Focus on Sarcoidosis
Correspondence for "clinical epidemiology of familial sarcoidosis: A systematic literature review"We have read the very interesting article titled “Clinical epidemiology of familial sarcoidosis: a systematic literature review” by Drs. Terwiel and van Moorsel published recently in Respiratory medicine . The work was based on the very stringent selection of 27 articles out of a total of 459 published on sarcoidosis in order to obtain a high level of statistical quality in the evaluation of the prevalence of familial forms. This review took into account all articles published until March 2018, date of submission to Respiratory Medicine.
Correspondence for “Clinical epidemiology of familial sarcoidosis: A systematic literature review”It is with great interest that we read the article titled “Clinical epidemiology of familial sarcoidosis: A systematic literature review” by Drs. Terwiel and van Moorsel published recently in Respiratory Medicine . Their systematic review on familial sarcoidosis highlights the great heterogeneity in familial disease prevalence and in familial relative risks among published studies. They also emphasize the high heritability (>60%) of sarcoidosis.
Economic burden of incident interstitial lung disease (ILD) and the impact of comorbidity on costs of careEvidence about the economic burden related to interstitial lung diseases (ILDs) and the cost-driving factors is sparse. In the knowledge that distinct comorbidities affect the clinical course of ILDs, our study investigates their impact on costs of care within first year after diagnosis.
Serum CXCL11 correlates with pulmonary outcomes and disease burden in sarcoidosisSarcoidosis is a systemic granulomatous disease of unknown etiology that affects the lungs in 90% of patients, but has a wide range of disease manifestations and outcomes including chronic and progressive courses. Noninvasive biomarkers are needed to assess these outcomes and guide decisions for long term monitoring and treatment. Interferon-gamma (IFN-γ)-inducible chemotactic cytokines (chemokines), CXCL9, CXCL10 and CXCL11, show promise in this regard because they have been implicated in the pathogenesis of and reflect the burden of granulomatous inflammation.
Direct and indirect economic and health consequences related to sarcoidosis in Denmark: A national register-based studySarcoidosis is a systemic disease that primarily affects the younger population. Longitudinal studies of the economic burden of sarcoidosis are scarce. This study evaluates overall the economic burden of sarcoidosis in Denmark before and after initial diagnosis.
Elderly sarcoidosis: A comparative study from a 42-year single-centre experienceTo describe the clinical features and outcomes in elderly patients with sarcoidosis and to compare them with younger patients.
Quantifying the relationship between symptoms at presentation and the prognosis of sarcoidosisAlthough it is the general consensus that sarcoidosis patients who present with sarcoidosis-related symptoms have a worse outcome than patients whose disease is detected incidentally without symptoms, this premise has not been rigorously examined.
Sarcoidosis: No longer a benign disease?Sarcoidosis is a multi-system disease of unknown cause characterized by noncaseating granulomatous inflammation that results in symptoms and organ dysfunction due to persistent inflammation and fibrosis. It predominately affects the lungs in over 90% of patients, but can injure any organ in the body. Sarcoidosis can be a life-threatening disease, particularly in those with pulmonary fibrosis, pulmonary hypertension, or cardiac involvement [1,2]. Opportunistic infections may also play a role in mortality in patients on immunosuppression [2,3].
The development of sarcoidosis in patients receiving daclizumab: A case series from multiple clinical trialsSeveral drugs have been associated with druginduced sarcoidosis-like reactions (DISRs) that are clinically indistinguishable from sarcoidosis. Daclizumab is a humanized monoclonal IgG1 antibody that binds to CD25 that has been studied for the treatment of multiple sclerosis (MS). During MS clinical trials of daclizumab, 12 subjects developed clinical conditions potentially consistent with sarcoidosis. Therefore, an independent adjudication committee of individuals with expertise in sarcoidosis was organized to determine the likelihood of these cases representing sarcoidosis.
Can the Sarcoidosis Health Questionnaire predict the long-term outcomes in Japanese sarcoidosis patients?The Sarcoidosis Health Questionnaire (SHQ) is the first sarcoidosis-specific health status questionnaire ever developed. Worse health status, as evaluated by the SHQ, may indicate higher risk for deterioration in the following 5 years.
Clinical epidemiology of familial sarcoidosis: A systematic literature reviewAlthough the presence of familial sarcoidosis has been confirmed, clinical and epidemiological data on its characteristics are scattered and sometimes paradoxical. The objective of this review is to assess what is known on the clinical epidemiology of familial sarcoidosis, by combining data from early case reports with recent population based data; aiming to support in clinical decision making and providing information to patients.
Sarcoidosis deaths in the United States: 1999–2016It has been over a decade since a comprehensive study has been published that has examined sarcoidosis deaths at the national level. The purpose of this study was to analyze sarcoidosis as the underlying cause of death using current national death certificate data. Results from this project can be used to evaluate and compare trends of sarcoidosis reported deaths across the U.S.
Response to Letter to Editor regarding "Sarcoidosis and IPF in the same patient-a coincidence, an association or a phenotype?"We thank Drs. Valentin, Hyde, Gomez and Patel for commenting on our recently published article “Sarcoidosis and IPF in the same patient-a coincidence, an association or a phenotype?” . Indeed, one of our objectives in reporting our observations was to provoke further interest in the scientific community regarding such patients and conduct of clinical studies in such cohorts.
