Background:Individuals with chronic obstructive pulmonary disease (COPD) often face direct and indirect medical costs from unplanned emergency department visits and hospitalizations for acute exacerbations, out-of-pocket expenses for inhaled bronchodilators, and income loss from disability. Yet financial toxicity, which describes the objective burden and subjective distress resulting from medical costs, has not been studied in COPD. Individual experiences of financial toxicity in COPD offer insight into challenges that may be unique to this population. Methods:We conducted semistructured interviews with 30 purposively sampled individuals with physician-diagnosed COPD. Transcripts were analyzed using inductive coding by 2 independent coders, and codes and were categorized through thematic analysis. Results:Thirty participants completed semistructured interviews, of whom 56% were women, 43% non-Hispanic White, and 43% non-Hispanic Black. The mean age was 69.5 years, and 24 (70%) had public health insurance only. Several themes emerged including: (1) the sources of material burden in COPD; (2) adjustments to disease management, such as medication nonadherence or foregoing treatments; (3) adjustments to financial planning, including both changes to day-to-day spending and disruptions in major financial plans; (4) emotional impact; and (5) communication with health care providers. Conclusion:Our findings are the first, to our knowledge, to describe the impact of financial toxicity in individuals with COPD. Financial toxicity in COPD is common and may adversely impact disease self-management, financial self-management, and psychological well-being. Additional research is needed to examine its impact on patient-reported outcomes and to develop interventions to reduce its burden.

RATIONALE: Current American Thoracic Society (ATS) guidelines for the diagnosis of sarcoidosis outline three major criteria including the histologic finding of non-caseating granulomas, a compatible clinical presentation, and the exclusion of alternative causes of granulomatous disease. Studies examining clinical outcomes in sarcoidosis have largely focused on populations with biopsy-proven sarcoidosis enrolled at Sarcoidosis Centers. However, it is unknown whether these criteria are used outside of Sarcoidosis Centers prior to labeling patients with a diagnosis of sarcoidosis. This study examines the number of diagnostic criteria satisfied in patients with an ICD diagnosis of sarcoidosis managed by non-sarcoidosis experts in a single-center retrospective cohort. METHODS: We reviewed data in the electronic medical record (EMR) of all adult patients (>18 years) with a single ICD-10 billing or encounter diagnosis for sarcoidosis between March 2020 and October 2020 at a large midwestern tertiary care center. Clinical evidence to support a diagnosis of sarcoidosis was defined by the ATS clinical practice guideline on the diagnosis and detection of sarcoidosis. Acid-fast bacilli (AFB) staining was used as a surrogate for evaluation of alternative causes of granulomatous disease. Lung involvement was defined by Scadding stage and categorized as unknown if no chest imaging was available for review. RESULTS: A total of 83 patients were identified with an ICD-10 diagnosis of sarcoidosis during the study period. In this cohort, 49.4% (n=41) of patients had a biopsy to support the diagnosis (Table 1). Of the patients with a biopsy supporting the diagnosis, 41.5% (n=17) had documented acid-fast bacilli (AFB) staining. Of the patients who did not have a biopsy supporting the diagnosis, 61.9% (n=26) had no clinical features determined by ATS to suggest a high probability of sarcoidosis. A majority of patients in the cohort had Scadding stage 0 or I disease (n=61, 73.5%), and 19.3% (n=16) of patients in the cohort had no chest imaging available for review. CONCLUSIONS: Our study demonstrates variability in the thresholds of evidence that providers outside of a Sarcoidosis Center use to make a diagnosis of sarcoidosis. Notably, 31.3% of patients in our cohort were labeled as having sarcoidosis without a biopsy or clinical features to suggest the diagnosis. This inconsistent use of society guidelines in diagnosing sarcoidosis may lead to inappropriate management. Further work is needed to examine the influences and consequences of diagnostic decision making in sarcoidosis.

