First patient-centred set of outcomes for pulmonary sarcoidosis: a multicentre initiative

Kampstra, Nynke A.; Grutters, Jan C.; Beek, Frouke T. van; Culver, Daniel A.; Baughman, Robert P.; Renzoni, Elisabetta; Wuyts, Wim; Kouranos, Vaslis; Wijsenbeek, Marlies; Biesma, Douwe H.; Wees, Philip J. van der; Nat, P. B. van der

doi:10.1136/bmjresp-2018-000394

Cited by 18 publications

(21 citation statements)

References 40 publications

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“…Patient-reported outcome measures and the six-minute walk test have been recommended in disease assessment [30]. A recent international Delphi study supported multi-faceted outcomes to assess disease severity for clinical and research purposes, including spirometry/DLCO, quality of life, assessment of progression as well as a biomarker [31]. These data support the importance of defining a comprehensive biomarker of disease progression.…”

Section: Discussionmentioning

confidence: 95%

Clinical characteristics and organ system involvement in sarcoidosis: comparison of the University of Minnesota Cohort with other cohorts

Perlman

Shenoy

et al. 2020

BMC Pulm Med

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Background: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Clinical cohort studies of different populations are important to understand the high variability in clinical presentation and disease course of sarcoidosis. The aim of the study is to evaluate clinical characteristics, including organ involvement, pulmonary function tests, and laboratory parameters, in a sarcoidosis cohort at the University of Minnesota. We compare the organ system involvement of this cohort with other available cohorts. Methods: We conducted a retrospective data collection and analysis of 187 subjects with biopsy-proven sarcoidosis seen at a tertiary center. Organ system involvement was determined using the WASOG sarcoidosis organ assessment instrument. Clinical phenotype groups were classified using the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis criteria. Results: Mean subject age at diagnosis was 45.8 ± 12.4, with a higher proportion of males (55.1%), and a higher proportion of blacks (17.1%) compared to the racial distribution of Minnesota residents (5.95%). The majority (71.1%) of subjects required anti-inflammatory therapy for at least 1 month. Compared to the A Case Control Etiologic Study of Sarcoidosis cohort, there was a higher frequency of extra-thoracic lymph node (34.2% vs. 15.2%), eye (20.9% vs. 11.8%), liver (17.6% vs. 11.5%), spleen (20.9% vs. 6.7%), musculoskeletal (9.6% vs. 0.5%), and cardiac (10.7% vs. 2.3%) involvement in our cohort. A multisystem disease with at least five different organs involved was identified in 13.4% of subjects. A restrictive physiological pattern was observed in 21.6% of subjects, followed by an obstructive pattern in 17.3% and mixed obstructive and restrictive pattern in 2.2%. Almost half (49.2%) were Scadding stages II/III. Commonly employed disease activity markers, including soluble interleukin-2 receptor and angiotensin-converting enzyme, did not differ between treated and untreated groups.

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Section: Discussionmentioning

confidence: 95%

Clinical characteristics and organ system involvement in sarcoidosis: comparison of the University of Minnesota Cohort with other cohorts

Perlman

Shenoy

et al. 2020

BMC Pulm Med

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“…35 More recently, an outcome measures set was defined for pulmonary sarcoidosis based on a multicenter initiative consisting of seven outcome measures: mortality, pulmonary function, soluble interleukin-2 receptor change as an activity biomarker, weight gain, quality of life, osteoporosis, and clinical outcome status. 36 It is hoped that this will establish a common ground for future studies on both monitoring and treatment of this condition. Similarly, imaging modalities including CT scan with CT activity score and positron emission tomography (PET)/CT are gaining increased attraction as potential tools to measure efficacy of treatment although further studies are needed before recommending either of them.…”

