respiratory medicine. In addition to a widespread lack of knowledge of integrative physiology, several tenets of CPET interpretation have relevant controversies and limitations which should be appropriately recognized. With the intent to provide a roadmap for the pulmonologist to realistically calibrate their expectations towards CPET, a collection of deeply entrenched beliefs is critically discussed. They include a) the actual role of CPET in uncovering the cause(s) of dyspnoea of unknown origin, b) peak O uptake as the key metric of cardiorespiratory capacity, c) the value of low lactate ("anaerobic") threshold to differentiate cardiocirculatory from respiratory causes of exercise limitation, d) the challenges of interpreting heart rate-based indexes of cardiovascular performance, e) the meaning of peak breathing reserve in dyspnoeic patients, f) the merits and drawbacks of measuring operating lung volumes during exercise, g) how best interpret the metrics of gas exchange inefficiency such as the ventilation-CO output relationship, h) when (and why) measurements of arterial blood gases are required, and i) the advantages of recording submaximal dyspnoea "quantity" and "quality". Based on a conceptual framework that links exertional dyspnoea to "excessive" and/or "restrained" breathing, I outline the approaches to CPET performance and interpretation that proved clinically more helpful in each of these scenarios. CPET to answer clinically relevant questions in pulmonology is a largely uncharted research field: I, therefore, finalize by highlighting some lines of inquiry to improve its diagnostic and prognostic yield.
Topics: Humans; Exercise Test; Pulmonary Medicine; Dyspnea; Anaerobic Threshold; Exercise; Exercise Tolerance
PubMed: 37100256
DOI: 10.1016/j.rmed.2023.107249 -
Current Opinion in Pulmonary Medicine Sep 2023Chronic thromboembolic pulmonary hypertension (CTEPH) is a deadly underdiagnosed form of pulmonary hypertension, traditionally treated with surgical extraction of... (Review)
Review
PURPOSE OF REVIEW
Chronic thromboembolic pulmonary hypertension (CTEPH) is a deadly underdiagnosed form of pulmonary hypertension, traditionally treated with surgical extraction of thrombo-fibrotic lesions via pulmonary thrombendarterectomy (PTE) surgery. More recently, treatment options have expanded to pulmonary vasodilator medical therapy and balloon pulmonary angioplasty (BPA). This has led to increased awareness and detection of CTEPH, as well as growing interest in performing PTE and BPA. This review will describe the steps required to build a successful CTEPH team in the context of the rapidly evolving treatment of CTEPH.
RECENT FINDINGS
CTEPH care requires a multidisciplinary team, including a Pulmonologist or Cardiologist expert in Pulmonary Hypertension, a PTE surgeon, a BPA interventionalist, a dedicated radiologist, cardiothoracic anesthesia and Vascular Medicine or Hematology. Careful assessment of precise imaging and hemodynamic data is needed for operability assessment in the context of the experience of the CTEPH team and surgeon. Medical therapy and BPA are indicated for inoperable CTEPH and residual CTEPH after PTE. Increasingly, multimodality approaches, including surgery, BPA and medical therapy are utilized for best outcomes.
SUMMARY
An expert CTEPH center requires a multidisciplinary team with dedicated specialists, and time to develop the experience and expertise to achieve high volumes and good outcomes.
Topics: Humans; Pulmonary Embolism; Hypertension, Pulmonary; Chronic Disease; Angioplasty, Balloon; Endarterectomy; Pulmonary Artery
PubMed: 37395507
DOI: 10.1097/MCP.0000000000000983 -
Journal of Thoracic Disease Sep 2023The long-term survival after lung transplantation (LT) is favorable in Japan. However, long-term survivors after LT are subject to late complications, including chronic... (Review)
Review
The long-term survival after lung transplantation (LT) is favorable in Japan. However, long-term survivors after LT are subject to late complications, including chronic lung allograft dysfunction (CLAD), malignancy, infection, and chronic kidney disease (CKD) because of the need for lifelong immunosuppression. The rates of single cadaveric LT (CLT) and living-donor lobar LT (LDLLT) are higher than that of bilateral CLT in Japan. Here, we will describe the management of late complications and long-term outcome after LT in Japan. Attention should be paid to not only the phenotype of CLAD but also the difference in CLAD after CLT and after LDLLT as well as the timing of lung re-transplantation for advanced CLAD, especially after single CLT. Since post-transplant lymphoproliferative disorder is the most common malignancy after LT, infection monitoring for infection-related malignancies and appropriate screening are keys to the early diagnosis and treatment of malignancy after LT. The long-term management of infection after LT is also important, especially with regard to community-acquired pathogens, Aspergillus, and cytomegalovirus. When providing long-term care after LT, physicians should be aware of CKD and the timing of renal replacement therapy in cases with severe CKD. The widespread use of computed tomography and dialysis in Japan are beneficial for long-term survivors of LT. The similar survival outcomes of single CLT and LDLLT, compared with bilateral CLT, might contribute to improved long-term survival in Japan. Pulmonologists are encouraged to become further involved in long-term management after LT in Japan.
PubMed: 37868843
DOI: 10.21037/jtd-22-1679 -
Pediatric Pulmonology Aug 2023In people with cystic fibrosis (pwCF), the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, such as...
BACKGROUND
In people with cystic fibrosis (pwCF), the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, such as Elexacaftor-Tezacaftor-Ivacaftor (ETI), on structural changes in the lungs is unclear.
OBJECTIVE
To determine the impact of ETI on clinical parameters and on structural lung disease as measured by the changes in the chest computed tomography (CT) scans in pwCF.
METHODS
Percent predicted forced expiratory volume in one second (ppFEV1), body mass index (BMI), and microbiologic data were collected at initiation and 3-month intervals for 1 year. Chest CT scans before starting ETI therapy (baseline) and at 1-year on ETI therapy were compared by two pulmonologists independently.
RESULTS
The sample size was 67 pwCF, 30 (44.8%) males, median age of 25 (16, 33.5) years. Significant increases in ppFEV1 and BMI observed by 3 months of ETI therapy persisted throughout 1 year of ETI therapy (p < 0.001 at all-time points for both). After 1 year on ETI, pwCF had significant reductions in Pseudomonas aeruginosa (-42%) and MRSA (-42%) positivity. None of the pwCF had worsening of chest CT parameters during 1 year of ETI therapy. Comparing chest CT findings at baseline and at 1-year follow-up, bronchiectasis was present in 65 (97%) pwCF and at 1-year follow-up decreased in 7 (11%). Bronchial wall thickening 64 (97%), decreased in 53 (79%). Mucous plugging in 63 (96%), absent in 11 (17%), and decreased in 50 (77%). Hyperinflation/air trapping in 44 (67%), decreased in 11 (18%), absent in 27 (44%) CONCLUSIONS: ETI significantly improved clinical outcomes and lung disease as documented by improvement in chest CT scans.
Topics: Male; Humans; Female; Cystic Fibrosis; Pyrazoles; Cystic Fibrosis Transmembrane Conductance Regulator; Aminophenols; Mutation; Benzodioxoles
PubMed: 37222417
DOI: 10.1002/ppul.26485