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Therapeutic plasmapheresis as an effective treatment for refractory anti-3-hydroxy-3-methylglutaryl coenzyme A reductase-positive immune-mediated necrotising myopathy.
BMJ Case Rep
January 2025
Faculty of Health and Medical Sciences, The University of Adelaide, Adelaide, South Australia, Australia.
Immune-mediated necrotising myopathy (IMNM) can be associated with autoantibodies to 3-hydroxy-3-methylglutaryl coenzyme A reductase (anti-HMGCR). We present a case of a man in his 60s with a 13-year history of relapsing anti-HMGCR-positive IMNM, intermittently partially responsive to various treatments including corticosteroids, methotrexate, mycophenolate, intravenous immunoglobulin, abatacept and rituximab. After a repeat presentation with severe weakness, plasmapheresis was commenced, resulting in rapid and significant improvement in muscle strength and biochemical markers, which was sustained for several months.
View Article and Find Full Text PDFPlasma Exchange in Anti-Signal Recognition Particle Myopathy: A Systematic Review and Combined Analysis of Patient Individual Data.
J Pers Med
April 2024
Connective Tissue Diseases Unit, Department of Internal Medicine, University Hospital of Salamanca-IBSAL, 37001 Salamanca, Spain.
Anti-signal recognition particle myopathy (anti-SRP myopathy) is a rare subtype of immune-mediated inflammatory myopathy characterized by muscle weakness and anti-SRP autoantibodies. Although plasma exchange (PE) is used in severe cases, its role remains unclear. A systematic review was conducted following PRISMA guidelines, identifying 23 patients with anti-SRP myopathy treated with PE.
View Article and Find Full Text PDFConnecting the dots: An updated review of the role of autoimmunity in narcolepsy and emerging immunotherapeutic approaches.
Sleep Med
January 2024
Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:
Background: Narcolepsy type 1 (NT1) is a chronic disorder characterized by pathological daytime sleepiness and cataplexy due to the disappearance of orexin immunoreactive neurons in the hypothalamus. Genetic and environmental factors point towards a potential role for inflammation and autoimmunity in the pathogenesis of the disease. This study aims to comprehensively review the latest evidence on the autoinflammatory mechanisms and immunomodulatory treatments aimed at suspected autoimmune pathways in NT1.
View Article and Find Full Text PDFUsing Cardiohelp, Quadrox, and Nautilus Extracorporeal Membrane Oxygenators as Vascular Access for Hemodialysis, Continuous Renal Replacement Therapy, and Plasmapheresis: A Brief Technical Report.
ASAIO J
November 2023
Division of Cardiovascular and Thoracic Surgery, UC San Diego Department of Surgery, La Jolla, California.
The use of intermittent hemodialysis (iHD), and continuous renal replacement therapy (CRRT), along with extracorporeal membrane oxygenation (ECMO) in patients with acute kidney injury (AKI) and end-stage renal disease (ESRD) is very common. In this technical report, we describe the methods to perform these dialytic therapies safely and effectively using the ECMO circuit in lieu of a separate dialysis catheter. Specifically, we describe in detail how to connect these kidney replacement therapy modalities to a Quadrox, Nautilus, and Cardiohelp HLS (combined oxygenator and pump) oxygenator.
View Article and Find Full Text PDFAcute Pancreatitis: Presenting in Different Clinical Settings.
J Assoc Physicians India
January 2023
GSL Medical College & General Hospital, Rajamahendravaram, Andhra Pradesh, India.
Introduction: Acute pancreatitis (AP) is one of the most important gastrointestinal emergencies with significant morbidity, mortality, and financial burden. It is potentially a life-threatening condition unless attended to earliest.
Materials: Acute Pancreatitis can present in various clinical scenarios with or without the involvement of multiple organ systems.
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