Section: Principles Of Managementmentioning

confidence: 97%

Contemporary optimized practice in the management of pulmonary sarcoidosis

Aryal

2019

Ther Adv Respir Dis

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Pulmonary sarcoidosis is the most common form of sarcoidosis, accounting for the initial presentation in over 70% patients and with eventual presence in 90% of patients with sarcoidosis. However, the course of the disease is often unpredictable; its manifestations can be highly variable and its treatment may not be effective in all patients. As such, the optimized treatment of pulmonary sarcoidosis often requires a thoughtful personalized approach with the need to get the patient involved in decisions of management. In many patients with pulmonary sarcoidosis, the disease is self-limited and nonprogressive, thus treatment is not necessary. In other patients, the presence of significant symptoms or functional limitation often associated with worsening radiological changes and pulmonary function tests warrants treatment. Corticosteroids are the first-line treatment for pulmonary sarcoidosis; antimetabolites are second-line agents, with methotrexate being most commonly employed. Antitumor necrosis alpha antibodies, especially infliximab, are emerging as potential third-line agents. A high index of suspicion should be held for pulmonary hypertension and other comorbidities that may complicate the course of patients with advanced sarcoidosis. Lung transplantation may be the only option for patients who have refractory disease despite maximal medical therapy.

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“…A recent multinational initiative, evaluating the feasibility of a set of the best standardized practice outcome measures for patients with sarcoidosis, has proposed that the KSQ and Fatigue Assessment Score (vide infra) be used as the standard PROMs for assessing HRQoL in patients with pulmonary sarcoidosis. 51,52 The Sarcoidosis Assessment Tool…”

Section: Sarcoidosis Health Questionnairementioning

confidence: 99%

“…153 Subsequent studies have, however, shown the FAS to be a robust instrument and a recent multinational initiative has recommended the FAS as the PROM of choice for assessing fatigue in sarcoidosis. 51,52 Studies utilizing the FAS have shown that sarcoidosisassociated fatigue occurs more commonly in females, 152,155 and has been associated with an increased sense of dyspnea, reduced 6MWD, and reduced exercise tolerance. 67 It is more severe in patients with pulmonary and extrapulmonary disease versus those with isolated pulmonary involvement.…”

Section: Fatigue Assessment Scalementioning

confidence: 99%

“…5,33,[48][49][50] A recent multinational initiative involving six expert interstitial lung disease (ILD) clinics in four countries includ-ing the U.S. identified HRQoL as one of seven core desired outcome measures to monitor in patients with pulmonary sarcoidosis. 51,52 At the 2011 World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) meeting, it was strongly recommended that everyday assessment of sarcoid patients and all sarcoid clinical trials incorporate HRQoL assessments through the use of patient-reported outcome measures (PROMs). 53,54…”

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confidence: 99%

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Health-Related Quality of Life in Sarcoidosis

Ndili

2020

Semin Respir Crit Care Med

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Health-related quality of life (HRQoL) describes an individual's perception of the impact of health, disease, and treatment on their quality of life (QoL). It is a reflection of how the manifestation of an illness and its treatment is personally experienced. Assessing HRQoL is particularly important in sarcoidosis because the attributable disease mortality is relatively low, and one of the major reasons for initiating treatment is to improve quality of life. HRQoL has been assessed in sarcoidosis using various generic and sarcoid-specific patient-reported outcome measures (PROMs). It is important that both the direct and indirect effects of the disease, as well as potential toxicities of therapy, are captured in the various PROMs used to assess HRQoL in sarcoidosis. This article provides a general overview of HRQoL in patients with sarcoidosis. It describes the various PROMs used to assess HRQoL in sarcoidosis and addresses the various factors that influence HRQoL in sarcoidosis. Specific attention is paid to fatigue, small fiber neuropathy, corticosteroid therapy, and other disease-specific factors that affect HRQoL in sarcoidosis. It also provides an insight into interventions that have been associated with improved HRQoL in sarcoidosis and offers suggestions for future research in this important area.

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First patient-centred set of outcomes for pulmonary sarcoidosis: a multicentre initiative

Cited by 18 publications

References 40 publications

Clinical characteristics and organ system involvement in sarcoidosis: comparison of the University of Minnesota Cohort with other cohorts

Clinical characteristics and organ system involvement in sarcoidosis: comparison of the University of Minnesota Cohort with other cohorts

Contemporary optimized practice in the management of pulmonary sarcoidosis

Health-Related Quality of Life in Sarcoidosis

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