Scleroderma in Cairns: an epidemiological study.

BACKGROUND: Systemic sclerosis (SSc) refers to an autoimmune fibrosing disorder with high disease burden and mortality. The prevalence of 23/100 000 in South Australia (SA) is among the highest documented, but anecdotally it is higher still in Cairns. AIMS: To ascertain the prevalence of SSc in Cairns and surrounding regions, and to compare the demographic and clinical characteristics of patients with SSc in Cairns with those in SA. METHODS: Patients with SSc in Cairns were ascertained through hospital records and by referrals from specialist physicians in the region. These patients were interviewed and completed a structured questionnaire. Their physical findings and autoantibodies were recorded. These patients were compared with the SA patients enrolled in the Australian Scleroderma Cohort Study. RESULTS: A total of 81 patients was identified in Cairns, giving an estimated cross-sectional prevalence of 33.7/100 000. Among 65 patients interviewed in Cairns, 23 were born in Cairns, 16 had migrated to Cairns to ameliorate their Raynaud phenomenon and 26 for other reasons. The clinical features in both cohorts were similar, although Cairns had a lower prevalence of digital ulcers (30.8% vs 46.6%; odds ratio (OR) = 0.5035, 95% confidence interval (CI): 0.2839-0.8929, P = 0.0271) and higher prevalence of calcinosis (29.2% vs 17.0%; OR = 2.005, 95% CI: 1.055-3.382). CONCLUSIONS: The higher prevalence of SSc in Cairns is partly, but not completely, due to migration. Differences in clinical features are not entirely explained by the warmer climate. There is a need for greater rheumatologic services in the Cairns region.

Tofacitinib, an Oral Janus Kinase Inhibitor: Pooled Efficacy and Safety Analyses in an Australian Rheumatoid Arthritis Population.

INTRODUCTION: In Australia, there is an unmet need for improved treatments for rheumatoid arthritis (RA). Tofacitinib is an oral Janus kinase inhibitor for the treatment of RA. To provide an overview of key study outcomes for tofacitinib in Australian patients, we analyzed the efficacy and safety of tofacitinib in the Australian subpopulation of global RA phase III and long-term extension (LTE) studies. METHODS: Data were pooled from the Australian subpopulation of four phase III studies and one LTE study (database not locked at cut-off date: January 2016). Patients in the phase III studies received tofacitinib 5 or 10 mg twice daily (BID), placebo (advancing to tofacitinib at months 3 or 6), or adalimumab, with background methotrexate or conventional synthetic disease-modifying antirheumatic drugs. Patients in the LTE study received tofacitinib 5 or 10 mg BID. Efficacy endpoints were American College of Rheumatology (ACR) 20/50/70 response rates, and change from baseline in the Disease Activity Score in 28 joints, erythrocyte sedimentation rate [DAS28-4(ESR)] and Health Assessment Questionnaire-Disability Index (HAQ-DI) scores. Safety endpoints included incidence of adverse events (AEs), serious AEs, and discontinuations due to AEs. AEs of special interest and laboratory parameters were analyzed in the LTE study. RESULTS: Across phase III studies (N = 100), ACR response rates and improvements in DAS28-4(ESR) and HAQ-DI scores were numerically greater with tofacitinib vs. placebo at month 3, and increased until month 12. The results were sustained in the LTE study (N = 99) after 60 months' observation. In general, the efficacy and safety profiles of tofacitinib were similar to those of the global RA population. CONCLUSIONS: In Australian patients with RA, tofacitinib therapy demonstrated sustained efficacy and consistent safety over ≥ 60 months' treatment. FUNDING: Pfizer Inc. TRIAL REGISTRATION NUMBERS (ALL CLINICALTRIALS.GOV): NCT00960440; NCT00847613; NCT00856544; NCT00853385; NCT00413699.

Risk factors for the development of systemic sclerosis: a systematic review of the literature.

OBJECTIVES: Although numerous studies have investigated the roles of various genetic, epigenetic and environmental factors that may impact its aetiology, SSc is still regarded as an idiopathic disease. Given that there is significant heterogeneity in what has been proposed to influence the development of SSc, this systematic review was conducted to assess the impacts of different factors on the aetiology of scleroderma. METHODS: The search was performed in the PubMed, CINAHL and SCOPUS databases on 17 May 2017. Any study that made explicit reference to scleroderma or SSc that had information about the risk factors or epidemiology of the disease was included. The extracted outcome variables were prevalence, gender preponderance, geographical distribution, family history and various proposed environmental risk factors. RESULTS: One thousand five hundred and seventy-four articles were screened for eligibility. Thirty-four articles were eligible for the systematic literature review. CONCLUSION: Age between 45 and 64 years, female sex, positive family history and exposure to silica were found to be risk factors. There were conflicting findings regarding the impact of exposure to organic solvents and microchimerism. No relationship between infectious agents, alcohol consumption or cigarette smoking and the development of SSc was identified.

Illness representations of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic illness that usually follows a fluctuating course and, therefore, can be difficult to diagnose. Consequently, individuals with SLE often hold beliefs about the nature and course of the illness that are at odds with medical opinion. Clearly, a shared understanding of the illness would be beneficial to illness management. In this study, the authors used semistructured interviews and interpretative phenomenological analysis to explore the content of the illness representations held by 36 individuals diagnosed with SLE. They also identified the ways in which these illness representations were generated initially and attempted to determine whether changes occurred over time in these illness representations, as predicted by the self-regulatory model.

Ross River virus disease in tropical Queensland: evolution of rheumatic manifestations in an inception cohort followed for six months.

OBJECTIVE: To describe the natural history of rheumatic manifestations of Ross River virus (RRV) disease. DESIGN: Prospective longitudinal clinical review. SETTING: North Queensland local government areas of Cairns, Douglas, Mareeba and Atherton during January to May 1998. PARTICIPANTS: General practice patients diagnosed with RRV disease on the basis of symptoms and a positive RRV IgM result. MAIN OUTCOME MEASURES: Rheumatic symptoms and signs assessed as soon as possible after disease onset and on two subsequent occasions (up to 6.5 months after onset). RESULTS: 57 patients were recruited, 47 of whom were reviewed three times (at means of 1.1, 2.4 and 3.6 months after disease onset). Results are reported for these 47: 46 (98%) complained of joint pain at first review, with the ankles, wrists, fingers, knees and metacarpophalangeal joints (II-IV) most commonly involved. Prevalence of joint pain decreased progressively on second and third reviews, both overall (92% and 68% of patients, respectively), and in the five joints most commonly affected. The prevalence of other common rheumatic symptoms and signs, and use of non-steroidal anti-inflammatory drugs, also progressively declined over the three reviews. CONCLUSIONS: Earlier studies may have overestimated the prevalence and duration of symptoms in RRV disease. Progressive resolution over 3-6 months appears usual.