The association of baseline sarcoidosis measurements with 6-month outcomes that are of interest to patients: Results from the On-line Sarcoidosis Assessment Platform Study (OSAP).

INTRODUCTION: The impact of common measures to assess sarcoidosis have not been compared longitudinally to outcomes that are meaningful to patients. We prospectively examined the relationship of baseline measurements of sarcoidosis status to outcomes of interest to patients longitudinally over 6 months. METHODS: Sarcoidosis patients cared for at 6 US medical centers were "phenotyped" at baseline with measurements of pulmonary function, organ involvement, health related quality of life (HRQoL) instruments, and their anti-sarcoidosis treatment history. These patients were followed for 6 months by monitoring outcomes of interest to patients (OIPs) including steps walked, calories expended, sleep, HRQoL measures, workdays missed and health care utilization. For each baseline phenotypic measurement, patients were dichotomized into two groups above and below a specified cutoff value. The area under the OIP versus time curve was compared between these two groups. RESULTS: The cutoff values for many baseline phenotypic measures distinguished the patients into groups with significantly different 6-month OIPs. The chosen cutoff for the patient global estimate of health status distinguished the most OIPs (13/15). The 6-min walk distance cutoff was associated with more OIPs than spirometric measures. All of the HRQOL measure cutoffs were associated with many OIPs, although most of them were other HRQOL measures. INTERPRETATION: Cutoffs for most of the phenotypic measures used to assess sarcoidosis distinguished groups of sarcoidosis patients with differing OIPs over the subsequent 6 months. The patients' global assessment of their disease was the most accurate of these measures. CLINICAL TRIAL REGISTRATION NUMBER: NCT04342403.

Assessment of dyspnea in sarcoidosis using the Baseline Dyspnea Index (BDI) and the Transition Dyspnea Index (TDI).

INTRODUCTION: The Borg and Modified Medical Research Council (mMRC) dyspnea scales have been used to evaluate dyspnea in sarcoidosis. The Baseline Dyspnea Index (BDI) and Transitional Dyspnea Index (TDI) are useful for the assessment of dyspnea in COPD. It is not known if the BDI-TDI accurately assesses dyspnea in sarcoidosis patients. METHODS: Data was analyzed from the Registry for Advanced Sarcoidosis (ReAS), a multi-national database enrolling patients with advanced sarcoidosis and a comparison group of sarcoidosis patients with non-advanced disease. At baseline, patients completed a BDI questionnaire along with spirometry, 6-min walk distance (6MWD), mMRC, Borg score, fatigue assessment score (FAS) and HRQoL assessments using Kings Sarcoidosis Questionnaire (KSQ) and St Georges Respiratory Questionnaire (SGRQ). At 12-months, patients with advanced disease completed a TDI questionnaire along with the other measures. Correlations between BDI and baseline variables, and between TDI and changes in baseline variables were evaluated. RESULTS: There was significant correlation (p < 0.001 for all) between BDI and baseline 6MWD (rho = 0.336), FVC% (rho = 0.387), FEV1% (rho = 0.285), DLCO% (rho = 0.355), mMRC (rho = -0.721), Borg score (rho = -0.389), FAS (rho = -0.669), SGRQ (rho = -0.785), and KSQ (rho = 0.318 to 0.724). At follow-up, TDI correlated with BDI, but not with changes in pulmonary function or other dyspnea measures. CONCLUSION: BDI scores correlated with pulmonary function, 6MWD, and other dyspnea measures. TDI scores did not correlate with changes in pulmonary function or other dyspnea measures. BDI may be a useful independent measure of dyspnea in sarcoidosis patients. The role of TDI needs further evaluation in longitudinal studies associated with changes in clinical parameters.

The Value of a Patient Global Assessment in Management of Sarcoidosis.

The patient global assessment (PGA) is a reported outcome instrument used to gauge the patient's well-being. We performed a prospective study of patients seen at the University of Cincinnati Sarcoidosis Clinic. Two groups were studied: those at first visit during the time period (initial) and those seen at least one more time by the same physician (follow-up). A total of 1006, including 677 initial, visits occurred during the six-month period. Patients in whom anti-inflammatory treatment was initiated or increased had a significantly lower PGA score (ANOVA p < 0.001, p < 0.05 for increased versus all others). There was no significant difference in initial PGA score based on race, sex, or age. The change in PGA was significantly lower for patients in whom treatment was increased (ANOVA p < 0.001, increased different from all others, p < 0.05). The PGA was significantly lower for patients in whom anti-inflammatory therapy was increased; however, there was overlap between groups.

Income and Other Contributors to Poor Outcomes in U.S. Patients with Sarcoidosis.