BACKGROUND:Corticosteroids, the most commonly prescribed treatment for sarcoidosis, are associated with significant toxicity, especially with long-term usage. New intervention trials designed to reduce or eliminate corticosteroids require pertinent and precise clinical trial end-points. However, consensus is lacking. The aim of the current study is to develop consensus for trial end-points in pulmonary sarcoidosis. METHODS:A Delphi survey was developed by an Expert Panel of 30 sarcoidosis investigators. For Round 1, the panel created 97 statements regarding potential end-points for a clinical trial of symptomatic pulmonary corticosteroid-treated sarcoidosis patients. After anonymous voting in Round 2, the 97 statements were refined by all Stakeholders, including the Expert Panel and an additional 70 individuals interested in sarcoidosis therapies. RESULTS:After Round 2, the Expert Panel achieved consensus for combination end-points on 38 statements across six domains that supported the following end-points: prednisone reduction by 50% or discontinuation, 10% predicted or greater improvement in forced vital capacity % predicted, forced expiratory volume in 1 s % predicted and diffusing capacity of the lung for carbon monoxide % predicted, and improvement in the King's Sarcoidosis Questionnaire Lung and General Health domains. Additionally, the majority of Stakeholders agreed to all statements which reached consensus by the Expert Panel. CONCLUSIONS:Utilising the Delphi technique, international sarcoidosis experts successfully achieved consensus for 38 specific clinical trial end-points which can be utilised in the development of novel steroid-sparing and eliminating therapies for pulmonary sarcoidosis.

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease presenting primarily in women of child-bearing age. It can occur sporadically or in association with tuberous sclerosis. Here, we discuss the case of a 29-year-old woman on oral contraceptive pills (OCPs) for years, who presented with sudden-onset chest pain and dyspnea found to have a pneumothorax. Serologic testing confirmed a diagnosis of LAM. An early diagnosis can lead to earlier treatment with sirolimus, which can improve lung function and survival among those with LAM.

Objectives The Steroid PRO is a treatment-specific patient-reported outcome questionnaire which measures the impact of glucocorticoids on health-related quality of life. It has 15 items grouped into 4 domains (Social impact, Impact on Appearance, Psychological Impact and Treatment Concerns). Initially developed and validated in rheumatic diseases, the Steroid PRO demonstrates potential for broader application in patients with other inflammatory conditions. The objective of this study was to assess face validity, content validity and feasibility of the Steroid PRO in (1) patients treated with glucocorticoids for inflammatory respiratory, dermatological and gastroenterological conditions and (2) clinicians working within these specialties in the UK and USA.Design Qualitative study with semistructured cognitive interview methods.Setting Online or face-to-face interviews with participants from seven departments across three secondary care hospitals in the UK and USA.Participants Inclusion criteria: (1) Adult patients with inflammatory respiratory, gastroenterological and dermatological conditions treated with glucocorticoids and (2) healthcare professionals (HCPs) working in respiratory, dermatology and gastroenterology departments in the UK and USA.Results Purposive sampling to ensure a range of patient and HCP participants. A total of 42 patient participants were recruited, from respiratory/pulmonology (n=14, 33.3%), dermatology (n=13, 31.0%) and gastroenterology (n=15, 35.8%) medical departments; 32 in the UK and 10 from the USA. Mean age 48.2 years (range 22–71) and 19 (45.2%) were female. Patient participants had a range of inflammatory lung, skin and bowel conditions, with a spectrum of demographics and patterns of glucocorticoid use. 14 HCPs participated from the UK (9) and USA (5). Face validity: 97% (30/31) patients and 100% (14/14) HCPs reported the Steroid PRO was ‘relevant or very relevant’ to them and their disease. Feasibility: 97% (30/31) patients and 100% (14/14) HCPs reported the Steroid PRO was ‘easy or very easy to complete’. Patients reported that the four domains of the Steroid PRO had relevance to them and that it was validating to see their concerns represented: ‘It’s obvious you guys know what you’re talking about—these are my issues. It’s very validating when you realise it’s not just you. These problems are real and they matter.… These are not questions my doctor asks me about. Doctors never ask about psychosocial aspects. It would be really great if they used this’ (female patient with asthma). Patients and clinicians felt the Steroid PRO would be suitable for use in clinical practice within their specialties and would aid in understanding of the impact of glucocorticoids.Conclusions The Steroid PRO demonstrated face validity and content validity for assessing the impact of glucocorticoids in patients with inflammatory respiratory, gastroenterological and dermatological conditions. Additionally, the feasibility of using the Steroid PRO with both patients and HCPs has been established. Future work should include quantitative testing of the Steroid PRO as an outcome measure within clinical trials in these conditions.Trial registration number NCT06314451.

