Predictors of cough-specific and generic quality of life in sarcoidosis patients.

BACKGROUND: Cough is frequent symptom in sarcoidosis and its impact on patient's quality of life (QoL) has not been adequately addressed so far. OBJECTIVES: The goal of this study was to determine the significant predictors of cough-specific and generic QoL in sarcoidosis patients. METHODS: In the prospective study 275 sarcoidosis patients administered Patient Reported Outcomes instruments for measurement of dyspnea (Borg and MRC scales) and fatigue (Fatigue Assessment Scale (FAS) and Daily Activity List (DAL)), as well as patients' QoL (cough-specific Leicester Cough Questionnaire (LCQ) and generic tool - 15D). The LCQ contains 3 domains covering physical, psychological and social aspects of chronic cough. Pulmonary function tests (spirometry and diffusing capacity for carbon monoxide) and serum angiotensin converting enzyme (sACE) were also measured. RESULTS: Dyspnea measured by Borg scale and impairment of daily activities determined by DAL instrument as well as sACE were the strongest predictors of all cough-specific QoL domains. Mental aspect of patients' fatigue was significantly correlated with all domains except with psychological LCQ domain. Regarding the generic QoL, the following significant predictors were: dyspnea measured by MRC scale, overall fatigue determined by FAS and physical domain of the LCQ. CONCLUSION: It is important to measure both cough-specific and generic QoL in sarcoidosis patients since they measure different health aspects and their predictors can be different. We demonstrated that physical domain of cough-specific QoL is significant predictor of generic QoL. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (2): 158-168).

Sarcoidosis and Work Participation: The Need to Develop a Disease-Specific Core Set for Assessment of Work Ability.

OBJECTIVE: Sarcoidosis, an inflammatory multi-organ disease with a wide variety of clinical manifestations, affecting people of working age. Patients suffer from a broad spectrum of physical symptoms of varying severity that impact function including cognitive impairment and disabling fatigue. The Dutch Sarcoidosis Society identified a knowledge gap in various facets related to work ability. The aim of this study was to assess sarcoidosis patients' perceived problems related to work performance, employer, and disability evaluations. METHODS: A cross-sectional web-based anonymous survey was conducted among Dutch sarcoidosis patients recruited through sarcoidosis patient societies and outpatient sarcoidosis clinics. This investigation queried work performance, employer support, and disability evaluations. RESULTS: The study sample included 755 patients of whom 43% (n = 328) had undergone disability evaluation and were significantly more likely to experience extrapulmonary symptoms, severe fatigue, reduced exercise capacity along with memory problems and concentration problems with higher mean FAS and SFNSL-scores. Of these 328, 37% (n = 121) perceived they had not been listened to or taken seriously at assessments, and 38% (n = 124) disagreed with the outcome of disability assessments by benefits authorities; 75% (n = 93) appealed or requested re-assessment. DISCUSSION: A better understanding of sarcoidosis-related impact on work ability and quantification of disease burden is needed. Education for medical examiners and employers on sarcoidosis may improve quality of assessments and work accommodations. Development of guidelines for benefit authorities, which consider the broad impact of sarcoidosis beyond that of reduced pulmonary function, including extra-pulmonary assessment like fatigue, cognitive difficulties, as well as other organ involvement are needed.

The Assessment of Cough in a Sarcoidosis Clinic Using a Validated instrument and a Visual Analog Scale.

PURPOSE: Cough is a common symptom of pulmonary sarcoidosis. We analyzed the severity of cough and factors associated with cough in a university sarcoidosis clinic cohort. METHODS: Consecutive patients completed the Leicester Cough Questionnaire (LCQ) and a cough visual analog scale (VAS). Clinical and demographic data were collected. Means of the LCQ were analyzed in patients who had multiple visits in terms of constant variables (e.g., race, sex). RESULTS: 355 patients completed the LCQ and VAS at 874 visits. Cough was significantly worse in blacks than whites as determined by the LCQ-mean (16.5 ± 2.6 vs. 17.8 ± 3.0, p < 0.001) and VAS-mean (3.8 ± 3.0 vs. 2.0 ± 2.6, p < 0.0001). Cough was worse in women than men as measured by the VAS-mean (2.7 ± 2.9 vs. 2.2 ± 2.7, p = 0.002), one of the LCQ-mean domains (LCQ-Social-mean 5.4 ± 0.9 vs. 5.2 ± 1.0, p = 0.03), but not the total LCQ-mean score. Cough was not significantly different by either measure in terms of smoking status, age, or spirometric parameter (FVC % predicted, FEV1 % predicted, FEV1/FVC). In a multivariable linear regression analysis, cough was significantly worse in blacks than whites and in pulmonary sarcoidosis than non-pulmonary sarcoidosis with both cough measures, in women than men for the VAS only, and not for spirometric parameters, Scadding stage, or age. The LCQ and VAS were strongly correlated. CONCLUSIONS: In a large university outpatient sarcoidosis cohort, cough was worse in blacks than whites. Cough was not statistically significantly different in terms of age, spirometric measures, Scadding stage, or smoking status. The LCQ correlated strongly with a visual analog scale for cough.

