The relationship between patient-reported and clinician-assessed outcome measures in Inclusion body myositis - insights from a retrospective cohort study.

Inclusion body myositis (IBM) is an inflammatory myopathy, characterised by slow progression of weakness, skeletal muscle atrophy, and heterogeneous clinical presentation. This variability in disease progression and presentation complicates tracking of clinical progress and intervention response in clinical trials, presenting challenges in identifying reliable outcome measures. We aimed to identify the most useful suite of clinician-assessed and patient-reported outcome measures (PROMs) for use in clinical practice and trials from a selection of the most commonly used outcome measures in IBM.

High-resolution HLA genotyping in inclusion body myositis refines 8.1 ancestral haplotype association to DRB1*03:01:01 and highlights pathogenic role of arginine-74 of DRβ1 chain.

Objectives: Inclusion body myositis (IBM) is a progressive inflammatory-degenerative muscle disease of older individuals, with some patients producing anti-cytosolic 5'-nucleotidase 1A (NT5C1A, aka cN1A) antibodies. Human Leukocyte Antigens (HLA) is the highest genetic risk factor for developing IBM. In this study, we aimed to further define the contribution of HLA alleles to IBM and the production of anti-cN1A antibodies.

Clinical associations of patients with anti-3-hydroxy-3-methylglutaryl CoA reductase antibody-associated immune-mediated necrotising myopathy.

Background: Anti-3-hydroxy-3-methylglutaryl CoA reductase (HMGCR) antibodies are associated with a subtype of immune-mediated necrotising myopathy (IMNM).

Aims: To determine clinical associations of anti-HMGCR antibodies for anti-HMGCR-associated IMNM (HMGCR-IMNM) among a cohort of patients in Western Australia and to determine whether serial HMGCR antibody levels parallel disease activity.

Methods: Adult patients with positive anti-HMGCR antibodies detected by enzyme-linked immunosorbent assay between January 2015 and November 2019 were included.

Idiopathic inflammatory myopathies: a review.

Idiopathic inflammatory myopathy (IIM) is the umbrella term including dermatomyositis (DM), polymyositis (PM), overlap myositis (OM), sporadic inclusion body myositis (IBM) and necrotising autoimmune myopathy (NAM), also known as immune-mediated necrotising myopathy. There is some debate as to whether PM exists as a discrete entity, or perhaps is an overly generalising term encompassing connective tissue disease associated myositis, or OM, and the previously poorly recognised NAM. As such, PM will not be covered in detail in this review.

Drug reaction with eosinophilia and systemic symptoms syndrome secondary to acetazolamide associated with markedly elevated procalcitonin.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is an important cause of multi-organ dysfunction and can mimic other disorders including sepsis. We describe a patient presenting with septic shock and accompanying high procalcitonin. Although initially treated empirically with antibiotics, the emergence of eosinophilia during the admission lead to a revised diagnosis of DRESS syndrome, presumed secondary to acetazolamide.

Polyarteritis nodosa isolated to muscles-A case series with a review of the literature.

Muscular polyarteritis nodosa where disease is isolated to skeletal muscle is a rare and often poorly recognised clinical entity. Patients typically present with fever and severe muscle pain limiting ability to ambulate without rise in creatine kinase. Often there is a significant delay between presentation and diagnosis, which requires histological confirmation.

The significance of anti-granulocyte-macrophage colony-stimulating factor antibodies in cryptococcal infection: case series and review of antibody testing.

We report two cases of cryptococcosis, associated with anti-granulocyte-macrophage colony-stimulating factor antibodies. We review this recently identified acquired form of autoimmune immune deficiency and discuss the potential applications of granulocyte-macrophage colony-stimulating factor antibody testing by enzyme-linked immunosorbent assay.

The use of omalizumab for treatment-refractory chronic spontaneous urticaria in a West Australian outpatient cohort.

There is a lack of real-world data on the use of omalizumab in treatment-refractory chronic spontaneous urticaria (CSU). A single-centre retrospective cohort study was performed to assess the efficacy and safety of omalizumab for treatment-refractory CSU. The overall response rate of 67% is comparable with that reported in the literature.

Hybrid treatment of an isolated immunoglobulin G4-related internal thoracic artery aneurysm.

True aneurysms of the internal thoracic artery (ITA) are rare and are associated with vasculitides, connective tissue diseases, and infections. We report a case of a 3-cm immunoglobulin G4-positive ITA aneurysm that was excised by a hybrid approach involving open ligation of the ITA origin and video-assisted thoracoscopic aneurysmectomy. This novel technique was able to acquire tissue for histopathologic diagnosis through a minimally invasive means.

Photo-acoustic sensor for detection of oil contamination in compressed air systems.

We demonstrate an online (in-situ) sensor for continuous detection of oil contamination in compressed air systems complying with the ISO-8573 standard. The sensor is based on the photo-acoustic (PA) effect. The online and real-time PA sensor system has the potential to benefit a wide range of users that require high purity compressed air.

The high frequency of autoantibodies in HIV patients declines on antiretroviral therapy.

Autoantibodies have been described in samples from HIV positive patients, but the effects of antiretroviral therapy (ART) remain unclear. In a retrospective longitudinal study, we applied clinical assays for autoantibodies to sera collected from 13 HIV positive patients as they began ART with <210 CD4 T-cells/μL and over 2 years on treatment. Twelve of the 13 patients had at least one autoantibody.