Background Individuals with sarcoidosis face many sources of illness uncertainty, including diagnostic delays, unpredictable therapeutic efficacy and toxicity, and disease-associated morbidity and mortality. Patient perspectives on illness uncertainty in sarcoidosis have not been evaluated critically and offer an opportunity for providers to contextualize and prioritize gaps in care and patient support. Research Question How do patients with sarcoidosis describe their lived experiences with the disease and challenges they face in receiving care? Study Design and Methods We conducted semistructured qualitative interviews with patients with biopsy-proven pulmonary sarcoidosis receiving treatment for the disease who were seen at a tertiary sarcoidosis center of excellence. Interviews examined patient experiences of living with sarcoidosis, including their journey with diagnosis, treatment, and monitoring of disease activity. Transcripts were coded and categorized into themes and subthemes. Saturation was defined as at least 3 interviews without new information. Results Twenty-five participants completed semistructured interviews. The median age was 60 years, with 64% of the participants being female and 68% identifying as Black. The impact of illness uncertainty was a shared component of their care journeys. Key themes that emerged were (1) the burden of limited disease awareness, (2) uncertainty about sarcoidosis management, and (3) the unpredictability of disease progression. Uncertainty emerged as a major challenge that contributed to delays in care, poor disease control, psychological distress, or a combination thereof. Interpretation Our findings are the first, to our knowledge, to highlight the impact of patient illness uncertainty on sarcoidosis disease outcomes and psychological distress. Individuals living with sarcoidosis may benefit by addressing the psychosocial impact of uncertainty. Individuals living with sarcoidosis may benefit significantly from targeted interventions to mitigate the impact of illness uncertainty.

Sarcoidosis is a disease with high morbidity that has variable epidemiology based on genetics and sociodemographic factors. The etiology of this variability remains incompletely understood. This narrative review describes how genetics, social determinants of health, and the interactions between them may contribute to the differences in epidemiology and health outcomes observed in different patient groups with sarcoidosis.

Introduction Cardiac sarcoidosis (CS) is characterized by cardiac inflammation resulting in heart failure, ventricular arrhythmias, and conduction disease. Diagnostic uncertainty persists in the subset of patients with non-histologic, isolated CS. Despite increasing use of tumor necrosis factor-alpha inhibitors (TNFai) to treat CS, their safety and outcomes in this particular CS phenotype has not been well described. Methods Patients seen in a tertiary referral Sarcoidosis Center were included if they met clinical (non-histologic) criteria for isolated CS (Japanese Circulation Society guidelines) and were treated with TNFai. Demographics, baseline characteristics and clinical outcomes, including fluorodeoxyglucose-positron emission tomography (FDG-PET) results, were retrospectively adjudicated and descriptive statistical analyses were performed. Results Six patients (50% female, 16% Black, mean age 55.1 ± 9.6 years) with clinical, isolated CS treated with TNFai were identified. Alternative causes of cardiomyopathy were evaluated, including 5 patients who underwent cardiac/arrhythmia genetic testing panel negative for pathogenic variants. All patients had a lymph node (n=2) or endomyocardial (n=4) biopsy negative for granulomatous inflammation. Patients were initiated on TNFai 1.9 ± 0.8 mean yrs after CS diagnosis and all patients had ongoing cardiac FDG uptake despite oral immunosuppression with both prednisone (19.2 ± 12 mg) and a steroid sparing agent at time of TNFai initiation. Mean LVEF at initiation was 41.6 ± 7.9% (range 30-55%) and 2 (33%) patients had right ventricular dilation (RV) with RV dysfunction in 1 patient. All patients were NYHA class I or II.On post-TNFai follow up FDG-PET (median 6 months), 2 (33%) had complete resolution of cardiac FDG, while 4 (67%) had ongoing inflammation. Ultimately, 5 patients were able to achieve complete resolution of cardiac FDG uptake (mean time 10.1 ± 6.1 months) however 1 of these patients had recurrent inflammation requiring increased infliximab dosing and change in oral agent. Prednisone dose was substantially lower at time of cardiac FDG resolution (mean 4 ± 4.1 mg). Conclusions We found that among patients with clinical, isolated CS, TNFai was effective in the majority of patients to control cardiac inflammation but may require frequent dose changes and take nearly one year for complete resolution. This case series highlights the gaps for which prospective trials are needed to identify ideal TNFai initiation timing (upfront versus third line) and dosing, optimal patient selection, and relevant treatment endpoints in isolated CS.