Rationale: Socioeconomic factors are associated with worse disease severity at presentation in sarcoidosis, but the relative importance of socioeconomic variables on morbidity and disease burden has not been fully elucidated.Objectives: To determine the association between income and sarcoidosis outcomes after controlling for socioeconomic and disease-related factors.Methods: Using the Sarcoidosis Advanced Registry for Cures database, we analyzed data from 2,318 patients with sarcoidosis in the United States to determine the effect of income and other variables on outcomes. We divided comorbidities arising after diagnosis into those likely related to steroid use and those likely related to sarcoidosis. We assessed the development of health-related, functional, and socioeconomic outcomes following the diagnosis of sarcoidosis.Measurements and Main Results: In multivariate analysis, low-income patients had significantly higher rates of new sarcoidosis-related comorbidities (<$35,000, odds ratio [OR], 2.4 [1.7-3.3]; $35,000-84,999, OR, 1.4 [1.1-1.9]; and ≥$85,000 [reference (Ref)]) and new steroid-related comorbidities (<$35,000, OR, 1.3 [0.9-2.0]; $35,000-84,999, OR, 1.5 [1.1-2.1]; and ≥$85,000 [Ref]), had lower health-related quality of life as assessed by the Sarcoidosis Health Questionnaire (P < 0.001), and experienced more impact on family finances (<$35,000, OR, 7.9 [4.9-12.7]; $35,000-84,999, OR, 2.7 [1.9-3.9]; and ≥$85,000 [Ref]). The use of supplemental oxygen, need for assistive devices, and job loss were more common in lower income patients. Development of comorbidities after diagnosis of sarcoidosis occurred in 63% of patients and were strong independent predictors of poor outcomes. In random forest modeling, income was consistently a leading predictor of outcome.Conclusions: These results suggest the burden from sarcoidosis preferentially impacts the economically disadvantaged.

Current concepts regarding calcium metabolism and bone health in sarcoidosis.

PURPOSE OF REVIEW: Vitamin D supplementation is widespread used in the general population. In sarcoidosis, up to 50% of patients, especially postmenopausal women and those taking corticosteroids, show evidence of increased bone fragility. The purpose of this review is to provide an evidence-based rationale on how to treat sarcoidosis patients with bone health issues. RECENT FINDINGS: Evidence from observational studies show that decreased 25-hydroxy vitamin D is common in sarcoidosis. However, the great majority of sarcoidosis patents have normal or often elevated levels of 1,25-dihydroxy vitamin D (calcitriol), a marker associated with disease activity. High calcitriol levels may often be associated with hypercalcemia and hypercalcuria. The few interventional randomized controlled studies in the field, suggest that vitamin D supplementation may not be well tolerated because of hypercalcemia, moreover without substantial benefit on bone health and risk for fractures in these patients. SUMMARY: Vitamin D supplementation may be withheld in sarcoidosis patients with bone fragility, unless calcitriol levels are below normal limits. A treating scheme is proposed.

Inter-rater reliability of cutaneous sarcoidosis assessment tools via remote photographic assessment.

Background: Recently two outcome instruments have been developed and validated for assessing cutaneous sarcoidosis in a live, in-person setting. Teledermatology is a rapidly growing field; yet, to date, no instrument has been validated for use in a remote setting, which could ultimately impact clinical trial design. Objective: To assess the interrater reliability of these outcome instruments for store-and-forward teledermatology. Methods: Seven sarcoidosis experts, including both pulmonologists and dermatologists, scored photographs of cutaneous sarcoidosis lesions in 13 patients utilizing the Cutaneous Sarcoidosis Activity and Morphology Index (CSAMI), the Sarcoidosis Activity and Severity Index (SASI) and the Physician Global Assessment (PGA). Interrater reliability was assessed for each instrument and was compared to results obtained from a prior study involving sarcoidosis experts evaluating the same patient population in an in-person setting. Results: Interrater reliability (presented as ICC [95%CI]) was poor for the CSAMI Activity scale (0.36 [0.16 - 0.65]) and the CSAMI Damage scale (0.17 [0.04 - 0.43]) and was fair for the Modified Facial SASI (0.59 [0.36 - 0.82]) and the PGA (0.47 [0.23 - 0.74]). All results were inferior to those obtained from the prior studies validating these instruments for in-person use. Conclusions: Given the superiority of these instruments when utilized in person, it is recommended to have an on-site sarcoidosis expert evaluate cutaneous sarcoidosis lesions whenever possible. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 165-169).

Sarcoidosis in America. Analysis Based on Health Care Use.

RATIONALE: There have been no recent comprehensive studies of the epidemiology of sarcoidosis in the United States. Changes in health care use have made available access to data on large numbers of patients with sarcoidosis. OBJECTIVES: To use a U.S. national health care database to gather data on patients with sarcoidosis identified over a 3-year period who were 18 years of age and older, and to determine health care costs for these patients. METHODS: The Optum health care database was queried for a 3-year period (2010-2013). This database includes approximately 15% of U.S. residents. The incidence rate of sarcoidosis was calculated for new cases identified in each year. Calculation of prevalence was based on any patient with sarcoidosis seen during the year. Incidence and prevalence rates are reported per 100,000 patients. MEASUREMENTS AND MAIN RESULTS: A total of 29,372 adult patients with sarcoidosis were identified. Of these, 14,700 (55%) were over 55 years of age at the time of diagnosis. The incidence and prevalence rates were higher for African Americans (17.8 and 141.4 per 100,000, respectively) than for white individuals (8.1 and 49.8), Hispanics (4.3 and 21.7), or Asians (3.2 and 18.9). Women were two times more likely to have sarcoidosis, with the highest prevalence for sarcoidosis noted in African American women (178.5). Overall, the yearly health care cost reported for patients with sarcoidosis was low, with a median of $18,663 per year. However, the yearly cost for the top 5% was $93,201. CONCLUSIONS: For patients 18 years of age and older enrolled in a U.S. national administrative database, sarcoidosis was more common among African Americans, but it was reported for all four of the major ethnic groups studied. While health care costs were relatively small for most patients, the cost of care for some patients was considerable.