Letter to the editorIt was with great interest we read the article by Dr. Collins and colleagues published in the August 2018 edition of Respiratory Medicine, titled “Sarcoidosis and IPF in the same patient-a coincidence, an association or a phenotype?” The authors describe the characteristics of a disease entity they coined “combined sarcoidosis and idiopathic pulmonary fibrosis (CSIPF)” . We appreciate the authors’ efforts to shed light on this clinical entity, which to date has sparked debate in terms of proper classification of patients who share radiographic and histopathologic features of sarcoidosis and idiopathic pulmonary fibrosis (IPF).
Sarcoidosis: A benign disease or a culture of neglect?The widespread perception that sarcoidosis is essentially a benign disease has undoubtedly contributed to a lack of investment in sarcoidosis-related public health and research initiatives. Respiratory clinicians managing both pulmonary sarcoidosis and idiopathic pulmonary fibrosis may be especially likely to subscribe to this view. Unlike most other forms of fibrotic lung disease, pulmonary sarcoidosis stabilizes with time, with or without therapy, in the majority of patients. Sarcoidosis patients with major cardiac or neurologic involvement are often managed by sub-specialists and may be under-represented in general sarcoidosis cohorts.
Combined sarcoidosis and idiopathic pulmonary fibrosis (CSIPF): Genuine disease entity, obscure clinical phenotype or diagnostic red herring?The diffuse parenchymal lung diseases (DPLDs), often collectively referred to as the interstitial lung diseases (ILDs), are a heterogeneous group of disorders that are classified together because of similar clinical, radiographic, physiologic, or pathologic manifestations . Universal features of the DPLDs include cellular proliferation, inflammation and/or fibrosis within the alveolar wall . Architectural distortion of the airways and alveoli is also frequently present. The DPLDs are categorized into those that are associated with known causes and those that are idiopathic.
Work ability before and after sarcoidosis diagnosis in SwedenInformation on how sarcoidosis affects one's ability to work is needed to clarify the burden of disease on the individual and society. Our aim was to describe the work ability of individuals with sarcoidosis before and after diagnosis compared to people without sarcoidosis.
Cardiac sarcoidosis: Diagnosis confirmation by bronchoalveolar lavage and lung biopsyThe diagnosis of cardiac sarcoidosis (CS) is difficult to ascertain due to the insensitivity of endomyocardial biopsy. Current diagnostic criteria require a positive endomyocardial biopsy or extra-cardiac biopsy with clinical features suggestive of CS. Common tests for diagnosis of pulmonary sarcoidosis include bronchoalveolar lavage (BAL), lung and mediastinal lymph node (MLN) biopsies. Our objective was to determine the diagnostic utility of these tests in patients with suspected CS and without prior history of pulmonary involvement.
Sarcoidosis and IPF in the same patient-a coincidence, an association or a phenotype?Idiopathic Pulmonary Fibrosis (IPF) and Sarcoidosis are distinct clinical entities. Fibrotic disease in pulmonary sarcoidosis is typically upper lobe predominant. In IPF fibrosis is basilar and peripheral predominant [usual interstitial pneumonia (UIP) pattern]. Sarcoidosis and UIP have rarely been observed in the same patient. We sought to characterize patients manifesting both sarcoidosis and IPF and compare clinical features and survival to that of patients with “Lone-IPF” (IPF only) and pulmonary sarcoidosis with fibrosis in a non-UIP pattern.
Monitoring cardiac sarcoidosis: The next frontierCardiac sarcoidosis is a potentially life-threatening form of the disease. Although most sarcoidosis-related deaths result from lung involvement, most of these deaths occur after years of the slow development of lung fibrosis from granulomatous inflammation [1,2]. Contrary to pulmonary sarcoidosis, cardiac sarcoidosis may cause life-threatening events and severe organ dysfunction relatively quickly because of myocardial granulomas deposited in unfortunate locations. Furthermore, myocardial sarcoid granulomas tend to progress relatively quickly to scar  that does not respond to anti-granulomatous therapy and is permanent.
Prevalence, incidence, and mortality of sarcoidosis in Korea, 2003–2015: A nationwide population-based studyThe prevalence and incidence of sarcoidosis, a granulomatous disorder involving multiple organ systems, varies among geographical regions and ethnicities. This study evaluated the incidence, prevalence, and mortality of sarcoidosis in a large nationwide population-based cohort in Korea.
Sleep disturbance and symptom burden in sarcoidosisSarcoidosis is a systemic inflammatory disease associated with myriad symptoms, including fatigue. It can affect physiological processes like sleep, leading to poor sleep quality and excessive daytime sleepiness. We hypothesized that sarcoidosis patients would report more severe sleep disturbance than healthy controls and that relationships would be found with sleep disturbance and the severity of other symptoms.
Sarcoidosis in WTC-Exposed workersWithout adequate attention to the incidence and prevalence of surveillance with diagnostic technology, and without suitable consideration of the technologic effects on what becomes detected or left unidentified, the rates of reported diseases simply represent the rates of diagnostic detection and cannot be accepted as scientific enumeration of the true incidence and prevalence of those diseases. .
Efficacy and safety of infliximab biosimilar Inflectra® in severe sarcoidosisInfliximab, a monoclonal antibody against tumor necrosis factor alpha (TNF-α) is effective third-line therapy in severe sarcoidosis. The originator product of Infliximab, Remicade®, is expensive, limiting universal access. Recently, a less expensive biosimilar of infliximab, Inflectra®, has become available, but the efficacy and tolerability has not been studied in sarcoidosis.
Effect of inhaled corticosteroids on lung function in chronic beryllium diseaseThe clinical effects of inhaled corticosteroids (ICS) on chronic beryllium disease (CBD) are unknown. Although frequently used for symptoms or disease not requiring systemic therapy, the clinical course of patients on ICS has not been evaluated.