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IgG4-related disease: features and treatment response in a multi-ethnic cohort in Singapore

Fong W, Liew I, Tan D, Lim KH, Low A, Leung YY

Clin Exp Rheumatol, 2018 05 24;36 Suppl 112(3):89-93.
PMID: 29846168

OBJECTIVES: To describe the features and treatment outcomes of IgG4-RD in multi-ethnic patients in Singapore.
METHODS: Retrospective study was performed on IgG4-RD patients identified from patient databases in a tertiary hospital.
RESULTS: Fourty-two patients (76% male) were included; 79% fulfilled the 2011 comprehensive diagnostic criteria for IgG4-RD for definite IgG4-RD. 81% were Chinese and 19% were Malays. Common initial manifestations included jaundice (52%), abdominal pain (36%) and swollen salivary glands (26%). Only 36% had a history of allergy. 83% had ≥ 1 organ involvement. Erythrocyte sedimentation rate, immunoglobulin E, IgG2 and IgG4 levels were elevated in 84%, 100%, 70% and 44% of patients, respectively. The most common histopathological feature was >10 IgG4+ cells per high power field (66%). 94% (34/36) of patients were treated with moderate to high doses of glucocorticoids, including 17 patients with combination immunosuppressants. Of these, all patients responded to therapy by 3 months. With a median (range) follow-up of 4.1 (0.4-13.8) years, 69% (25/36) needed low dose of glucocorticoids to maintain disease remission. Twenty-six per cent had relapse of disease, of which 82% had disease recurrence in the same organs.
CONCLUSIONS: Pancreatitis, lymphoadenopathy and cholangitis were the commonest manifestations in Asians with IgG4-RD. All patients responded to glucocorticoid therapy by 3 months, two-thirds required maintenance therapy with glucocorticoids, and one-quarter developed relapse of disease.
Current DMOAD options for the treatment of osteoarthritis

Yeap SS

Clin Exp Rheumatol, 2020 03 05;38(4):802.
PMID: 32141438
High-resolution oesophageal manometry and 24-hour impedance-pH study in systemic sclerosis patients: association with clinical features, symptoms and severity

Raja J, Ng CT, Sujau I, Chin KF, Sockalingam S

Clin Exp Rheumatol, 2016 Sep-Oct;34 Suppl 100(5):115-121.
PMID: 26843456

OBJECTIVES: To evaluate the associations between objectively measured gastroesophageal involvement using high-resolution manometry and 24- hour impedance-pH study, and clinical presentations in systemic sclerosis (SSc) patients.
METHODS: This cross-sectional study was conducted in University of Malaya Medical Centre (UMMC) with 31 consecutive SSc patients recruited into this study. Clinical symptoms of gastroesophageal involvement, high-resolution impedance-manometry and 24-hour impedance-pH monitoring were assessed. Their associations with serological features and other organ involvement were evaluated.
RESULTS: Twenty-five (80.6%) patients had gastroesophageal reflux disease (GORD) symptoms, mainly heartburn (45.1%), regurgitation (32.2%) and dysphagia (29%). Using manometry, oesophageal dysmotility was detected in 24 (88.9%) patients, while hypotensive lower oesophageal sphincter (LOS) was observed in 17 (63%) patients. 21 (84%) patients had GORD based on pH study. Hypotensive LOS was significantly associated with presence of digital ulcers. The main gastroesophageal symptoms were absent in majority of the SSc patients including in those with severe gastroesophageal manifestations demonstrating failed peristalsis >75%, hypotensive LOS, Demeester score >200 and acid reflux >200 per day. Demeester score >200 is associated with severity of GORD symptoms. Demeester score >200 was also associated with restrictive lung pattern (p=0.001). Significant association between GORD severity (daily number of acid reflux episodes >200) and pulmonary fibrosis was seen (p=0.030).
CONCLUSIONS: The presence and severity of gastroesophageal symptoms may not accurately reflect the seriousness of oesophageal involvement. GORD severity is associated with presence of restrictive lung pattern and pulmonary fibrosis. Oesophageal manometry and 24-hour pH study should be considered more frequently in the assessment of SSc patients.
Relation of Doppler ultrasound synovitis versus clinical synovitis with changes in native complement component levels in rheumatoid arthritis patients treated with biologic disease-modifying anti-rheumatic drugs

Montoro Alvarez M, Chong OY, Janta I, González C, López-Longo J, Monteagudo I, et al.