The WASOG Sarcoidosis Organ Assessment Instrument: An update of a previous clinical tool.

INTRODUCTION: A Case Control Etiology of Sarcoidosis Study (ACCESS) sarcoidosis organ assessment instrument has been used for more than a decade to establish uniform standards for the probability of sarcoidosis organ involvement. The ACCESS instrument has become increasingly outdated as new technologies have been developed. Furthermore, the ACCESS instrument failed to address all possible organs involved with sarcoidosis. For these reasons, the World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) developed a new sarcoidosis organ assessment instrument. METHODS: Clinical sarcoidosis experts assessed various clinical manifestations for the probability of sarcoidosis organ involvement. Two criteria were required to apply this assessment: 1) histologic evidence of granulomatous inflammation of unknown cause in an organ that was not being assessed; 2) the clinical manifestation being addressed required that alternative causes other than sarcoidosis had been reasonably excluded. Clinical manifestations were assessed as either: a) highly probable: likelihood of sarcoidosis causing this manifestation of at least 90%.; b) probable: likelihood of sarcoidosis causing this manifestation of between 50 and 90%; c) possible: likelihood of sarcoidosis causing this manifestation of less than 50%. The sarcoidosis experts voted on the likelihood of sarcoidosis causing each manifestation using Delphi study methodology where at least 70% agreement of the experts was needed for consensus. RESULTS: Various clinical manifestations were classified as highly probable, at least probable, possible, or indeterminate when no consensus could be reached. CONCLUSION: An instrument was developed by expert opinion that may be useful for the clinician and researcher in establishing criteria for sarcoidosis organ involvement.

Health care-associated pneumonia (HCAP): a critical appraisal to improve identification, management, and outcomes--proceedings of the HCAP Summit.

Increasingly, patients are receiving treatment at facilities other than hospitals, including long-term-health care facilities, assisted-living environments, rehabilitation facilities, and dialysis centers. As with hospital environments, nonhospital settings present their own unique risks of pneumonia. Traditionally, pneumonia in these facilities has been categorized as community-acquired pneumonia (CAP). However, the new designation for pneumonias acquired in these settings is health care-associated pneumonia (HCAP), which covers pneumonias acquired in health care environments outside of the traditional hospital setting and excludes hospital-acquired pneumonia (HAP), ventilator-associated pneumonia (VAP), and CAP. Although HCAP is currently treated with the same protocols as CAP, recent evidence indicates that HCAP differs from CAP with respect to pathogens and prognosis and, in fact, more closely resembles HAP and VAP. The HCAP Summit convened national infectious disease opinion leaders for the purpose of analyzing current literature, clinical trial data, diagnostic considerations, therapeutic options, and treatment guidelines related to HCAP. After an in-depth analysis of these areas, the infectious disease investigators participating in the summit were surveyed with regard to 10 clinical practice statements. The results were then compared with results of the same survey as completed by 744 Infectious Diseases Society of America members. The similarities and differences between those survey results are the basis of this publication.

Six-minute walk test and health status assessment in sarcoidosis.

BACKGROUND: The 6-min walk test has proved useful in assessing impairment and prognosis in various lung diseases. METHODS: A prospective study of 142 sarcoidosis patients seen during a 6-week period at one tertiary sarcoidosis clinic. All patients completed spirometry, 6-min walk testing, St. George Respiratory Questionnaire (SGRQ), fatigue assessment scale, and dyspnea score. Parameters assessed included the 6-min walk distance (6MWD) and the initial and lowest oxygen saturations during the test. RESULTS: One hundred three of 142 patients had extrapulmonary manifestations, 10 patients had left ventricular dysfunction, and 14 patients had documented pulmonary hypertension. Seventy-three patients (51%) completed a 6MWD < 400 m, and 32 patients (22%) walked < 300 m. The 14 patients with documented pulmonary hypertension had a shorter 6MWD (median, 280 m; range, 61 to 404) than those without documented pulmonary arterial hypertension (median, 411 m; range, 46 to 747; p < 0.0001). Several components of the pulmonary function and quality of life correlated with 6MWD. Using a stepwise multiple regression analysis, only SGRQ activity component (t = - 7.498, p < 0.0001), FVC (t = 4.415, p < 0.0001), and lowest oxygen saturation (t = 2.55, p < 0.02) were independent predictors of 6MWD. CONCLUSIONS: 6MWD was reduced in the majority of sarcoidosis patients. Several factors were associated with a reduced 6MWD, including FVC, oxygen saturation with exercise, and self-reported respiratory health. Both 6MWD and quality of life measures are useful in assessing the functional status of sarcoidosis patients.