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Background Clinical manifestations of sarcoidosis vary widely, depending on the intensity of the inflammation and the organ systems affected. So far, no curative treatment exists; the disease can only be suppressed. All treatment options cause side effects affecting quality of life. The aim of this study was to establish and rank the prevalence of self-reported gastrointestinal side effects of drugs used in the treatment of sarcoidosis. Methods A cross-sectional web-based anonymous survey about complaints and side effects was conducted among sarcoidosis patients in the Netherlands, United Kingdom, and United States of America. Results Of the participants, 70% were being treated with one or more drugs. The most important reported side effect was weight gain, associated with increased appetite among prednisone users (as monotherapy as well as in combination with other drugs). Methotrexate (MTX) users especially experienced nausea, with monotherapy as well as combination therapy. Vomiting and weight loss were most prominent among azathioprine and mycophenolate mofetil (MMF) users, whereas diarrhoea was frequently mentioned by MMF and MTX users. The reported side effects of hydroxychloroquine were generally rather mild. Conclusion The current study ranked the gastrointestinal side effects associated with pharmacotherapy in sarcoidosis patients. Pharmacotherapy does have multiple gastrointestinal side effects. The strongest association between a reported side effect and drug use was that of weight gain associated with increased appetite among prednisone users. It would therefore be useful for future research to look further into dietary interventions to counter these side effects and reduce their burden., Author(s): M. Drent [sup.1] [sup.2] [sup.3] , V. L. J. Proesmans [sup.3] [sup.4] , M. D. P. Elfferich [sup.3] , N. T. Jessurun [sup.3] [sup.5] , S. M. G. de [...]

Purpose Idiopathic pulmonary fibrosis (IPF) is an inexorably progressive disease, which has a great impact on patients' lives. Pirfenidone and nintedanib are approved and recommended antifibrotic drugs for patients with IPF. The aim of this study was to evaluate self-reported gastrointestinal side effects of antifibrotic drugs in 176 Dutch IPF patients. Methods A cross-sectional web-based anonymous survey about complaints and side effects was conducted among IPF patients in the Netherlands. Logistic regression was used to quantify whether pirfenidone and nintedanib caused complaints of nausea, vomiting, diarrhoea, appetite loss, weight loss or loss of taste or smell perception. Results The questionnaire was completed by 176 IPF patients, 71 of whom used pirfenidone and 85 nintedanib, while 20 patients did not use any antifibrotic drugs. Nintedanib users reported complaints of diarrhoea, vomiting, weight loss and loss of appetite (p < 0.01). Nausea was a significant adverse reaction (p < 0.05). Pirfenidone caused increased appetite loss (p < 0.01) and the risk of weight loss (p < 0.05). The increase in loss of appetite and weight loss did not differ significantly between the two drugs. Conclusion The current study showed that nintedanib causes a significant increase in diarrhoea, vomiting, weight loss and loss of appetite, while pirfenidone led to loss of appetite. Our results suggest new avenues regarding dietary recommendations for IPF patients., Author(s): V. L. J. Proesmans [sup.1] [sup.4] , M. Drent [sup.2] [sup.3] [sup.4] , M. D. P. Elfferich [sup.4] , P. A. H. M. Wijnen [sup.4] [sup.5] , N. T. [...]

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., Author(s): C. M. R. Hendriks [sup.1] [sup.2] [sup.3] , L. A. Saketkoo [sup.4] [sup.5] [sup.6] , M. D. P. Elfferich [sup.1] , J. De Vries [sup.1] [sup.7] [sup.8] , P. [...]

Background: Sarcoidosis patients suffer from fatigue and exercise limitation. The aim of this study was to establish whether a physical training program improves these and other outcomes important to sarcoidosis patients., Methods: From 11/2012 to 9/2014, 201 sarcoidosis patients were referred to the ild care expertise team, Ede, the Netherlands. In our center, all patients are routinely recommended to undergo testing at baseline to determine their physical functioning and encouraged to complete a 12-week, supervised physical training program. Ninety patients underwent baseline testing and returned for repeat testing at 3 months in the interim, 49 completed the training program (Group I) and 41 chose not to participate (Group II). Change over time (from baseline to 3 months) in fatigue, exercise capacity, and skeletal muscle strength were assessed between the two groups., Results: At baseline, there were no between-group differences for fatigue, DLCO%, FVC%, or exercise capacity [assessed by percent predicted six-minute walk distance (6MWD%) and Steep Ramp Test (SRT)]. The 6MWD for Group I improved between baseline and 3 months, while the 6MWD remained the same in Group II (F = 72.2, p < 0.001). Group I showed a significantly larger decrease of fatigue compared with Group II (F = 6.27, p = 0.014). Lung function tests did not change in either group., Conclusion: A supervised physical training program improves exercise capacity and fatigue among sarcoidosis patients and should be included in their management regimen.

