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The current study aimed to evaluate the psychometric properties of the Dutch version of the Multiple Sclerosis Work Difficulties Questionnaire-23 (MSWDQ-23). Two hundred and thirty-nine employed persons with multiple sclerosis (MS) and 59 healthy controls completed the MSWDQ-23. To verify the factor structure, a confirmatory factor analysis was conducted. To assess construct validity, the MSWDQ-23 scores were correlated to measures of physical disability, fatigue, cognitive and neuropsychiatric problems, depression, health-related quality of life, and work-related variables. MSWDQ-23 scores were compared within different age groups, gender, education levels, and job types. Predictive validity was assessed using a logistic regression analysis to predict a deterioration in employment status after one year based on MSWDQ-23 scores. The internal consistency of the MSWDQ-23 was acceptable (α = 0.913, 95% CI = 0.897–0.928) and the results indicated a fair fit. The MSWDQ-23 showed acceptable construct validity, confirming 94% of the hypotheses. The total scale and the psychological/cognitive subscale were able to predict a deterioration in employment status after one year (χ2(1)=18.164, p < 0.001). The Dutch version of the MSWDQ-23 is a valid and internally consistent instrument to measure self-reported work difficulties in persons with MS. The Dutch version of the 23-item Multiple Sclerosis Work Difficulties Questionnaire (MSWDQ-23) is a reliable and valid tool to measure self-reported work difficulties in people with multiple sclerosis (MS). More psychological and cognitive work difficulties are predictive of a deteriorated employment status after one year. The MSWDQ-23 is a helpful tool for researchers and (occupational) health professionals to identify current work difficulties in persons with MS and identify persons at risk for a deterioration in employment one year later. [ABSTRACT FROM AUTHOR]

It has been suggested to avoid drug-induced lymphopenia as this might be a risk factor for contracting COVID-19 or for a more severe COVID-19 course in MS patients.[2] In a French COVID-19 MS cohort, lymphocyte counts prior to COVID-19 were not associated with the severity of COVID-19 course.[3] However, when excluding fingolimod-induced lymphopenia, the authors did find an association with more severe course of COVID-19 and lymphopenia. With interest we read the comments of Evangelou and Garjani regarding our report of coronavirus disease 2019 (COVID-19) in the Dutch MS population.[1] In our cohort, we could not find an association regarding lymphopenia and severity of COVID-19 course. We collected lymphocyte counts that were sampled half a year prior to the first COVID-19 case in the Netherlands in patients without known COVID-19 infection later on during the pandemic. [Extracted from the article]

Here, we provide an extensive overview of all reported COVID-19 cases in multiple sclerosis (MS) patients in the Netherlands between 27 February and 9 June 2020, gathered by the Dutch MS Taskforce of the Netherlands Society of Neurology. A total of 86 MS patients were reported, 43 of whom tested positive for COVID-19. Of 43 patients who tested positive, 22 patients were hospitalized. Three intensive care unit (ICU) admissions and four deaths were reported. Our findings show no apparent difference in disease-modifying treatment (DMT) use and COVID-19 disease course in Dutch MS patients. In addition, a clear link between low lymphocyte count and severe disease was not observed.

Background: Previous studies found that 40-60% of the sarcoidosis patients suffer from small fiber neuropathy (SFN), substantially affecting quality of life. SFN is difficult to diagnose, as a gold standard is still lacking. The need for an easily administered screening instrument to identify sarcoidosis-associated SFN symptoms led to the development of the SFN Screening List (SFNSL). The usefulness of any questionnaire in clinical management and research trials depends on its interpretability. Obtaining a clinically relevant change score on a questionnaire requires that the smallest detectable change (SDC) and minimal important difference (MID) are known. Objectives: The aim of this study was to determine the SDC and MID for the SFNSL in patients with sarcoidosis. Methods : Patients with neurosarcoidosis and/or sarcoidosis-associated SFN symptoms (N=138) included in the online Dutch Neurosarcoidosis Registry participated in a prospective, longitudinal study. Anchor-based and distribution-based methods were used to estimate the MID and SDC, respectively. Results: The SFNSL was completed both at baseline and at 6-months' follow-up by 89/138 patients. A marginal ROC curve (0.6) indicated cut-off values of 3.5 points, with 73% sensitivity and 49% specificity for change. The SDC was 11.8 points. Conclusions: The MID on the SFNSL is 3.5 points for a clinically relevant change over a 6-month period. The MID can be used in the follow-up and management of SFN-associated symptoms in patients with sarcoidosis, though with some caution as the SDC was found to be higher. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 333-341) ., (Copyright: © 2018 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.)

