Chen-Hung Chen

Background and Objective: The International Map of Axial Spondyloarthritis (IMAS) explores the physical, psychological, and social experiences of patients with axial spondyloarthritis (axSpA). This initiative is now being expanded to Taiwan as the Taiwanese Map of Axial Spondyloarthritis (TMAS). We aim to provide rheumatologists with insights into the perspectives of Taiwanese patients, enabling physicians to better understand the unmet needs of these patients and optimize their management. Materials and Methods: The TMAS is a cross-sectional study gathering data through an online survey of axSpA patients, promoted by the Ankylosing Spondylitis Caring Society of R.O.C. (ASCARES), conducted from July 2017 to March 2018 by Ipsos, and analyzed by the Health & Territory Research (HTR) group of the University of Seville. The questionnaire includes 99 questions that cover domains such as patient profile, diagnosis, habits/lifestyle, employment status, physical/psychological health status, social support, use of healthcare services, and treatments. Results: A total of 112 axSpA patients were included in this survey. The mean age was 38.6 years and 75.0% were male. The average diagnostic delay was 3 years, and 19.6% reported extra-articular manifestations. Out of the 49 respondents who reported HLA-B27 information, 35 were HLA-B27-positive. The disease burden was high, with a mean BASDAI score of 4.9 and 75.9% having a mild to moderate degree of spinal stiffness. Furthermore, they were socially and psychologically burdened, with 88.4% experiencing work-related issues and 25.9% suffering from anxiety. Conclusions: The TMAS sheds light on the overall perspective of axSpA patients in Taiwan. The TMAS shows shorter diagnostic delay compared to patients from the EMAS. However, high disease activity and significant psychological distress still trouble the patients, causing functional impairments and even leading to career failures. Understanding the perspective of axSpA patients can help rheumatologists adjust treatment strategies to their unmet needs and improve their disease outcomes.

Full Papers; Authors: C.-H. Chen, H.-T. Liao, H.-A. Chen, Y.-N. Yen, C.-H. Chen

To investigate the association of blood pressure and hypertension with disease severity among the patients with ankyloing spondylitis (AS). There were 167 AS patients enrolled in the cross-sectional study. Blood pressure was measured and the presence of hypertension was recorded. Patient’s disease severity, including disease activity, functional ability, patient’s global assessments, physical mobility and radiographic damage were evaluated. ESR and CRP levels were tested. We recorded patient’s medication use of NSAIDs, DMARDs and TNF-α blockers. Smoking, exercise habit, diabetes mellitus, hypercholesterolemia and obesity indices were assessed. Multivariate linear regression showed that systolic blood pressure was associated with TNF-α blocker [standard coefficient (β) = 0.194, P = .007], DMARDs (β = 0.142, P = .046), age (β = 0.211, P = .003), male gender (β = 0.242, P = .001) and body mass index (BMI) (β = 0.245, P = .001). Diastolic blood pressure was associated with cervical rotation (β = −0.174, P = .037), lateral lumbar flexion (β = −0.178, P = .019), m-SASSS (β = 0.198, P = .038) and BMI (β = 0.248, P = .003). Notably, multivariate logistic regression showed that hypertension was associated with m-SASSS (OR = 1.033, P = .033), age (OR = 1.098, P = .0010) and BMI (OR = 1.210, P = .003). Using ROC cure analyses, age, BASMI, BASRI-Total, m-SASSS, waist circumference, BMI and waist-to-height ratio were useful in predicting hypertension, and m-SASSS is the best (AUC = 0.784, P < .001). Advanced radiographic damage is an independent risk factor of hypertension in AS, and m-SASSS is the most useful disease severity parameter in predicting the presence of hypertension. Advanced radiographic damage, poor cervical rotation, lateral lumbar flexion, older age, male gender, TNF-α blocker, DMARDs use and obesity are associated with increased blood pressure.

To investigate the association of sleep disturbance with calcium regulatory hormones, disease severity and health index among the patients with ankylosing spondylitis (AS). There were 104 AS patients enrolled in the cross-sectional study, and their sleep quality was recorded. Serum levels of calcium, parathyroid hormone, vitamin D3 and calcitonin were measured. We evaluated patient's disease activity, functional ability, patient's global assessment, physical mobility, radiographic damage and health index. Blood ESR and CRP levels were tested. Sleep quality was positively correlated with serum calcitonin levels (r = 0.260, P = .008). Bad sleep and advanced radiographic damage were found among the AS patients with detectable serum calcitonin levels (P < .05). Sleep quality was significantly correlated with disease duration, CRP, BASDAI, ASDAS-ESR, ASDAS-CRP, BASFI, BAS-G, BASMI and ASAS-HI among the AS patients (all P < .05). Female gender, longer disease duration, higher ASDAS-CRP and serum calcitonin levels (OR [95% CI] = 3.210 [1.012–10.181], P = .048) were independent factors associated with bad sleep. Inflammation, disease activity, functional ability, patient's global assessment and cervical rotation were useful in predicting bad sleep among the AS patients, and ASDAS-CRP was the best predictor (AUC = 0.772, P < .001). Serum calcitonin levels was elevated in the AS patients with bad sleep, and may participate in the pathophysiology of sleep disturbance. Bad sleep was associated with female gender, longer disease duration, higher inflammation, disease activity, functional impairment, mobility restriction, poor patient's global assessment and health index in AS. ASDAS-CRP was best in predicting bad sleep.

