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Aim The aim of this qualitative study was to report the findings of the Defining rheumatoid arthritis progression using Doppler Ultrasound in Clinical practice (DEDUCE) Medical Practice Activity, which was developed to facilitate the utilization of Doppler ultrasound (DUS) by Australian rheumatologists in the treatment of patients with rheumatoid arthritis (RA). Method Twenty-one rheumatologists recruited a total of 80 patients with RA in Disease Activity Score of 28 joints (DAS28) remission for DUS assessment and completed a pre- and post-activity questionnaire assessing their experience with DUS, as well as a 6-month follow-up questionnaire. Rheumatologists discussed DUS results with patients using visual aids. Patients completed a pre- and post-DUS assessment questionnaire. Data were summarized using descriptive statistics. Results Following completion of the activity, 95% of rheumatologists (20/21) believed DUS was a useful assessment tool for patients with RA. The majority found the DUS results useful and more than half thought the DUS assessment fit well into their consultation. A majority of rheumatologists indicated they would use DUS imaging in patients with low disease activity and remission, and for disease activity assessment to inform in therapeutic decision-making. All patients who responded found the visual aids useful and most felt that discussing DUS results improved understanding of their disease and would help with medication adherence. Conclusion Incorporation of DUS imaging into routine clinical practice is feasible, encourages rheumatologists to utilize and expand their clinical application of DUS imaging in patients with RA, and may improve patient understanding of their disease and adherence to medication.

Aim. Examine the efficacy of once-weekly subcutaneous tocilizumab (SC-TCZ) on joint damage at 24 weeks based on radiography of the hands and feet and magnetic resonance imaging (MRI) of the hand in subjects with moderate to severe rheumatoid arthritis (RA). Methods. In this Australian open-label, multicentre, prospective, single-arm study, subjects received 162 mg SC-TCZ weekly. Primary endpoint was change in radiographic Genant-modified Total Sharp Score (TSS) between baseline and Week 24. Secondary endpoints included change from baseline to Week 24 in RA MRI scoring (RAMRIS) of erosions, synovitis, and osteitis and Cartilage Loss Score (CARLOS) in the dominant hand and disease activity score 28 (DAS28). Results. 52 subjects were enrolled (80% female, mean (SD) age 57 (12) years). Radiography showed mild but not significant progression of joint damage (mean (SD) change in TSS 0.46 (1.29)). Synovitis reduced significantly on MRI; however, osteitis, erosion, and cartilage loss did not change significantly. DAS28 improved significantly by Week 24; 78% of subjects achieved DAS28 remission. SC-TCZ was generally well tolerated. Conclusion. Synovitis and DAS28 decreased significantly; however, no significant change in osteitis or joint damage was observed at Week 24. Trial registration. This trial is registered with Clinicaltrials.gov registration number NCT01951170 (ML28703).

Background. Rheumatoid arthritis (RA) is a chronic systemic inflammatory disease affecting

A 55-year-old woman with newly diagnosed Takayasu arteritis was followed for 7 years, during which time she underwent bare metal stenting, drug eluting stenting and coronary bypass grafting for critical coronary and renal artery stenoses. Interventions were initially successful but restenosis occurred within 24 months for all modalities. In contrast, native vessel disease was largely stable after the introduction of immunosuppressive therapy. We advocate a conservative revascularization approach in Takayasu arteritis in the absence of critical end organ ischemia and early optimization of medical therapy.

