Incidence of biopsy-proven giant cell arteritis (GCA) in South Australia 2014-2020.

Objective: To determine the incidence of biopsy proven giant cell arteritis (GCA) in South Australia. Methods: Patients with biopsy-proven GCA were identified from pathology reports of temporal artery biopsies at state-based pathology laboratories, from 1 January 2014 to 31 December 2020. Incidence rates for biopsy-proven GCA were calculated using Australian Bureau of Statistics data for South Australian population sizes by age, sex, and calendar year. Seasonality was analyzed by cosinor analysis. Results: There were 181 cases of biopsy-proven GCA. The median age at diagnosis of GCA was 76 years (IQR 70, 81), 64% were female. The estimated population incidence for people over 50 was 5.4 (95% CI 4.7, 6.1) per 100,000-person years. The female: male incidence ratio was 1.6 (95% CI 1.2, 2.2). There was no ordinal trend in GCA incidence rates by calendar year (p = 0.29). The incidence was, on average, highest in winter, but not significantly (p = 0.35). A cosinor analysis indicated no seasonal effect (p = 0.52). Conclusion: The incidence of biopsy-proven GCA remains low in Australia. A higher incidence was noted compared to an earlier study. However, differences in ascertainment and methods of GCA diagnosis may have accounted for the change.

Physical therapy interventions for gross motor skills in people with an intellectual disability aged 6 years and over: a systematic review.

AIM: The systematic review was undertaken to investigate the effectiveness of physical therapy interventions for improving gross motor skills (GMSs) in people with an intellectual disability aged 6 years and older. There is a lack of physical therapy research for GMSs in this population, and no prior systematic review. People with an intellectual disability may require specific teaching approaches within therapy interventions to accommodate their cognitive and learning needs. People with an intellectual disability who suffer from GMS deficits can benefit from physical therapy to help improve their GMSs. METHODS: Data sources were PubMed, CINAHL, Embase, and ProQuest. Reference lists of relevant identified articles were also hand searched. Papers published in English from 1 January 2008 to 22 October 2014 were considered for inclusion. This start date was chosen to reflect the tenets of the United Nations Convention on the Rights of Persons with Disabilities which was ratified in 2008.Eligible study designs for inclusion were randomized controlled trial (RCT), pseudo-RCT, repeated measures, and case report. Overall, 887 potential articles were identified, of which 42 were retrieved for full-text review, and seven were finally included. Critical appraisal was independently conducted by two reviewers using the Joanna Briggs Institute appraisal checklists; no articles were excluded following critical appraisal. Data extraction was performed using Joanna Briggs Institute Meta-Analysis of Statistics Assessment and Review Instrument data extraction instruments. RESULTS: High heterogeneity between the studies precluded meta-analysis of the results; a narrative synthesis was completed instead. Two RCTs, two pseudo-RCTs, two repeated measures studies, and one case report were included. Studies varied in regard to participants' intellectual disability, and the clinical interventions used. Interventions were well tolerated with negligible adverse effects. Significant improvements were reported for cadence and nondimensionalized gait velocity following body-weight-supported gait training; cadence following lower limb strengthening exercises; and the Gross Motor Function Measure-88 measures following adapted judo training. These results suggest that task-specific training may be useful. However, the overall quality of evidence was low. CONCLUSION: The evidence identified by this systematic review supporting physical therapy for improving GMSs in people with an intellectual disability was limited because of the low quality of studies (only two were RCTs) and only three statistically significant findings. Given the current paucity of evidence, a future systematic review, which sought to identify evidence regarding interventions used by a range of health and education professionals for improving GMSs in people with an intellectual disability, could provide a broader evidence base for clinical interventions which may be utilized by physical therapists.

Complete heart block in a Caucasian woman with Behçet's disease: a case report.

BACKGROUND: Behçet's disease is a progressive diffuse inflammatory vasculitis characterized by recurrent oral and genital ulceration and ocular inflammation. Cardiac involvement is a rare but well-documented manifestation of Behçet's disease. Complete heart block in non-Caucasian populations has been reported previously; however, in this report, we describe a unique case of complete heart block in a Caucasian woman with Behçet's disease. CASE PRESENTATION: A 48-year-old Caucasian woman presented to our hospital with symptomatic complete heart block requiring a pacemaker implant on a background of recurrent oral and genital ulcers and oligoarthritis of 10 months' duration. She also had a history of recurrent diarrhea with a single episode of ocular inflammation in the recent past. She had no evidence of cardiac ischemia, and her autoimmune antibodies were within normal ranges. She was diagnosed with Behçet's disease according to international study group criteria and was commenced on prednisolone and sulfasalazine, to which she responded very well. CONCLUSIONS: Cardiac complications should be considered when making a diagnosis of Behçet's disease, even in Caucasian patients. While mucocutaneous ulceration is indeed the most common manifestation of Behçet's disease, cardiovascular involvement tends to cause the most morbidity and mortality.

