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Clin Exp Dermatol 2021 Aug 3. Epub 2021 Aug 3.

Department of Dermatology, Mayo Clinic, Rochester, MN, USA.

Background: Although MDA-5 autoantibodies are widely explored in dermatomyositis (DM), most studies relied on MDA-5 autoantibody testing performed in research settings and not with the now-available commercial laboratory tests.

Objective: To characterize the clinical and histopathologic data in patients with DM and circulating MDA5-autoantibodies, as defined by commercial-based testing.

Methods: Retrospective review of patients with DM who underwent MDA-5 antibody testing. Read More

Clin Exp Rheumatol 2021 Jul-Aug;39 Suppl 131(4):57-65. Epub 2021 Jul 28.

Division of Angiology, Department of Internal Medicine, Medical University of Graz, Austria.

Objectives: Limited cutaneous systemic sclerosis (lcSSc) is characterised by vasculopathy contributing to vascular apoptosis, structural and functional changes. The aim of this study was to investigate parameters of endothelial dysfunction and their association to clinical events in lcSSc patients with early-stage vasculopathy.

Methods: Patients with lcSSc and early-stage vasculopathy defined as absent pre-existing pulmonary arterial hypertension (PAH), digital ulcers, and symptomatic cardiovascular diseases were recruited together with age-, race- and sex-matched controls with primary Raynaud's phenomenon. Read More

F1000Res 2020 30;9:829. Epub 2020 Jul 30.

Clinical pharmacology, Grenoble Alpes University Hospital, Grenoble, France.

 Raynaud's phenomenon is induced by excessive vasoconstriction of the peripheral microcirculation in response to environmental factors, essentially cold, but also stress or emotions. The objective of the present study is to evaluate the impact of global warming on the worldwide prevalence and severity of Raynaud's phenomenon over the 21 century. We first estimated the correlation between average temperature and prevalence and severity of Raynaud's phenomenon. Read More

BMJ Case Rep 2021 Jul 27;14(7). Epub 2021 Jul 27.

Pediatric Emergency Medicine, Beaumont Health, Royal Oak, Michigan, USA.

Raynaud's phenomenon (RP) is a well-known disorder of self-limiting paroxysmal vasospasms occurring in small arteries of the digits, in the order of skin pallor (white), followed by cyanosis (blue), ending with hyperemia (red). These designative triphasic colour changes with exposure to cold, or emotional response is diagnostic in adults. RP is a very rare phenomenon in the young paediatric population as noted by Nigrovic with 123 patients <19 year old in a large children's centre over 10 years and only 4 patients being <2 years old, with 69% of these being primary RP. Read More

Radiology 2021 Aug;300(2):484-488

From the Departments of Ophthalmology (V.P.D., K.A.A.D., B.K.C.) and Radiology (K.L.R.), Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114; and Department of Neurology, Neuro-Ophthalmology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (B.K.C.).

History A 24-year-old right-handed woman presented to a neuro-ophthalmology clinic in Massachusetts in the summer with acute binocular diplopia when looking down and to the left, which started about 1 month earlier. Her medical history was notable for Raynaud syndrome, recurrent streptococcal pharyngitis, and an allergy to amoxicillin. Three days prior to developing diplopia, she presented to an outside emergency department due to fever, chills, and back pain. Read More

Cell Mol Life Sci 2021 Jul 23. Epub 2021 Jul 23.

Institut de Pharmacologie et Biologie Structurale, IPBS, Université de Toulouse, CNRS, UPS, Toulouse, France.

Different types of multinucleated giant cells (MGCs) of myeloid origin have been described; osteoclasts are the most extensively studied because of their importance in bone homeostasis. MGCs are formed by cell-to-cell fusion, and most types have been observed in pathological conditions, especially in infectious and non-infectious chronic inflammatory contexts. The precise role of the different MGCs and the mechanisms that govern their formation remain poorly understood, likely due to their heterogeneity. Read More

Open Access Rheumatol 2021 16;13:201-212. Epub 2021 Jul 16.

Internal Medicine Department, Division of Rheumatology and Immunology, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Objective: This study was conducted to estimate the frequency of anti-nuclear antibodies (ANAs), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies in juvenile systemic lupus erythematosus (JSLE) patients and their association with different clinical manifestations and disease activity.