Background: The King's Sarcoidosis Questionnaire (KSQ) is a brief questionnaire assessing health status using five modules (General Health Status, Lung, Eyes, Skin, Medication) in patients with sarcoidosis. The KSQ was only validated in one English sarcoidosis cohort., Objective: The aim of this study was to validate the KSQ in a Dutch sarcoidosis population., Methods: The KSQ was translated according to international guidelines and tested in interviews with patients. Consecutive outpatients completed multiple questionnaires twice, two weeks apart. Construct validity, internal consistency and repeatability were determined., Results: Of the 98 patients included 85 had lung, 22 skin and 24 eye disease. There was good construct validity of the KSQ General Health Status module against the World Health Organization Quality of Life-BREF questionnaire. The Medication module correlated weak to moderate with most questionnaires. The correlations with organ-specific questionnaires varied from strong for Eyes (r=0.75), Skin (r=-0.62) to moderate for Lung (r=-0.45 with MRC breathlessness scale). Internal consistency was good for all KSQ modules (Cronbach's α 0.72-0.93). Intraclass correlation coefficients (0.70-0.90) and Bland-Altman plots showed good repeatability of the KSQ., Conclusion: The Dutch KSQ is the first translation of the English KSQ, validated in a Dutch sarcoidosis population.

The clinical spectrum of neurosarcoidosis is highly diverse; virtually any neurological syndrome can present, depending upon granuloma localization. There are indications that neurosarcoidosis is insufficiently recognized as a separate clinical entity. In the absence of major prospective clinical trials, we launched a large national online registry of neurosarcoidosis patients in the Netherlands in June 2014 at www.neurosarcoidose.nl. When a patient with sarcoidosis presents with neurological symptoms that may compatible with sarcoidosis, it is important to locate sites of disease activity accessible for biopsy outside the nervous system. Small nerve fibre neuropathy is a common neurological complication of sarcoidosis. It is considered an epiphenomenon, and therefore classified as 'para-neurosarcoidosis'. Neurological symptoms causing disability are an indication for immunosuppressive therapy. There is increasing evidence for the effective treatment of refractory neurosarcoidosis using biologicals. Whether the intravenous administration of immunoglobulins or ARA290, an erythropoietin derivative with TNF-α-antagonistic and tissue-protective characteristics, is effective for small nerve fibre neuropathy in sarcoidosis is subject for study.

Responsiveness to tumour necrosis factor (TNF) inhibitors has been associated with the TNF-α G-308A polymorphism in rheumatoid arthritis. The aim of this study was to examine the association between the presence of this polymorphism and the response to TNF inhibitors in patients with refractory sarcoidosis. Patients (n=111) who started TNF-inhibitor treatment (76 infliximab, 35 adalimumab) were followed for at least 1 year. The main symptoms in these patients were fatigue (n=100, 90.1%), small fibre neuropathy (n=91, 82.0%), pulmonary involvement (n=69, 62.2%), and/or uveitis (n=31, 27.9%). Patients were additionally genotyped for the presence of the TNF-α G-308A polymorphism. Treatment response was assessed using clinical outcome measures and questionnaires. Three-quarters (n=83, 74.8%) of the patients responded well. Of the patients without the variant A-allele 93.6% (73 out of 78, p<0.001) improved, while 30.3% (10 out of 33) of variant A-allele carriers responded favourably to TNF inhibitors. For patients with the GG-genotype, the probability of improving compared with remaining stable or deteriorating was three times higher (risk ratio 3.09, 95% CI 1.84-5.20). Sarcoidosis patients without the TNF-α -308A variant allele (GG-genotype) had a three-fold higher response to TNF inhibitors (adalimumab or infliximab). Further research is needed to evaluate the value of genotyping for the TNF-α G-308A polymorphism in order to tailor TNF-inhibitor treatment., (©ERS 2014.)

We describe a patient with severe small fiber neuropathy (SFN) accompanied by autonomic involvement, who was experimentally treated with infliximab, an anti-tumour necrosis factor-alpha (TNF-alpha) therapy. Six months after this treatment was started his symptoms completely resolved. Until now they did not return. Repeated temperature threshold testing (TTT) as well as cardiovascular autonomic function test clearly improved after one year therapy. This case reveals two important issues. First, it shows that SFN seems not an irreversible disorder, even in severe cases. Second, TNF-alpha may be a crucial cytokine in the pathogenesis of SFN in sarcoidosis and eventually also in other immune mediated inflammatory diseases.