Assessment of right ventricular systolic and diastolic parameters in pulmonary sarcoidosis.

The clinical manifestations of cardiac involvement are seen in about 5% of patients with sarcoidosis; however, the incidence of cardiac involvement is higher in the autopsy series. About 14% of patients with pulmonary sarcoidosis (PS) without known cardiac involvement had diastolic dysfunction.We aimed to determine the role of parameters of right ventricular (RV) systolic and diastolic function in patients with PS without evidence of cardiac symptoms. Our study population consisted of 28 patients with grades 1-4 PS and 24 healthy subjects. This study was a clinical prospective cohort study. RV end-diastolic area was found to be significantly higher in the PS group (p=0.032). RV fractional area change (RVFAC) and tricuspid annular plane systolic excursion (TAPSE) were shown to be statistically lower in the PS group as compared to the control group (p<0.001). However, pulmonary arterial systolic pressure was significantly higher in the PS group (p=0.003). The tricuspid E velocity and E/A ratio were found to be significantly lower in the PS group (p=0.025 and 0.009, respectively), while the tricuspid A velocity and myocardial performance index (MPI) were found to be significantly lower in the control group (p=0.034 and 0.007, respectively). Early detection of cardiac involvement in PS is crucial because of the increased morbidity and risk of sudden cardiac death. RV diastolic Doppler parameters, tissue Doppler MPI, RVFAC and TAPSE are practical and cheap techniques in the diagnosis of cardiac involvement in patients with PS. A thorough transthorasic echocardiographic examination including RV systolic and diastolic functions and tissue Doppler MPI should constitute the mainstay of initial management and follow-up in PS.

Patient confidence and quality of life in idiopathic pulmonary fibrosis and sarcoidosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) and sarcoidosis impact significantly on health-related quality of life (HRQOL). There are few studies on the impact of patient confidence on HRQOL in these conditions. OBJECTIVES: 1. To investigate whether patient confidence is associated with HRQOL, anxiety, depression, dyspnoea or fatigue. 2. To assess if patient confidence is associated with inpatient admissions, access to community healthcare and, for IPF patients, mortality and disease severity. METHODS: Study participants self-completed seven questionnaires: Hospital Anxiety and Depression Scale, EuroQol 5D (EQ5D), King's Brief Interstitial Lung Disease questionnaire, St George's Respiratory Questionnaire, MRC dyspnoea scale, Fatigue Assessment Scale and a non-validated questionnaire assessing patient confidence, symptom duration and access to community healthcare. Lung function and follow-up data were collected from hospital electronic databases. Spearman's rank correlation coefficients were calculated to assess for correlation between patient confidence, questionnaire variables and inpatient admissions. Chi-square tests were performed to assess for association between patient confidence, mortality and disease severity. RESULTS: 75 IPF patients and 69 sarcoidosis patients were recruited to the study. Patient confidence in IPF was significantly negatively correlated with depression and fatigue, and significantly positively correlated with EQ5D scores, but not healthcare outcomes. No associations were found between confidence and any of the variables assessed in sarcoidosis. CONCLUSIONS: Lower levels of confidence in IPF patients are associated with higher levels of depression and fatigue and worse HRQOL. Efforts should be made to improve patient confidence to assess the impact on HRQOL.

The effect of corticosteroids on quality of life in a sarcoidosis clinic: the results of a propensity analysis.

BACKGROUND: Both sarcoidosis and its treatment may worsen health related quality of life (HRQoL). We performed a propensity analysis of sarcoidosis-specific HRQoL patient reported outcome measures (PRO) to disentangle the effects of sarcoidosis and corticosteroid therapy on HRQoL in sarcoidosis outpatients. METHODS: Consecutive outpatient sarcoidosis patients were administered modules from two sarcoidosis-specific HRQoL PROs: the Sarcoidosis Health Questionnaire (SHQ) and the Sarcoidosis Assessment Tool (SAT). Patients were divided into those that received ≤500 mg of prednisone (PRED-LOW) versus >500 mg of prednisone (PRED-HIGH) over the previous year. SAT and SHQ scores were initially compared in the two corticosteroid groups. Then a multivariate analysis was performed using a propensity score analysis adjusted for race, age, gender and the severity of illness. RESULTS: In the unadjusted analysis, the PRED-HIGH group demonstrated the following worse HRQoL scores compared to the LOW-PRED group: SHQ Daily (p = 0.02), SAT satisfaction (p = 0.03), SAT daily activities (p = 0.03). In the propensity analysis, the following domains demonstrated worse HRQoL in the PRED-HIGH group than the PRED-LOW group: SAT fatigue (p < 0.0001), SAT daily activities (p = 0.03), SAT satisfaction (p = 0.03). All these differences exceeded the established minimum important difference for these SAT domains. The SHQ Physical score appeared to demonstrate a borderline improved HRQoL in the PRED-HIGH versus the PRED-LOW group (p = 0.05).). In a post-hoc exploratory analysis, the presence of cardiac sarcoidosis may have explained the quality of life differences between the two corticosteroid groups. CONCLUSIONS: Our cohort of sarcoidosis clinic patients who received ≤500 mg of prednisone in the previous year had an improved HRQoL compared to patients receiving >500 mg on the basis of two sarcoidosis-specific PROs after adjusting for severity of illness. These data support the need to measure HRQoL in sarcoidosis trials, and suggest that the search should continue for effective alternative medications to corticosteroids.

