Characteristics of anti-transcriptional intermediary factor 1 gamma autoantibody-positive dermatomyositis patients in Singapore.

INTRODUCTION: This study aimed to determine the clinical profile and outcome of anti-transcriptional intermediary factor 1 gamma autoantibody (anti-TIF1-γ Ab)-positive dermatomyositis patients and propose cancer screening programmes based on regional cancer trends. METHOD: Data on history, physical findings and investigations were collected using chart review on dermatomyositis patients seen at a tertiary hospital in Singapore from 1 January 2015 to 30 June 2021. Comparisons were made between anti-TIF1-γ Ab-positive and anti-TIF1-γ Ab-negative dermatomyositis. RESULTS: Ninety-six dermatomyositis patients were analysed and 36 patients were positive for anti-TIF1-γ Ab. Anti-TIF1-γ Ab-positive patients had more frequent heliotrope rashes, shawl sign, periungual erythema, holster sign, Gottron's papules, dysphagia and truncal weakness (P<0.05). They had less frequent interstitial lung disease, polyarthritis, cutaneous ulcers, palmar papules and mechanic's hands (P<0.05). After 48 months of follow-up, a higher proportion of anti-TIF1-γ Ab-positive patients developed cancer compared with Ab-negative patients (63.9% versus 8.5%; odds ratio 19.1, 95% confidence interval 6.1-59.8; P<0.001). Nasopharyngeal carcinoma (NPC) and breast cancer were the most common malignancies, followed by bowel, lung and non-Hodgkin lymphoma. Most malignancies (78.3%) occurred within 13 months prior to, or 4 months after the onset of dermatomyositis. The mortality rate for anti-TIF1-γ Ab-positive patients was significantly higher than Ab-negative patients (36.1% vs 16.7%, P=0.031), and Kaplan-Meier survival estimates at 24 months were 66% and 89%, respectively (P=0.0153). CONCLUSION: These observational data support periodic screening of NPC and other malignancies in patients with anti-TIF1-γ Ab-positive dermatomyositis in Singapore.

Severe retinal vasculitis in systemic lupus erythematosus leading to vision threatening paracentral acute middle maculopathy.

Systemic lupus erythematosus can affect any organ system at presentation and throughout its course. Lupus retinal vasculitis is responsible for the visual loss of variable severity and typically ascribed to the involvement of pre-capillary superficial arterial vasculature. Using multimodal imaging techniques and electrophysiology, we demonstrate the first-ever case of severe lupus retinal vasculitis resulting in the rare association of paracentral acute middle maculopathy. The findings showcase the involvement of both superficial and deep retinal capillary vasculature and the localisation of retinal dysfunction. This precise evaluation facilitated the timely use of combination immunomodulatory therapy with localised pan-retinal laser photocoagulation, resulting in the preservation of vision.

An evaluation of the effectiveness of teaching anatomy to rheumatologists through combined musculoskeletal sonoanatomy and human cadaveric dissection.

OBJECTIVE: Our aim was to evaluate the effectiveness of teaching anatomy through combined musculoskeletal sonoanatomy and human cadaveric dissection for rheumatologists practising musculoskeletal US. METHODS: The principal focus was on scanning and then dissecting relevant musculoskeletal structures. Outcomes measured included confidence levels and objective knowledge. A mixed-methods approach of evaluation and descriptive statistical data analysis was performed. RESULTS: The change in confidence ratings by delegates after the teaching event as represented by the mean difference (s.d.) (s.e.m.) for identification of surface anatomy was 1.846 (1.281) (0.355), with Student's paired t = 5.196 and P=0.000223. The mean difference (s.d.) (s.e.m) for performing IA injections was 1.538 (1.266) (0.351), with Student's paired t = 4.382, P=0.001, and for recognizing sonoanatomical structures it was 1.769 (1.235) (0.343), with Student's paired t = 5.165 and P= 0.000235. There was a significant increase in correct identification of anatomical and sonoanatomical knowledge in the pre- and post-course assessments. Rotator cuff interval region improved from 13 to 73%, P = 0.004; knee tendons insertion sites from 47 to 93%, P = 0.016; and muscles not adjacent to joints from 27 to 93%, P = 0.002. CONCLUSION: Dissection of joints enabled a three-dimensional relational mind map of the relevant regions of the human body, producing clarity in understanding regional relational topographic anatomy and sonoanatomy. The combination of US and cadaveric dissection improved learners' satisfaction, confidence and knowledge in areas where soft tissue complaints are common, which is likely to lead to accurate early diagnosis and cost-conscious, better overall care.

Singapore chapter of rheumatologists' updated consensus statement on the eligibility for government subsidization of biologic and targeted therapy for the treatment of psoriatic arthritis.