Background and Aim: Sarcoidosis has been reported to be associated with the HLA genes, in particular DQB1., Methods: High resolution DQB 1 of 103 sarcoidosis patients was obtained by sequence-based typing; low resolution HLA-A/B/DRB 1 typing was performed by serological and molecular methods. Small fiber neuropathy (SFN) was established by thermal threshold testing., Results: Sixty-seven patients suffered from SFN (SFN+), in 36 patients SFN was absent (SFN-). Comparing HLA DQB 1 typings of SFN+ patients, SFN- patients and control individuals revealed a significant increase of the allele DQB 1 0602 in SFN+ patients compared to controls., Conclusion: This association might be correlated with a severe course of the disease.

Background and Aim: Recently we found that small fiber neuropathy (SFN) occurs frequently in sarcoidosis. Autonomic dysfunction may be a feature of SFN. Since cardiac autonomic dysfunction has been identified as a strong predictor of morbidity and mortality, recognition of cardiac autonomic involvement is of clinical relevance. It was hypothesised that SFN might be related to cardiac sympathetic denervation in sarcoidosis., Methods: In the present study 45 consecutive sarcoidosis patients (13 without SFN assessed by thermal threshold testing (TTT), 32 with SFN (abnormal TTT) were enrolled. To rule out bias due to myocardial ischemia, cases with abnormal Thallium (201Tl) perfusion scintigraphy were excluded (n = 2). Cardiovascular autonomic function testing (Ewing tests) and 123I-MIBG (metaiodobenzylguanidine) scintigraphy were used to assess cardiac autonomic function. Further cardiac diagnostic work-up included ECG, Holter recording and echo Doppler cardiography., Results: Mild to moderate heterogeneity of 123I-MIBG uptake regional in the myocardium was demonstrated in a substantial number of the studied sarcoidosis population, especially in those with SFN (abnormal TTT). Mean inferior-anterior ratios were 0.85+/-0.17 (SFN) and 1.0+/-0.17 (no SFN; p = 0.003), respectively. Four out of the 14 cases with abnormal MIBG scintigraphy and SFN showed an abnormal Ewing test., Conclusion: Cardiac sympathetic dysfunction assessed by use of 123I-MIBG myocardial scanning appeared to be heterogeneous in sarcoidosis patients and dependent on the presence or absence of SFN. MIBG scintigraphy may be of additional value in the management and follow-up of sarcoidosis patients. Future study is warranted to explore possible prognostic and therapeutic implications of these findings in sarcoidosis.

Background and Aim: Although pain is prevalent in sarcoidosis, this has never been studied systematically. The aim of the present study was to evaluate the presence and impact of pain in sarcoidosis., Methods: Members from the Dutch Sarcoidosis Society without co-morbidity (n = 821) participated in this study. The World Health Organisation Quality of Life assessment instrument (WHOQOL-100) was completed, as well as a symptom inventory questionnaire addressing the presence of various categories of pain, i.e., muscle pain, chest pain, abdominal pain, arthralgia, and/or headache., Results: Pain was reported by 594 patients (72.4%). Arthralgia was experienced most frequently (53.8%), followed by muscle pain (40.2%), headache (28.0%) and chest pain (26.9%). The number of types of pain a patient was suffering from (ranging from 0-5) was related to the WHOQOL- 100 Pain and Discomfort scale (r = 0.49, p < 0.001). Patients with more types of pain had lower quality of life (QOL). In addition, the total amount of experienced pain categories was associated with the WHOQOL-100 domain Level of Independence (r = -O.43, p < 0.001), and the facet Energy and Fatigue (r = -0.38, p < 0.001). The number of types of pain was predicted by using analgesics, psychological/neurological medication, NSAIDs, being female, indicating to feel tired, more negative feelings and less energy (F(7.635) = 35.2, p < 0.001; R2 = 27.9%)., Conclusions: Pain appeared to be a major problem in sarcoidosis, especially arthralgia. Although negative feelings and fatigue were related to pain, it could not fully explain pain. Future studies are needed to address mechanisms of pain, pain behaviour, and the best therapeutic approach to pain in sarcoidosis.