Introduction Shear-wave elastographic ultrasound (SW-EUS) assesses the stiffness of human tissues. It is used in liver, thyroid and breast imaging but has not been studied in synovium. Soft tissues have a slower shear-wave velocity (SWV) than stiff tissues. We hypothesised that rheumatoid arthritis (RA) patients would have softer synovium than controls and this could be quantified with a slower SWV. We also assessed whether SWV varied with disease activity. Methods Nine patients with RA were consecutively recruited and matched with five controls. Participants underwent clinical assessment, blood sampling, grey scale ultrasound (GSUS), power Doppler ultrasound and SW-EUS of MCP joints 2–5 on the dominant hand. Results Average age was 60. Mean RA disease activity (DAS28-ESR) was moderate at 3.65. Patients with RA had lower maximum synovial SWV than controls (6.38 m/s vs. 6.99 m/s P = 0.042). Negative Pearson's correlation coefficients (PCC) were observed between maximum SWV and disease activity markers including GSUS graded synovial thickness (PCC = −0.57, P = 0.03) and ESR (PCC = −0.46, P = 0.095). Intra- and interobserver reliability was good with intraclass correlation coefficients (ICC) of 0.66 and 0.58, respectively, for quantitative maximum SWV and ICC > 0.80 for colour scale rated SWV. Conclusion This is the first pilot study of SW-EUS in synovium. Maximum synovial SWV was significantly lower in RA than controls. There was a negative correlation between maximum SWV and GSUS synovial thickening. Further study is warranted to confirm the role of SW-EUS in diagnosing and assessing disease activity in RA.

Musculoskeletal ultrasound is an increasingly used tool for the evaluation and management of rheumatologic diseases. Its utilisation by rheumatologists is varied around the world and is dependent upon training and local expertise. Its applications can be broadly categorised into three main areas; for the education of patients, to aid the rheumatologist in the diagnosis and monitoring of disease, and therapeutically for joint aspiration and injection. Ultrasound is a safe, portable, easily repeatable, dynamic images in multiple planes and cheap form of imaging. Ultrasound however, is operator dependent and has a number of artefacts that can result in misinterpretation. Musculoskeletal Ultrasound uses a number of modes to characterise joint pathology, including grey scale, colour and power Doppler, spectral Doppler, 3D imaging, elastography. Musculoskeletal ultrasound can detect and monitor multiple joint pathologies including synovitis, tenosynovitis, and tendon pathologies, entheseal processes, bone erosions and osteophytes, cartilage changes and bursal pathologies.

Imaging continues to assume an increasingly important role in the assessment of patients with rheumatoid arthritis. Application of MRI and Doppler ultrasound to this group of patients has revealed important insights into disease processes in rheumatoid arthritis, identifying previously concealed processes that cannot be viewed with conventional imaging. These processes include osteitis, synovitis and early bone erosion. Together, these imaging techniques provide methods for assessment of treatment response as well as short- and long-term outcome. This article examines the current state of imaging in rheumatoid arthritis, with an emphasis on assessment of treatment outcome.

Aim Autoantibodies directed to centromere protein F (CENP-F) were first reported in 1993 and their association with malignancy has been well documented. We present a case of this autoantibody detected in a 47-year-old female with BRCA1 gene mutation associated with bilateral breast cancer and ovarian cancer. Method and results Antinuclear autoantibody immunofluorescence carried out for possible inflammatory arthropathy showed high titre nuclear speckled-II (NSII) pattern consistent with CENP-F that was confirmed by addressable laser bead immunoassay (ALBIA) for the C-terminal p-F4, an immunodominant CENP-F peptide. Discussion We review the current literature on CENP-F, its association with breast cancer and present the first documented case of this antibody being identified in a person with BRCA1 gene mutation.

Musculoskeletal ultrasound (US) now thrives as an established imaging modality for the investigation and management of chronic inflammatory arthritis. We summarize here results of the Outcome Measures in Rheumatology (OMERACT) US working group (WG) projects of the last 2 years. These results were reported at the OMERACT 12 meeting at the plenary session and discussed during breakout sessions. Topics included standardization of US use in rheumatic disease over the last decade and its contribution to understanding musculoskeletal diseases. This is the first update report of WG activities in validating US as an outcome measure in musculoskeletal inflammatory and degenerative diseases, including pediatric arthritis, since the OMERACT 11 meeting.