Recent developments in the physiological assessment of sarcoidosis: clinical implications.

PURPOSE OF REVIEW: This review emphasizes key findings in physiologic research of sarcoidosis reported over the past year. RECENT FINDINGS: Sarcoidosis, a multiorgan disease involving the formation of epithelioid-cell granulomas, is characterized by reduced lung volumes, compliance, and diffusion capacity (D(L)CO), and, in a small number of cases, by airflow limitation. Recent studies do not show a close relationship between changes in lung volume and radiographic stage. Fatigue and exercise limitation are characteristic of this condition, and can be assessed by health-related quality of life (HRQOL) instruments. Recent investigations have focused on the evaluation of the extent of parenchymal and nodal inflammatory activity by PET using 18F-fluorodeoxyglucose (FDG-PET imaging). Pulmonary hypertension in advanced cases of sarcoidosis contributes to increased physical impairment, and decreased HRQOL and survival. It is best associated with ambulatory desaturation, reduced D(L)CO, and abnormal cardiopulmonary exercise testing findings indicative of pulmonary vascular disease. If pulmonary hypertension is suspected, it should be screened for by echocardiography and confirmed by right heart catheterization. Selected patients with progressive disease unresponsive to medical therapy or with severe pulmonary hypertension should be considered for lung transplantation. Current criteria for lung transplantation include New York Heart Association functional class III-IV, pulmonary hypertension, and/or right atrial pressure at least 15  mmHg. SUMMARY: Periodic assessment of HRQOL measures, exercise-induced hypoxemia, and right-sided cardiac pressures for pulmonary hypertension provides, to date, the best insight into the magnitude of physiologic impairment, serving as guideposts for management (including lung transplantation) and prognosis.

Assessment of respiratory mechanics in patients with sarcoidosis using forced oscillation: correlations with spirometric and volumetric measurements and diagnostic accuracy.

BACKGROUND: The forced oscillation technique (FOT) is a promising method for providing a detailed analysis of respiratory mechanics during spontaneous breathing. There is limited data about the use of FOT in patients with sarcoidosis. OBJECTIVES: The aims of this study were to test the ability of FOT to describe the changes in respiratory mechanics in sarcoidosis and to evaluate the clinical potential of FOT. METHODS: Twenty healthy subjects and 31 patients were studied. All subjects performed spirometric exams, and pulmonary volumes were measured in patients. Resistive data were interpreted using the zero-intercept resistance (R0), the slope of the resistive component of the impedance (S), and the mean resistance (Rm). The analysis of reactance was used to measure the mean reactance (Xm) and the respiratory system dynamic compliance (C(dyn)). The total mechanical load was evaluated using the absolute value of the respiratory impedance (Z4Hz). RESULTS: In close agreement with pathophysiological fundamentals, significant (p < 0.001) increases in R0, Rm, and Z4Hz, and reductions in C(dyn) (p < 0.003) were observed. S and Xm presented smaller modifications (p < 0.02). All FOT parameters were significantly correlated with spirometric indices. Z4Hz was the most adequate parameter for clinical use (Se = 75%, Sp = 75%), followed by R0 and Rm. CONCLUSIONS: FOT parameters were consistent with the pathophysiology of sarcoidosis and were also able to detect the effects of this disease. Because the FOT is easy to perform, such a technique may represent an alternative and/or a complement to other conventional exams to help the clinical evaluations of sarcoidotic patients.

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.

Pulmonary sarcoidosis: role of CT in the evaluation of disease activity and functional impairment and in prognosis assessment.

PURPOSE: To investigate the role of computed tomography (CT) in determining disease activity and functional impairment and in predicting the prognosis of lung involvement in patients with sarcoidosis. MATERIALS AND METHODS: Two groups of patients underwent CT, pulmonary function tests, and bronchoalveolar lavage. Disease activity was based on serum angiotensin converting enzyme activity and/or lymphocytosis at bronchoalveolar lavage. CT findings in group 1 were correlated with indexes of disease activity and functional parameters at diagnosis. In group 2, evaluation initially and at follow-up led to investigation of correlations between initial CT findings and evolution of disease activity and functional impairment. RESULTS: CT abnormalities in group 1 were nodules, air-space consolidation, lung distortion, septal and nonseptal linear areas of high attenuation, ground-glass attenuation, and honeycombing. Profusion of septal lines was the only CT finding that correlated with disease activity. Statistically significant (P < .05) but low correlation (r < .48) was observed between the extent of abnormalities at CT and impairment of lung function, except for nodules. CONCLUSION: Profusion of lung changes reflects functional impairment but not disease activity. CT findings cannot help predict the evolution of lung changes over time.