Purpose: The purpose of this study was to examine changes in the prevalence of exercise intolerance, reduced muscle strength, and fatigue and the changes in these parameters in individual patients during a 2-year follow-up study., Methods: Ninety sarcoidosis patients (62 males and 28 females; mean age: 46.0 ± 10.2 years) participated in a 2-year follow-up study. At the baseline and follow-up measurements, patients performed a 6-min walk test and elbow flexor muscle strength, quadriceps peak torque, and hamstrings peak torque tests. Maximal inspiratory pressure was recorded. All patients completed the Fatigue Assessment Scale., Results: Both at baseline and follow-up, a substantial proportion of the patients showed a reduced 6-minute walk test (41.6 and 34.8 %, respectively), elbow flexor muscle strength (6.7 and 14.6 %), quadriceps peak torque (21.3 and 18 %), hamstrings peak torque (13.5 and 12.4 %), and maximal inspiratory pressure (45.9 and 48.6 %). The majority of the patients reported fatigue (86 and 77 %). These physical impairments remained stable during the follow-up period. The prevalence of these physical impairments in patients diagnosed with sarcoidosis <2 years before inclusion in this study was similar to that in patients with a longer history of the disease., Conclusions: Exercise intolerance, muscle weakness, and fatigue are frequent problems in symptomatic sarcoidosis patients with a stable and persistent character. This study highlights that beyond medical treatment a rehabilitation program should be considered as adjunct therapy in the multidisciplinary management of sarcoidosis patients even though the achieved benefit needs future studies.

Background: Fibrosing interstitial pneumonias (IPs) include idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP). It has been suggested that oxidative damage plays a role in the pathophysiology of idiopathic interstitial pneumonias. Diffuse alveolar hemorrhage (DAH) can cause oxidative stress. Accordingly, we hypothesized that episodes of DAH might trigger fibrosing IP development., Methods: Patients using coumarins with confirmed DAH were retrospectively gathered during a 9 year period and reviewed for the development of IPF or fibrosing NSIP., Results: A total of 65 patients with DAH could finally be included, 31 (48 %) of whom subsequently developed a fibrosing IP. The majority of these 31 patients developed the fibrosing IP within 3 years after DAH confirmation. A total of 41 (63 %) patients died within 3.0 ± 0.9 (range 1.3-4.7) years after the DAH diagnosis had been confirmed. Twenty-two of the deceased (54 %) had finally developed fibrosing IP., Conclusions: Almost half of the patients with established episodes of DAH developed fibrosing IP; therefore it seems that DAH might be a trigger for the development of fibrosing IP. This observation warrants prospective studies to further evaluate the clinical impact of these findings.

Background: Cardiopulmonary exercise testing (CPET) with blood gas analysis may be helpful when there is a discrepancy between clinical findings and physiologic tests at rest. The aim of this study was to examine the added value of CPET compared to the measurement of the diffusing capacity of the lung for carbon monoxide (DLCO) in detecting impaired pulmonary gas exchange in sarcoidosis patients., Methods: The clinical records of 160 (age = 41.3 ± 10.0 years; number of females = 63) sarcoidosis patients referred to the former MUMC ild care center were retrospectively reviewed. Patients performed a symptom-limited incremental exercise test with blood gas analysis on a bicycle ergometer. DLCO was measured by the single-breath method., Results: DLCO (mean = 83.2 ± 18.0 %) below 80 % of predicted was demonstrated by 38 % of the sarcoidosis patients in our sample. Of the patients with normal DLCO (n = 99, 61.9 %), the P(A-a)O(2) at maximal exercise [P(A-a)O(2)max] was moderately increased (>2.5 kPa) in 69.7 % and excessively increased (>4.7 kPa) in 18.2 %. Pulmonary gas exchange impairment (PGEI) was more obvious in patients with lower DLCO values. A DLCO value below 60 % of predicted indicated substantial gas exchange impairment. PaO(2) at rest, DLCO, and FVC as a percentage of predicted and radiographic staging predicted 40 % of the PGEI at maximal exercise., Conclusion: A substantial number of the symptomatic sarcoidosis patients with normal DLCO appeared to have PGEI at maximal exercise, suggesting that normal DLCO at rest is an inappropriate predictor of abnormal pulmonary gas exchange during exercise. CPET appeared to offer added value in detecting impaired gas exchange during exercise in sarcoidosis patients with unexplained disabling symptoms.