Autoantibodies to the 75-kDa and 100-kDa subunits of the PM/Scl nucleolar protein complex are associated with an overlap syndrome, manifesting with clinical features of systemic sclerosis and idiopathic inflammatory myopathy. We describe the diverse clinical features in a series of 4 cases with anti-PM/Scl-75 and/or anti-PM/Scl-100 antibodies, including severe proximal muscle weakness, oesophageal dysfunction, respiratory weakness requiring mechanical ventilation, Raynaud’s, calcinosis cutis, sclerodactyly and critical digital ischaemia. Despite the severity of striated and oesophageal muscle weakness, all patients responded very well to immune suppression, and calcinosis cutis in one case regressed substantially. We highlight the efficacy of Rituximab and intravenous immunoglobulin therapy (IVIg) in these cases, enabling return to normal muscle function within six months. Rituximab was preferentially chosen for cases with hyper-gammaglobulinemia and multiple autoantibodies in addition to anti-PM/Scl, and IVIg was utilised for cases where a rapid onset of effect was required, such as severe ventilator-dependent respiratory muscle weakness and oesophageal dysfunction.
Background Giant cell arteritis is a large vessel vasculitis of the arteries in the head and neck. The mainstay of management is with high-dose corticosteroids, and patients often face difficulties stopping or reducing steroids without recurrence of symptoms. Corticosteroids are well established to have numerous associated side effects, including osteoporosis, weight gain, and diabetes. Therefore, when tocilizumab was approved for up to 1 year for cases of relapsing or refractory giant cell arteritis by the National Institute of Health and Care Excellence (NICE) in April 2018, this offered an opportunity to benefit from new funding and to reduce steroid burden. Case presentation This case series describes the impact of the establishment of a new hub and spoke referral pathway for the use of tocilizumab in refractory or relapsing giant cell arteritis, with case examples from consecutive patients who accessed the funding between August 2018 and April 2021. A total of 16 patients were identified: 11 female and 5 male, with an average age of 72.4 (range 61–82) years, with a majority of 11 ethnically white. The central assessing hub is St George’s University Hospitals NHS Foundation Trust Hospital, serving a population of 1.3 million in the south of England. This is the first large case series looking into the impact of the establishment of a regional clinical pathway for the new tocilizumab funding. Conclusions The case series demonstrates that the use of tocilizumab has reduced both the duration and the dose of corticosteroids in these 16 cases (mean prednisolone reduction 20.4 mg: 95% CI 13.0–27.8 mg), with 50% of patients continuing on tocilizumab after the initial 12 month funding period. The disease course, patterns of response, and maintenance of remission are discussed, and we describe the benefits of replicating this hub and spoke tocilizumab pathway in other centers.
AimsTo identify errors at the measurement and/or recording phase of the respiratory rate in the National Early Warning Score (NEWS). MethodsDuring a 1-week period at three London hospitals, foundation year one (FY1) doctors manually counted and recorded the respiratory rate (RR) into the medical notes during a ward round (WR). This was compared to what had been documented in the NEWS observation chart.Three outcomes were measured: 1a -presence of a signifi cant (>5 RR) discrepancy between documentation in NEWS chart and what the RR was on the WR; 1b -presence of a specifi c RR value documented rather than '12-20'; 1c -presence of there being >4 hours since the WR and the last RR recorded; 2 -any change in behaviour of the medical team as a result of being told an accurate RR; 3 -number of times the FY1 was unable to measure the RR (for 30 seconds) because of the brevity of the interaction. ResultsSample size: N=208; 1a -12 instances (5.8%) where there was a signifi cant discrepancy (mean difference of nine); 1b -39 instances (18.8%) where specifi c RR was documented; 1b -23 instances (11.1%) where there was >4 hours since last observation; 2 -One instance (0.5%) of change of behavior; 3 -49 instances (24.1%) where there was insuffi cient time to record RR. ConclusionsWe identifi ed signifi cant variation in the way that the RR was measured and documented in the NEWS at three London hospitals, all belonging to the same trust. We have arranged additional training for relevant stakeholders (nursing staff, healthcare assistants and other clinical staff) to ensure that this most sensitive of markers for illness becomes more accurately measured and recorded.
Background/Aims Pericardial syndromes (PS) comprising pericarditis, pericardial effusions, cardiac tamponade, and constrictive pericarditis, can be presenting features of connective tissue disease (CTD) including lupus (SLE) and rheumatoid arthritis (RA). PS comprise 0.1% of all hospital admissions and 5% of acute non-cardiac chest pain. PS occur as single episodes but recur in 30% of patients within 18 months after the first episode. The 2015 European Society of Cardiology (ESC) pericardial disease guidelines recommend assessment of NSAID response with follow-up and tests in those who are NSAID non-responders or who have recurrent PS. We audited the management of patients presenting with PS in a tertiary referral centre, using the 2015 ESC guideline standards to determine if investigations to uncover autoimmune diseases as a cause of PS were performed. Methods We obtained data on all patients coded with PS presenting to St George’s Hospital Emergency Department between Jan 1st, 2019, and October 31st, 2020. We also searched data including patients’ length of stay, test results including blood tests, imaging (X-ray, ultrasound, CT and MRI), initial management, outpatient follow-up, recurrence numbers and identifiable cause. Data were compared to the 2015 ESC guidelines. Results We identified 132 PS cases, of which 37 (28%) had recurrent pericardial disease. Of these, 15 (41%) were screened for connective tissue disease with antinuclear antibody (ANA) testing. Of those tested, nine (60%) had a positive ANA (titre 1/80 or greater), with three (33%) positive for extractable nuclear antigens (anti-Ro, anti-Sm and anti-RNP). Only 14 patients (38%) with recurrent disease had rheumatoid factor (RhF) tested, with four of these (29%) positive, while one of eight (12.5%) patients tested was anti-CCP antibody positive. Only 13 of 37 recurrent cases (35%) had both ANA and RhF tested. Furthermore, 37 of all PS cases (28%) were not followed up after initial presentation, while only five (14%) of recurrent cases received any follow up. By contrast, presumed infection tested serologically was found in only 7.8% of all cases tested, most commonly Mycoplasma or raised ASOT. Conclusion A high proportion of recurrent PS patients screened in our study had a positive ANA, ENA or RhF suggesting screening can identify new potential CTD, including SLE or RA. These patients require different treatment strategies to infectious PS. We demonstrated screening was inconsistent despite the absence of obvious infective causes in most cases and limited to 41% of patients with recurrent disease, which present the highest risk of association with CTD. ESC guidelines recommend all patients be followed up after 1 week, yet few high-risk patients received any outpatient review, from any speciality. There is a pressing need to ensure potential cases of CTD presenting as PS are not missed as doing so may prolong symptoms and worsen outcome. Disclosure D. Srikantharajah: None. V. Joseph: None. A. Kaul: None.
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