Design and experimental verification of a photoacoustic flow sensor using computational fluid dynamics.

A photoacoustic (PA) sensor for fast and real-time gas sensing is demonstrated. The PA sensor is a stand-alone system controlled by a field-programmable gate array. The PA cell has been designed for flow noise immunity using computational fluid dynamics (CFD) analysis.

The Significance of Anti-Beta-2-Glycoprotein I Antibodies in Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is a thrombophilic disorder that classically presents with vascular thrombosis and/or obstetric complications. APS is associated with antiphospholipid antibodies: a heterogeneous group of autoantibodies that are directed against membrane phospholipids in complex with phospholipid-binding proteins. Beta-2-glycoprotein I (B2GPI) binds anionic phospholipids and is considered to be the predominant antigen in APS and antibodies against B2GPI (anti-B2GPI) are recognised in the laboratory criteria for APS diagnosis.

A versatile integrating sphere based photoacoustic sensor for trace gas monitoring.

A compact versatile photoacoustic (PA) sensor for trace gas detection is reported. The sensor is based on an integrating sphere as the PA absorption cell with an organ pipe tube attached to increase the sensitivity of the PA sensor. The versatility and enhancement of the sensitivity of the PA signal is investigated by monitoring specific ro-vibrational lines of CO(2) in the 2 μm wavelength region and of NO(2) in the 405 nm region.

Immunoglobulin G is the only anti-beta-2-glycoprotein I isotype that associates with unprovoked thrombotic events among hospital patients.

This study aimed to determine the strength of association between anti-beta-2-glycoprotein I (anti-β(2)GPI) isotypes and thrombotic events by phenotyping hospital patients tested for diagnostic purposes with at least one positive anti-β(2)GPI isotype. A laboratory database search identified patients who had undergone anti-β(2)GPI testing during a 3 year period. Medical records of patients with a positive anti-β(2)GPI result were reviewed and clinical events ascertained.

Exploring the link between pholcodine exposure and neuromuscular blocking agent anaphylaxis.

Neuromuscular blocking agents (NMBAs) are the most commonly implicated drugs in IgE-mediated anaphylaxis during anaesthesia that can lead to perioperative morbidity and mortality. The rate of NMBA anaphylaxis shows marked geographical variation in patients who have had no known prior exposure to NMBAs, suggesting that there may be external or environmental factors that contribute to the underlying aetiology and pathophysiology of reactions. Substituted ammonium ions are shared among NMBAs and are therefore thought to be the main allergenic determinant of this class of drugs.

Experimental determination of the ²⁴Mg I (3s3p)³P₂ lifetime.

We present the first experimental determination of the electric-dipole forbidden (3s3p)³P₂→(3s²)¹S₀ (M2) transition rate in ²⁴Mg and compare to state-of-the-art theoretical predictions. Our measurement exploits a magnetic trap isolating the sample from perturbations and a magneto-optical trap as an amplifier converting each ³P₂→¹S₀ decay event into millions of photons readily detected. The transition rate is determined to be (4.

Metastable Magnesium fluorescence spectroscopy using a frequency-stabilized 517 nm laser.

We present a laser operating at 517 nm for our Magnesium laser-cooling and atomic clock project. A two-stage Yb-doped fiber amplifier (YDFA) system generates more than 1.5 W of 1034 nm light when seeded with a 15 mW diode laser.

New limits on coupling of fundamental constants to gravity using 87Sr optical lattice clocks.

The 1S0-3P0 clock transition frequency nuSr in neutral 87Sr has been measured relative to the Cs standard by three independent laboratories in Boulder, Paris, and Tokyo over the last three years. The agreement on the 1 x 10(-15) level makes nuSr the best agreed-upon optical atomic frequency. We combine periodic variations in the 87Sr clock frequency with 199Hg+ and H-maser data to test local position invariance by obtaining the strongest limits to date on gravitational-coupling coefficients for the fine-structure constant alpha, electron-proton mass ratio mu, and light quark mass.

Frequency ratio of Al+ and Hg+ single-ion optical clocks; metrology at the 17th decimal place.

Time has always had a special status in physics because of its fundamental role in specifying the regularities of nature and because of the extraordinary precision with which it can be measured. This precision enables tests of fundamental physics and cosmology, as well as practical applications such as satellite navigation. Recently, a regime of operation for atomic clocks based on optical transitions has become possible, promising even higher performance.

Accurate optical lattice clock with 87Sr atoms.

We report a frequency measurement of the 1S0-3P0 transition of 87Sr atoms in an optical lattice clock. The frequency is determined to be 429 228 004 229 879(5) Hz with a fractional uncertainty that is comparable to state-of-the-art optical clocks with neutral atoms in free fall. The two previous measurements of this transition were found to disagree by about 2 x 10(-13), i.

Hyperpolarizability effects in a Sr optical lattice clock.

We report the observation of a higher-order frequency shift due to the trapping field in a (87)Sr optical lattice clock. We show that, at the magic wavelength of the lattice, where the first-order term cancels, the higher-order shift will not constitute a limitation to the fractional accuracy of the clock at a level of 10(-18). This result is achieved by operating the clock at very high trapping intensity up to 400 kW/cm(2) and by a specific study of the effect of the two two-photon transitions near the magic wavelength.