The Americas Association of Sarcoidosis and Other Granulomatous Disorders (AASOG) 2024 conference, held in Baltimore, Maryland, leveraged a multidisciplinary approach to disseminating and addressing the latest updates, challenges and opportunities in multisystemic sarcoidosis. The conference, aptly titled "The Art of Working Together for Progress," featured insights from diverse perspectives in sarcoidosis both nationally and internationally. This review summarizes the key takeaways from the six conference sessions: I. Sarcoidosis Multidisciplinary Care, II. Health Disparities in Sarcoidosis, III. The Search for Precision in Sarcoidosis, IV. Clinical Outcomes in Sarcoidosis, V. Clinical Trials in Sarcoidosis, and VI. Advanced Disease in Sarcoidosis.

BACKGROUND:Sarcoidosis, when clinically isolated to the heart, is characterized by a high burden of disease, diagnostic uncertainty, and treatment challenges. Tumor necrosis factor-alpha inhibitors (TNF-αi) are increasingly used to treat cardiac sarcoidosis (CS), however, hesitancy and limited guidance remain regarding the treatment of patients with isolated CS. CASE SUMMARY:Here, we present 6 cases of clinically isolated CS treated with TNF-ai. We describe diagnostic testing, treatment history, and outcomes after initiation of TNF-ai. DISCUSSION:There is hesitancy and lack of evidence in treating non-biopsy confirmed isolated CS with TNF-ai because of diagnostic uncertainty and often more advanced cardiomyopathy. We find that this treatment approach is generally well tolerated, with favorable outcomes. A shared decision-making approach should be included. TAKE-HOME MESSAGE:TNF-ai treatment can improve cardiac inflammation on fluorodeoxyglucose positron emission tomography and reduce corticosteroid exposure in sarcoidosis isolated to the heart.

Sarcoidosis is a systemic disease characterized by marked clinical equipoise regarding optimal methods for disease diagnosis, monitoring, and treatment. As a result of these challenges, patients with sarcoidosis face substantial delays in care and have reported psychological distress from the uncertainty they face throughout their care journeys. In complex diseases with multisystemic involvement, multidisciplinary care models can help provide diagnostic clarity and streamline care. Although experts and guidelines in the field advocate for multidisciplinary care to improve clinical management of sarcoidosis, limited primary literature describes implementation of these care models in sarcoidosis. In this review, we outline best practices and common challenges associated with establishing a multidisciplinary care team for sarcoidosis. We describe the development of the Johns Hopkins Sarcoidosis Center (JHSC) multidisciplinary team as well as the formation of the Johns Hopkins Sarcoidosis patient advisory board, which helps inform the team's goals and initiatives. Finally, we review the broader literature on multidisciplinary care models in sarcoidosis and interstitial lung disease, identifying areas for further study.