A case of intractable IgG4-related orbital disease (IgG4-ROD) was successfully treated by debulking surgery combined with low-dose prednisolone and azathioprine as maintenance therapy. A 64-year-old man visited our clinic with progressive bilateral upper eyelid swelling and right eye fullness of a year’s duration. He was previously treated with systemic corticosteroids for the IgG4-ROD and experienced a partial clinical response but relapsed upon prednisolone cessation. The patient underwent debulking surgery of the right lacrimal gland and right upper eyelid. His post-operative medication was oral prednisolone (5 mg) every other day and 50 mg azathioprine per day. The patient’s right eye remained asymptomatic during the 18 months of follow-up. Debulking surgery combined with low-dose prednisolone and azathioprine, as a maintenance therapy, is an effective and alternative treatment for the long-term control of intractable IgG4-ROD.

Aim: To investigate total and central obesity in ankylosing spondylitis (AS), and assess the association with inflammation, disease severity and cardiovascular risk factors.Methods: There were 105 AS patients enrolled. Anthropometry was measured to determine total (body mass index [BMI]) and central obesity (waist circumference [WC], waist‐to‐height ratio [WHtR]). We evaluated patients’ disease activity, functional ability, global assessment, physical mobility, radiographic damage and health index. Erythrocyte sedimentation rate, C‐reactive protein (CRP) and blood biochemistry profile were tested. Retrospective radiographic change was assessed in 39 patients. Presence of diabetes and hypertension were examined.Results: The obese AS patients had higher inflammation (CRP), disease activity (Ankylosing Spondylitis Disease Activity Score [ASDAS] ‐ CRP), physical mobility (Bath Ankylosing Spondylitis Metrology Index [BASMI]), radiographic damage (modified Stoke Ankylosing Spondylitis Spinal Score [m‐SASSS]), liver function and blood pressure (all P < .05). Obesity (BMI, WC, WHtR) positively correlated with inflammation (CRP), physical mobility (BASMI), radiographic damage (m‐SASSS), health index (Assessment of SpondyloArthritis International Society Health Index), liver function and blood pressure (all P < .05). Moreover, presence of central obesity (WC, WHtR) had correlation with disease activity (ASDAS‐CRP) (r = .218, P = .027; r = .221, P = .025), and predicted longitudinal radiographic change (m‐SASSS) (standard coefficient = 0.300, P = .041; standard coefficient = 0.288, P = .045). Importantly, central obesity was better in predicting high inflammation, disease activity, physical mobility, radiographic damage and health index in AS, and WHtR was the best for predicting m‐SASSS (area under the curve = 0.734, P < .001). Obesity was associated with increased risk of diabetes and hypertension in AS.Conclusion: Obesity was associated with higher inflammation, disease activity, physical mobility, radiographic damage, health index, liver function and cardiovascular risk factors in AS. Central obesity could predict a patient's longitudinal radiographic change. Central obesity is a useful predictor for high disease severity in AS.

Primary Sjögren’s syndrome (PSS) is an autoimmune disease targeting exocrine glands. It ten times more dominantly affects women than men with an onset peak at menopause. The genetic factor predisposing women to PSS remains unclear. Therefore, we aimed to identify susceptibility loci for PSS in women. We performed genome-wide association study (GWAS) using 242 female PSS patients and 1444 female control in Han Chinese population residing in Taiwan. Replication was conducted in an independent cohort of 178 female PSS and 14,432 control subjects. We identified rs117026326 on GTF2I with GWAS significance (P = 1.10 × 10⁻¹⁵) and rs13079920 on RBMS3 with suggestive significance (P = 2.90 × 10⁻⁵) associating with PSS in women. The association of RBMS3 was further evidenced by imputation in which rs13072846 (P = 4.89 × 10⁻⁵) was identified and confirmed as female PSS associating SNP within the same LD with rs13079920. PSS pathogenesis involves both immune (effector) and exocrine (target) system. We suggested that while GTF2I is a previously reported associating gene which may function in immune system, RBMS3 is a novel susceptibility gene that predisposes women to PSS potentially through modulating acinar apoptosis and TGF-β signaling in target exocrine system.

Adult-onset Still’s disease (AOSD) is an inflammatory disorder characterized by high-spiking fever, arthritis, and an evanescent rash. The pattern of chronic arthritis is a universal feature of AOSD. However, chronic bursitis with the formation of numerous rice bodies is a very rare presentation in a patient with AOSD. To our knowledge, no case of formation of numerous rice bodies in AOSD has been reported thus far. We describe the case of a 28-year-old man with AOSD with rice bodies in his left shoulder joint.

Polymyositis may occur along with other manifestations of chronic graft vs host disease after allogeneic bone marrow transplantation (BMT). Donor lymphocyte infusion (DLI) could produce durable remissions in relapsed patients with chronic myelogenous leukemia (CML) but it may contribute to the development of polymyositis. We report in this study a 25-year-old man who suffered from a relapse of CML 4 years after a sibling human leukocyte antigen-matched allogenic BMT. The patient developed polymyositis 18 months after DLI. Mini-pulse therapy with methylprednisolone was effective for his proximal weakness and elevated creatine phosphokinase. There was no relapse of symptoms of polymyositis on tapering of the medication.