Objective To evaluate the performance characteristics of power Doppler ultrasound as a diagnostic and monitoring tool in the assessment of musculoskeletal disease through a systematic review of the literature. Methods Search Strategy: We performed a literature search of PUBMED (1966 to June 2005). Selection Criteria: Only original research reports written in English involving musculoskeletal disease and power Doppler ultrasound were included. Reviews were noted but not included. Data Extraction/Reporting: The design, subjects, methods, imaging protocols, and performance characteristics studied in the research papers were reported. Results Of 3568 identified reports, 139 involved power Doppler ultrasound of the musculoskeletal system. Fifty-three of these reports met the inclusion criteria. Ultrasound machine settings were specified in 63% of reports. Rheumatoid arthritis was the most commonly studied musculoskeletal disease (64% of papers). Validity was the most studied performance characteristic (94% of reports), while reliability and responsiveness were studied in 17 and 34%, respectively. Conclusions Although the majority of research reports of power Doppler ultrasound assessment of the musculoskeletal system evaluated validity, less than half reported reliability and responsiveness. Further work is needed to evaluate power Doppler ultrasound assessment of the musculoskeletal system before it can be used to guide clinical decisions or be used as an endpoint in clinical trials.

The aim of this study was to determine if transducer pressure modifies power Doppler assessments of rheumatoid arthritis synovium at the metacarpophalangeal joints and metatarsophalangeal joints. Five rheumatoid arthritis patients of varying degrees of 'disease activity' and damage were assessed with power Doppler ultrasound scanning of the dominant hand second to fifth metacarpophalangeal joints. Two rheumatoid arthritis patients had their dominant foot first to fifth metatarsophalangeal joints assessed with power Doppler ultrasound. Ultrasonography was performed with a high frequency transducer (14 MHz) with a colour mode frequency of 10 Mhz, and a standard colour box and gain. In the joint that showed the highest power Doppler signal, an image was made. A further image was taken after transducer pressure was applied. In all patients, there was increased flow to at least one joint. After pressure was applied, power Doppler signal intensity markedly reduced in all images and in some there was no recordable power Doppler signal. Increased transducer pressure can result in a marked reduction or obliteration in power Doppler signal. This power Doppler 'blanching' shows the need for further studies to evaluate sources of error and standardization before power Doppler ultrasound becomes a routine measure of 'disease activity' in rheumatoid arthritis.

Palmar fasciitis and polyarthritis syndrome is a rare para- neoplastic condition that may portend a diagnosis of malignancy. We describe the case of a 73-year-old man who presented with progressive palmar swelling, erythema, pain, and contractures of both hands, This presentation and associated weight loss eventually led to the diagnosis of metastatic pancreatic adenocarcinoma. This case highlights the often delayed, but important diagnosis of this unusual paraneoplastic phe- nomenon which can mimic arthropathy, Dupuytren contracture, and scleroderma. Our case is also the first documentation of the extensive inflammatory magnetic resonance imaging changes in palmar fasciitis and polyarthritis syndrome, which affects all tissue planes including the synovium and explains its confusing clinical manifestations.

Purpose: To report an unusual case of retinal microvasculopathy secondary to mixed connective tissue disease (MCTD) on a background history of fragile-X syndrome (FXS). Methods: Case report and literature review. Results: A cotton wool spot was discovered in a 29-year-old female who presented with an ischaemic digit secondary to Raynaud’s phenomenon. She also has a background history of MCTD and FXS. Fundus examination and automated perimetry findings were normal. Magnet resonance imaging and computed tomography aortogram did not demonstrate any evidence of vasculitis in the head and neck. She was tested positively for U1-ribonuclear peptide. Interestingly, the re-distribution of Fragile-X related gene 1 has been suggested to trigger autoimmune responses in experiments. This finding makes the case peculiar as it suggests an alternate explanation for this patient’s clinical findings. Conclusion: Retinal vasculopathy is a rare complication of MCTD. The background history of FXS potentially highlights an alternate autoimmune pathogenetic mechanism.