Clin Exp Rheumatol, 2015 Mar-Apr;33(2):141-5.
PMID: 25665178

The complement system plays a fundamental role in mediating the activity of rheumatoid arthritis (RA). Biologic therapy can reduce native complement component levels and its activation. We aimed to study the relation of Doppler ultrasound (US) synovitis versus clinical synovitis with changes in native complement component levels in RA patients on biologic therapy.
New insights into Behçet's disease in Ireland: the Midwest cohort study

Adeeb F, Stack AG, Fraser AD

Clin Exp Rheumatol, 2018 11 28;36(6 Suppl 115):33-39.
PMID: 30582512

OBJECTIVES: The epidemiology of Behçet's disease (BD) remains poorly understood with limited international data on disease burden, progression and treatment outcomes. The aims of this study were to determine the natural history of BD in the Midwest region of Ireland and compare our findings with those from other European and Mediterranean studies.
METHODS: We established a cohort of patients with BD in the Midwest Region of Ireland based on ISGBD and/or ICBD criteria. Longitudinal data were captured on demographic and clinical characteristics, disease activity and clinical outcomes.
RESULTS: The cohort included 24 Caucasian patients (16 women, 8 men) and one male patient with Middle Eastern ancestry, who satisfied the diagnostic criteria for BD. Based on the ISGBD criteria, the point prevalence of BD was 6.2 per 100,000 population. The most common clinical manifestation was oral aphthosis (100%) followed by genital aphthosis (92%) and skin lesions (92%), arthralgia/arthritis (40%), ocular involvement (32%), vascular thrombosis (12%) and pathergy phenomenon (8%). Only 1 patient was HLA-B*51 positive. A long-term multidisciplinary approach that included physician specialists, nurse specialists, and general practitioners was adopted for ongoing patient care.
CONCLUSIONS: The prevalence of BD in Ireland is higher than previously reported with a significant proportion experiencing laryngeal destruction. There are many similarities as well as several differences in the epidemiology of BD by country and indeed within countries. We fully advocate the need for national and international collaborative efforts in order to further understand the complex aetiology and immunopathology of BD in order to improve the clinical, physical, psychological wellbeing of patients.
Gut microbiome profiling in systemic sclerosis: a metagenomic approach

Tan TC, Chandrasekaran L, Leung YY, Purbojati R, Pettersson S, Low AHL

Clin Exp Rheumatol, 2023 Aug;41(8):1578-1588.
PMID: 36826808 DOI: 10.55563/clinexprheumatol/jof7nx

OBJECTIVES: The early gastrointestinal (GI) manifestation of systemic sclerosis (SSc) suggests a possible GI microbiota engagement in the pathophysiology and/or progression of SSc. Previous studies have revealed dysbiosis among Caucasian SSc patients. This study extends these findings to Asian SSc patients.
METHODS: Adult SSc patients, stratified according to 1) on immunosuppressive (On-IS) drugs or 2) no immunosuppressive drugs (No-IS), and age-and-sex-matched healthy controls (HC) were recruited. Metagenomic sequencing of stool DNA was compared between SSc patients and HC, and between SSc (On-IS) and (No-IS) patients. Alpha and beta-diversity, taxonomic and functional profiling were evaluated.
RESULTS: Twenty-three female SSc patients (12 On-IS; 11 No-IS; 5 diffuse and 18 limited SSc subtype) and 19 female HC, with median age of 54 years and 56 years, respectively, were recruited. Median SSc disease duration was 3.3 years. Alpha diversity was significantly higher in SSc versus HC (p=0.014) and in SSc (No-IS) versus HC (p=0.006). There was no significant difference in beta diversity between SSc and HC (p=0.307). At the phyla level, there were significantly increased abundance of Firmicutes and Actinobacteria in SSc versus HC, and reduced abundance of Bacteroidetes (all p<0.001). At the species level, there were significantly increased abundance of several Lactobacillus, Bifidobacterium, and Coprococcus species in SSc, and increased abundance of Odoribacter, Bacteroides and Prevotella species in HC. KEGG pathway analysis demonstrated distinct differences between SSc versus HC, and between SSc (No-IS) and SSc (On-IS).
CONCLUSIONS: Using metagenomic sequencing, our study further underlines distinct alterations in microbiota profiling among Asian SSc patients.