The tumor necrosis factor-α (TNF-α) inhibitors etanercept and certolizumab pegol (CZP) have been associated with sarcoid-like granulomatous reactions. Unlike other TNF-α inhibitors like infliximab and adalimumab that can be effective treatments for refractory sarcoidosis, etanercept and CZP do not induce cytoxic complement-induced cell lysis. To our knowledge, cardiac involvement in anti-TNF-α sarcoidosis has been reported in one patient previously. Here, we report two cases of cardiac sarcoidosis associated with etanercept and CZP.1. A 57-year-old woman with history of psoriasis presented with dyspnea. She had been on etanercept therapy for 3 years prior to presentation. She was found to be in complete heart block requiring urgent temporary pacemaker placement. Transthoracic echocardiography revealed moderately reduced left ventricular (LV) systolic function. She had mild coronary artery disease on coronary angiography. Cardiac MRI showed transmural late gadolinium enhancement in the LV basal septal wall and mid lateral wall (Figure 1A). Cardiac PET showed multifocal patchy areas of intense myocardial FDG avidity involving the left ventricle (Figure 1B) and prominent mildly avid right hilar nodes. She underwent implantation of a CRT-D and was initiated on prednisone and a steroid sparing agent for possible isolated cardiac sarcoidosis. Biopsies of hilar lymph nodes obtained one month following immunosuppression initiation were non-diagnostic for sarcoidosis.2. A 55-year-old woman with history of myasthenia gravis, Hashimoto's thyroiditis, and Sjogren's syndrome presented with productive cough. She had been on CZP for 8 months prior to symptom-onset. Chest imaging revealed mediastinal adenopathy. She underwent mediastinal lymph node biopsy revealing non-necrotizing granulomatous inflammation. Cardiac MRI showed subendocardial and mid-myocardial late enhancement in the basal inferior and inferolateral segments of the LV (Figure 1C). She had mild myocardial inflammation in the basal segment of the lateral and inferolateral segments on cardiac PET with FDG avid mediastinal lymphadenopathy and spleen concerning for active systemic sarcoidosis (Figure 1D). She was initiated on methotrexate and infliximab for probable cardiac sarcoidosis and biopsy-proven pulmonary sarcoidosis.In cases of pulmonary sarcoidosis associated with TNF-α inhibition, withdrawal of the agent alone can lead to improvement. Alternative TNF-α inhibitors like infliximab and adalimumab have also been reported to be effective for the treatment of sarcoidosis associated with etanercept. Further studies are necessary to elucidate the exact mechanisms that underlie the pathophysiology of sarcoidosis associated with etanercept and CZP, and the optimal therapies for these patients.

Prior work has reported differences in the prevalence of sarcoidosis as well as patient outcomes based on race, gender, and socioeconomic status. We investigated whether sociodemographic factors were associated with referral to pulmonary medicine at a large academic center for patients with an incident international classification of diseases (ICD) diagnosis of sarcoidosis. We conducted a single-center retrospective study examining the associations between sociodemographic factors and time to pulmonary medicine referral in patients with an incident ICD diagnosis of sarcoidosis between October 31, 2011, and October 30, 2021. In our center, referral to pulmonary medicine for sarcoidosis is equivalent to referral to the sarcoidosis clinic. Additional outcomes were associations between pulmonology referral and receiving a pulmonary function test (PFT), electrocardiogram (EKG), or computed tomography scan of the chest (CT-chest). We identified 1,017 patients with an incident ICD diagnosis of sarcoidosis. Only 276 (27

EditorialNew Updates in Sarcoidosis Research: Defining and Renewing the QuestCatherine A. Bonham and Michelle SharpCatherine A. BonhamDepartment of Medicine, University of Virginia, Charlottesville, VA, United States, andMichelle Sharp* Corresponding Author; email: [email protected]Department of Medicine, Johns Hopkins University, Baltimore, MD, United States*Published Online:15 Mar 2024https://doi.org/10.1152/ajplung.00082.2024MoreSectionsPDF (113 KB)Download PDF ToolsExport citationAdd to favoritesGet permissionsTrack citations ShareShare onFacebookTwitterLinkedInWeChat Back to Top Next Download PDF FiguresReferencesRelatedInformation More from this issue > Articles in PressPublished 3/15/24 1:00 AM Crossmark Copyright & PermissionsCopyright © 2024, American Journal of Physiology-Lung Cellular and Molecular Physiologyhttps://doi.org/10.1152/ajplung.00082.2024PubMed38487816History Received 29 February 2024 Accepted 14 March 2024 Published online 15 March 2024 KeywordsSarcoidosis Metrics