Objective In early rheumatoid arthritis (RA), longitudinal studies have demonstrated that magnetic resonance imaging (MRI) is more sensitive than radiography in demonstrating progressive erosive joint damage. The present study evaluated the progression of erosive damage in patients with established RA by using limited field of view MRI and comparing the results with those obtained by radiography. Methods MRI and radiographic studies were available from 47 of 60 patients enrolled in a 2-year RA observational study. MRI of the metacarpophalangeal (MCP) joints was performed at baseline and 2 years later, and a single observer scored all of the MR images with the use of an MRI scoring method developed by the Outcome Measures in Rheumatology Clinical Trials MRI RA study group. MR images from 14 patients were reread by the same observer after 1 week to assess intraobserver reliability. Radiographs were obtained at baseline and at 2 years, and were scored by an observer using the Scott modification of the Larsen score. Radiographs from 14 patients were reread after 1 week to assess the intraobserver reliability. The smallest detectable difference (SDD) was calculated for the MRI scores, the total Larsen scores, and the Larsen scores of the dominant-hand MCP joints (MCPs 2–5) for direct comparison with the MRI results. Results The median disease duration was 5.1 years (range 0.5–29 years). Evidence of erosion progression was identified by MRI in 30 patients (64%). The SDD based on the intraobserver scores was calculated as ±3.25 units. Using this result, 11 patients (23%) showed evidence of erosion progression on MRI that was greater than the SDD. The SDD for progression based on the intraobserver total Larsen radiographic scores was 0.77 units, and the SDD for the Larsen scores of the dominant-hand MCP joints was 1.55 units. On the basis of these results, radiographic progression was noted in 19 patients (40%) by the total Larsen score and 7 patients (15%) by the dominant-hand MCP Larsen score. The most striking finding was that although MRI and radiograph scores identified a similar group of patients as having progression of joint damage, the radiographs of both hands appeared to be more responsive to change, albeit with the caveat that radiographic progression was most marked outside the dominant-hand MCP joints. Conclusion There was no clear advantage of MRI with a limited field of view as compared with radiographic imaging of both hands in detecting progression of joint damage over 2 years in this group of patients with established RA. The conclusion drawn from this study is not that radiographs are better than MRI or vice versa, but that careful analysis is required to determine the optimal imaging method, or combination of imaging methods, for each study population, depending on the objective and duration of the study.

Polyarteritis nodosa (PAN) as a paraneoplastic vasculitis is rarely described, especially in association with squamous cell carcinoma (SCC). Furthermore, only 5% of all PAN patients have central nervous system (CNS) involvement, almost exclusively in the form of cerebral infarction or intracerebral haemorrhage. We report the first case of PAN with multiple immunosuppressant-responsive, cerebral vasculitic lesions in association with metastatic SCC.

Common variable immune deficiency (CVID) is characterized by hypogammaglobulinemia, impaired antibody production, and recurrent bacterial infections. Reports of ocular manifestations of CVID are rare. The authors discuss a patient with recurrent keratoconjunctivitis that presented as an initial manifestation of CVID. The diagnosis of CVID should be entertained where multiple recurrences of keratoconjunctivitis are unresponsive to antibiotic and steroid therapy and when other causes of immune deficiency have been excluded. Intravenous immunoglobulin (IVIG) was used successfully as treatment and is required lifelong as prophylaxis against further episodes of keratoconjunctivitis as well as the other infectious and inflammatory complications of this primary immunodeficiency syndrome.

We report three cases of scurvy, with differing musculoskeletal presentations, from a tertiary teaching hospital in Sydney, Australia. Case 1 was a man with cerebral palsy who presented with knee swelling following a minor injury. In Case 2, a patient with thalassaemia major presented with purpuric rash, difficulty walking and distal thigh swelling and ecchymosis. Case 3 was a man with Down's syndrome who presented with acute ankle arthritis. Scurvy in Cases 1 and 3 were related to abnormal dietary preferences, whereas in Case 2, scurvy was thought to be related to thalassaemia. All three cases responded rapidly to vitamin C replacement. The subjects did not appear malnourished as they had adequate carbohydrate and protein intake.

This report presents the results of a recent systematic review performed by the OMERACT Ultrasound Group on the metric properties of ultrasound for the detection of synovitis in inflammatory arthritis. Reviews were conducted for the hand, wrist, elbow, shoulder, knee, ankle, and foot; most reports were related to the hand and knee, and the most common disease process was rheumatoid arthritis. The review highlights the current gaps in the literature, including a lack of reliability data with respect to intra-occasion and intra- and inter-reader reliability. Current work by our group is addressing these issues.