AIM: There have been major advances in biologic treatment options for psoriatic arthritis (PsA) since the publication of the 2015 consensus recommendations by the Chapter of Rheumatologists, College of Physicians, Academy of Medicine, Singapore, for government-assisted funding, thus warranting a revision of this guideline. METHODS: Recent trials and nine published guidelines on the use of biologic therapy for PsA were reviewed. Based on the synthesized evidence, a task force panel (TFP), consisting of 10 practicing rheumatologists in Singapore, rated the statements pertaining to the use of biologic therapy, using a modified Delphi approach. Consensus was obtained if >70% agreed on a statement. RESULTS: The TFP agreed on 10 recommendations pertaining to the initiation, choice and continuation of biologic therapy. A biologic is indicated in patients with PsA: (a) with at least three swollen and tender joints, digits or entheses; and (b) who have failed at least two conventional synthetic disease-modifying anti-rheumatic drug (csDMARD) strategies for a minimum of 3 months each. Any approved drug class including tumor necrosis factor inhibitors, interleukin-17 inhibitors (IL-17i), IL-12/23i or targeted synthetic DMARDs may be considered as first-line treatment, and continued only if a response is achieved by 6 months. CONCLUSION: These recommendations developed through a formal consensus method may be useful to guide funding considerations for appropriate and equitable use of biologic therapy for eligible patients with PsA.

Update on recommendations for eligibility of government subsidization of biologic disease-modifying antirheumatic drugs for the treatment of axial spondyloarthritis in Singapore.

AIMS: The field of axial spondyloarthritis (axSpA) has undergone significant changes recently in particular with disease classification, assessment of disease activity and increased treatment options for biologics. In order to reflect these developments, we aimed to update the local consensus recommendations for subsidization of biologics. METHODS: A modified Delphi approach was used. Six published guidelines from major rheumatology societies and healthcare authorities on axSpA were reviewed. Findings were synthesized and used in formulating updated recommendation statements. Recommendations were rated by 10 practicing rheumatologists in Singapore. Consensus was reached if there was more than 70% agreement or disagreement. RESULTS: Ten statements achieved consensus. Patients may be considered for subsidization of biologic therapy if they fulfill the Assessment of Spondyloarthritis International Society or modified New York criteria, with persistently active disease (defined either by Ankylosing Spondylitis Disease Activity Score ≥ 2.1 or Bath Spondylitis Disease Activity Index ≥ 4), despite 4 weeks of full-dose non-steroidal anti-inflammatory drugs and regular exercise. Either tumor necrosis factor inhibitors or interleukin 17 inhibitors may be used as first-line therapy, and should be continued if adequate response is achieved at 6 months. CONCLUSION: Recommendation statements were formulated through a formal consensus process by local experts with a view to assist relevant authorities in funding considerations and for use in clinical practice.

Singapore Chapter of Rheumatologists updated consensus statement on the eligibility for government subsidization of biologic and targeted-synthetic therapy for the treatment of rheumatoid arthritis.

INTRODUCTION: Approximately 30% of patients with rheumatoid arthritis (RA) respond inadequately to conventional-synthetic disease-modifying anti-rheumatic drugs (csDMARDs). However, widespread use of biologic DMARDs (bDMARDs) and targeted-synthetic (tsDMARDs) is limited by cost. We formulated updated recommendations for eligibility criteria for government-assisted funding of bDMARDs/tsDMARDs for RA patients in Singapore. MATERIALS AND METHODS: Published guidelines regarding use of bDMARD and tsDMARDs were reviewed. We excluded those without a systematic literature review, formal consensus process or evidence grading. Separately, unpublished national reimbursement guidelines were included. RESULTS: Eleven recommendations regarding choice of disease activity measure, initiation, order of selection and continuation of bDMARD/tsDMARDs were formulated. A bDMARD/tsDMARD is indicated if a patient has: (a) at least moderately active RA with a Disease Activity Score in 28 joints/erythrocyte sedimentation rate (DAS28-ESR) score of ≥3.2; (b) failed ≥2 csDMARD strategies, 1 of which must be a combination; (c) received an adequate dose regimen of ≥3 months for each strategy. For the first-line bDMARD/tsDMARD, either tumor necrosis factor inhibitors (TNFi), non-TNFi (abatacept, tocilizumab, rituximab), or tsDMARDs, may be considered. If a first-line TNFi fails, options include another TNFi, non-TNFi biologic or tsDMARDs. If a first-line non-TNFi biologic or tsDMARD fails, options include TNFi or another non-TNF biologic or tsDMARD. For continued bDMARD/tsDMARD subsidization, a patient must have a documented DAS28-ESR every 3 months and at least a moderate European League Against Rheumatism response by 6 months. CONCLUSION: These recommendations are useful for guiding funding decisions, making bDMARD/tsDMARDs usage accessible and equitable in RA patients who fail csDMARDs.

Kaposi's sarcoma in a patient with severe relapsing polychondritis.

This report describes the case of a young man who developed Kaposi's sarcoma (KS) after corticosteroid treatment for severe tracheal involvement of relapsing polychondritis (RP). The etiopathogenetic mechanisms that may have led to the evolution of this unusual neoplasm are discussed. To our knowledge, this is the first case reported of concomitant RP and KS.