Primary sclerosing cholangitis associated with CREST (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia) in an elderly woman: a case report.

INTRODUCTION: CREST (calcinosis, Raynaud phenomenon, oesophageal dysmotility, sclerodactyly, and telangiectasia) syndrome comprising calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia and primary sclerosing cholangitis are both chronic fibrotic diseases but the association between them is extremely rare. While primary sclerosing cholangitis has been associated with diffuse cutaneous scleroderma, the association with limited cutaneous scleroderma or CREST has not been previously reported in the literature. This case report illustrates the association between CREST and primary sclerosing cholangitis. CASE PRESENTATION: We report the case of an 84-year-old Asian woman with a long history of CREST who was admitted with abdominal pain, fatigue and progressive derangement of her liver enzymes. This was initially thought to be secondary to her bosentan therapy for pulmonary hypertension but it persisted despite bosentan being ceased. Primary sclerosing cholangitis was subsequently diagnosed on magnetic resonance cholangiopancreatography and she was referred to a hepatologist for treatment. CONCLUSIONS: This case highlights the need to consider primary sclerosing cholangitis in patients with CREST who present with abdominal symptoms and deranged liver enzymes when other causes have been excluded. Relevant differential diagnoses for this presentation, which can be difficult to exclude, include immunoglobulin G4-associated cholangitis and antimitochondrial antibody negative primary biliary cirrhosis. It is of particular significance to rheumatologists and gastroenterologists but has broader relevance to all medical specialists involved in the care of patients with CREST.

Behcet's Disease: Is There Geographical Variation? A Review Far from the Silk Road.

Behcet's Disease (BD) is a systemic vasculitis characterized by the triad of recurrent mouth and genital ulcers with eye involvement. To date there are no laboratory tests specific for the disease and diagnosis continues to remain on clinical grounds. Multiple criteria have been created as guides for diagnosis; however, given the wide spectrum of organ involvement, some cases remain undiagnosed. The diagnosis of Behcet's Disease may only be made over time as the clinical manifestations emerge sometimes separated by months and even years. With an increased recognition of this disease it has become apparent that there is geographical variation in clinical manifestations. In particular cardiac manifestations are not seen commonly in Caucasians compared to Asian and Middle Eastern patients, while neurological manifestations are more common in Caucasians. Use of immunosuppressive and immunomodulatory drugs to suppress inflammation remains the cornerstone of treatment.

Polymyalgia rheumatica: clinical update.

BACKGROUND: Polymyalgia rheumatica is a relatively common inflammatory rheumatic disease. There are no validated international guidelines available for the diagnosis and treatment of PMR; however, diagnostic and classification criteria are currently being developed. OBJECTIVE: The aim of this article is to summarise the main management options suggested by American College of Rheumatology and discusses the role of the general practitioner in the diagnosis and early management of PMR. DISCUSSION: Diagnosis is made on the basis of a combination of clinical and laboratory findings. Patients typically present with shoulder and hip girdle pain with pronounced stiffness. Inflammatory markers are usually elevated and an ultrasound and MRI of the shoulder and hip can be done to localise inflamed tissues. Response to steroids should not be used as a defining feature of PMR but treatment with low dose prednisone should be considered. PMR has an excellent prognosis if diagnosis is prompt and therapy adequate.

Difficulties with assessing the benefit of glucosamine sulphate as a treatment for osteoarthritis.

Osteoarthritis is a chronic disease with a major impact on quality of life for a large proportion of the population. It is a disease for which to date there has been no disease-modifying therapy identified. As a result of its physiological role in articular cartilage, glucosamine sulphate has been postulated as a treatment for osteoarthritis. Claims have included symptomatic relief and even reduction in the rate of disease progression. Despite promising in vitro studies, however, the role of glucosamine sulphate in the management of osteoarthritis remains unclear. Studies addressing this issue have generated a wide range of conclusions, and these are discussed here. Methodological issues need to be addressed in order to gauge whether there is true benefit. On current evidence, it would appear that the benefits of dietary supplementation with glucosamine sulphate are limited to mild symptomatic relief, while a disease-modifying agent for this disease remains elusive.

Deteriorating tactile sensation in patients with hand syndromes associated with diabetes: a two-year observational study.