◆Clinical record A 64-year-old woman who had never consumed alcohol presented to hospital with a several-day history of vomiting and severe diarrhoea, secondary to Clostridium difficile colitis. She had suffered a similar episode 3 months earlier. Her symptoms settled after 7 days’ treatment with vancomycin and metronidazole, but her admission was complicated by nosocomial pneumonia and her oral intake was poor. Fifteen days into her stay, she became drowsy and less communicative and developed generalised weakness. Clinical examination revealed signs of encephalopathy. She had slowed mentation, paucity of facial expression and slow limb movements. She could follow one-stage commands but only slowly and with significant prompting. Her eye movements were normal. She had mild generalised weakness in all four limbs, but her deep tendon reflexes were intact and plantar responses were downgoing. Ataxia could not be assessed reliably due to impaired consciousness. Electroencephalography showed generalised slowing without focal features. Magnetic resonance imaging (MRI) of the patient’s brain revealed symmetrical changes of high signal in the periaqueductal grey matter, superior colliculi and medial thalami, most evident on the fluid-attenuated inversion recovery (FLAIR) sequences (Box 1, A–C). These symmetrical periventricular changes were consistent with typical findings recently described for Wernicke’s encephalopathy. 1 Given the clinical history of dietary deprivation and colitis, together with the typical radiological changes, a diagnosis of Wernicke’s encephalopathy was strongly suspected. Hence, intravenous thiamine 500 mg three times a day was administered, after which the patient made a dramatic clinical and radiological recovery (Box 1, D–F) over a period of about 7 days. Despite this

The Medical Journal of Australia ISSN: 0025-729X 4 February 2008 188 3 164-165©The Medical Journal of Australia 2007www.mja.com.auLessons from Practiceur patient had an enlarged iliopsoas bursa associated withosteonecrosis of the femoral head mimicking a tubercu-lous psoas abscess.Rheumatoid arthritis is a recognised cause of giant iliopsoasbursae.

To assess the inter-reader reliability of 3 rheumatologist readers before and after training using 2 methods of assessment: magnetic resonance imaging (MRI) computerized erosion volume assessment and MRI scoring using the OMERACT-5 Rheumatoid Arthritis MRI Score (OM-5 RAMRIS) criteria.Erosion volumes were measured in 10 patients [5 wrist and 5 metacarpophalangeal (MCP) joint studies] with rheumatoid arthritis. Erosion scores were derived from this group and 8 additional subjects to provide a total of 18 subjects (10 wrist and 8 MCP joint studies) with MRI scores for comparison. Subjects were selected from existing MRI databases to provide a spectrum of joint damage for assessment. Initial reading was undertaken after the 2 inexperienced readers were provided with instructions regarding OSIRIS computer software and definitions of the OMERACT score; no other formal training was undertaken. One month after the initial reading, the 2 inexperienced readers undertook a 3 hour training session and all 3 readers then took part in 2 subsequent 2 hour calibration sessions. Each reader then reread the original MRI studies using the computerized erosion volume method and the OMERACT MRI RA score. The interval between the baseline and post-training reading was 2 months. All reading was undertaken on a computer workstation and readers were blinded to other readers' results.For the wrist MRI studies, inter-reader agreement improved considerably after training for both the computerized MRI volume method and the OMERACT MRI score [intraclass correlation coefficients (ICC) 0.21 and 0.46, respectively, pre-training; 0.92 and 0.85 post-training]. The correlation between all readers' scores and volumes was excellent at baseline and post-training. For the MCP joint studies, inter-reader agreement was moderate at baseline for the erosion volume and score (ICC 0.51 and 0.61). While there was some improvement in agreement post-training for the scoring method (ICC 0.75), there was no significant improvement for the erosion volumes (ICC 0.58).Overall, inter-reader agreement for erosion scoring and volume measurement was higher for the wrist joint. The lack of improvement in the MCP joint region for the erosion volume measurements appears to relate primarily to difficulties in estimating the erosion border in the proximal MCP joints using the manual outlining tool. This limits the usefulness of erosion volume measurements in this joint region.

We describe a case of a Salmonella typhimurium mediastinal abscess in a patient with systemic lupus erythematosus (SLE). Patients with SLE are predisposed to nontyphoidal salmonella infection with a high incidence of bacteraemia and abscess formation. Our case is the first report of a mediastinal abscess from Salmonella typhimurium in an SLE patient and highlights the need for thorough assessment and treatment of SLE patients who have this organism identified.