AIMS: To observe the natural history of hand function during a two-year period in participants with hand syndromes associated with diabetes and to determine factors related to changing function. METHODS: Hand function was measured over three annual visits using Disability of the Arm, Shoulder and Hand (DASH) and SF-36v2 questionnaires, grip strength, light touch and 9-hole peg tests. Light touch was tested with WEST monofilaments at 7 sites on the hand (score 35 to 0). Data were analyzed using repeated-measures ANOVA, Spearman's correlation, and Wilcoxon signed-rank tests. RESULTS: Participants (n=60) were aged 61 ± 10.5 years, 57% female, diagnosed with diabetes and at least one of four associated hand disorders. Presentations of carpal tunnel syndrome, or past release (n=27, 45%) and trigger finger (n=24, 40%) were common. Tactile sensation was reduced during the two-year period (median, range; 30 months, 25-40 months). Initial median (inter-quartile range) scores for the dominant hand of 25.5 (22-28.5) were reduced to 23 (21.5-27). This sensory loss was weakly associated with HbA1c (r=0.30, p=0.05) and occurred predominantly in participants with trigger finger (p=0.05). CONCLUSIONS: Light touch perception was reduced in longstanding diabetic hand syndromes. Tactile abnormalities that were detected by clinical examination progressed during a two year period and were related to metabolic control and musculoskeletal diagnosis.

Intra-articular hyaluronic acid preparations for use in the treatment of osteoarthritis.

Intra-articular hyaluronic acid preparations have been available for the treatment of osteoarthritis of the knee for over 15 years. Their use has been limited by cost, difficulties of administration and conflicting evidence of efficacy. Difficulties in conducting adequate clinical trials have resulted in the appearance of multiple meta-analyses whose findings are not congruent. The appearance of a single injection agent and a better understanding of the mechanisms by which this intervention is effective may help to solve this ongoing problem in healthcare research.

Osteoporosis education improves osteoporosis knowledge and dietary calcium: comparison of a 4 week and a one-session education course.

BACKGROUND: Education is ideal for osteoporosis because many risk factors are modifiable. However, the efficacy of shortened education courses compared to a standard 4 week course for improving osteoporosis knowledge and healthy behaviours is not known. This study aimed to assess whether education changed knowledge and healthy behaviours over 3 months of follow-up; and whether changes in these outcomes were different between participants receiving the different education courses. METHODS: Adults aged ≥ 50 years presenting to Emergency with mild to moderate trauma fractures received either the Osteoporosis Prevention and Self-Management Course (OPSMC) (4 × 2.5 h) (n = 75) or a one-session course (1 × 2.5 h) (n = 71) in a non-randomised prospective study with single-blinded allocation. Participants completed questionnaires measuring osteoporosis knowledge, dietary calcium, physical activity, calcium and exercise self-efficacy, and osteoporosis medications at baseline and 3 months. Data were analysed using mixed models and GEE regression models. RESULTS: Osteoporosis knowledge and calcium from food (% of RDI) increased from baseline to 3 months in both groups (P < 0.01). Use of osteoporosis medications increased between baseline and 3 months in the OPSMC group while decreasing in the one-session group (P = 0.039). There were no differences between the groups or over time in physical activity, calcium or exercise self-efficacy. CONCLUSIONS: Osteoporosis education (either the OPSMC or the one-session course) improved osteoporosis knowledge and calcium intake after 3 months. Participants attending the OPSMC maintained medication compliance. We were unable to determine other differences between the courses. This study confirms the value of osteoporosis education for improving osteoporosis knowledge.

Hand syndromes associated with diabetes: impairments and obesity predict disability.

OBJECTIVE: We determined patterns of disability in diabetic hand conditions and identified factors that contributed to functional limitations. METHODS: Hand assessments were performed on 60 adults with DM1 or DM2 and carpal tunnel syndrome, trigger finger, Dupuytren's disease, or the syndrome of limited joint mobility. The examination included measurement of grip strength, light touch perception, and dexterity, as well as self-reported function using the Disabilities of the Arm, Shoulder and Hand (DASH) instrument and the Medical Outcomes Study Short Form-36 questionnaire. Associations with hand disability were analyzed using correlation and regression. RESULTS: The most frequent presentation was carpal tunnel syndrome (45%) but it was common for patients to present with clinical features associated with more than one hand syndrome (47%). Overall, women had greater difficulties, with significantly higher DASH scores than men [mean 30.3 (95% CI 23.2, 37.5) vs 18.0 (95% CI 12.1, 23.9), respectively; p = 0.01]. Grip strength, dexterity, and obesity were associated with hand disability (p < 0.05). CONCLUSION: In adults with hand syndromes associated with diabetes, disability was related to impaired muscle function and carpal tunnel syndrome. Obesity and overall physical functioning influenced hand disability, particularly in women.

Patient education--the forgotten link in managing osteoporosis.

BACKGROUND: Osteoporosis and bone fractures are common, yet osteoporosis is under diagnosed in Australian settings. Osteoporosis can now be reliably diagnosed, and safely and effectively treated, but patient education strategies are under utilised. OBJECTIVE: This article discusses the role of education in osteoporosis risk reduction and management. DISCUSSION: Osteoporosis education initiatives such as the Osteoporosis Prevention and Self Management Course can increase knowledge, self efficacy and health related behaviours such as calcium intake and exercise over the short to medium term.