Patients And Methods: A cross-sectional study that includes 100 JSLE patients from Ain Shams University Hospital was conducted. All subjects underwent history taking, clinical examination, assessment of disease activity based on the SLE disease activity index (SLEDAI), laboratory investigations, and tests for autoantibodies, namely ANA, anti-dsDNA, and anti-ENA antibodies, including anti-Ro (SSA), anti-La (SSB), anti-Smith (Sm), and anti-U1-ribonucleoprotein (U1-RNP). Read More

Cutis 2021 May;107(5):E24-E26

Drs. Ren and Tan are from the Department of Dermatology, Children's Hospital of Chongqing Medical University, China. Drs. Pruitt and Elston are from the Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston.

J Rheumatol 2021 Jul 15. Epub 2021 Jul 15.

Sources of Support: Research was supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health under Award Numbers R01 AR073270 (MH). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Section of Cardiovascular Medicine, Department of Internal Medicine, Yale School of Medicine, New Haven, CT USA; Vascular Biology and Therapeutics Program, Yale School of Medicine, New Haven, CT USA; Department of Pharmacology, Yale School of Medicine, New Haven, CT USA; Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, CT USA 5 Department of Biomedical Engineering, Yale University, New Haven, CT USA; Section of Rheumatology, Department of Internal Medicine, Yale School of Medicine, New Haven, CT USA; Department of Internal Medicine, Clinical and Translational Research Accelerator, Yale School of Medicine, New Haven, CT USA. Conflict of Interest: MH has received consulting fees from AbbVie and from Boehringer Ingelheim unrelated to the current study. EJM reports grant funding from NIH, Bracco, Eidos, Pfizer, and Alnylam as well as consulting fees from Bracco, Eidos, Pfizer, and Alnylam, all unrelated to the current study. AJS reports grant funding from Siemens and Jubilant as well as consulting fees from MicroVide, LLC, all unrelated to the current study. Corresponding Author: Monique Hinchcliff, MD MS, Director, Yale Scleroderma Program, Yale School of Medicine, Section of Allergy, Rheumatology & Immunology, The Anlyan Center.

Objective: To investigate the association between Raynaud phenomenon (RP) and coronary microvascular dysfunction, we measured myocardial flow reserve (MFR) using positron emission tomography/computed tomography (PET/CT) in primary and secondary RP patients and controls.

Methods: RP patients, patient controls and healthy participants who underwent dynamic reststress 82-Rubidium PET/CT were studied. Differences in heart rate-blood pressure product corrected MFR and clinical predictors of reduced MFR (< 2. Read More

Front Immunol 2021;12:686699. Epub 2021 Jun 28.

Department of Public Health and Caring Sciences, Unit of General Practice, Uppsala University, Uppsala, Sweden.

The coronavirus disease (COVID-19) is a respiratory tract infection caused by the new virus SARS-CoV-2. The acute phase of the infection may in certain individuals be followed by another longer phase of disease (long COVID) of unknown etiology probably associated in certain cases with autoimmune activation. It has been shown that COVID-19 can trigger autoantibody production and in genetically predisposed patients may cause the onset or exacerbation of autoimmune diseases. Read More

Rheumatology (Oxford) 2021 Jul 14. Epub 2021 Jul 14.

Sint Maartenskliniek, Department of Research, Nijmegen, The Netherlands.

Objective: Systemic sclerosis is a complex connective tissue disease affecting mental and physical health. Fatigue, hand function loss, and Raynaud's phenomenon are the most prevalent disease-specific symptoms of systemic sclerosis. This study aimed to develop consensus and evidence-based recommendations for non-pharmacological treatment of these symptoms. Read More

J Neurosurg 2021 07 24;135(1):126-135. Epub 2021 Jun 24.

1Department of Otolaryngology-Head and Neck Surgery, and.

Objective: The object of this study was to ascertain outcomes of cochlear implantation (CI) following stereotactic radiosurgery (SRS) for vestibular schwannoma (VS).

Methods: The authors conducted a retrospective chart review of adult patients with VS treated with SRS who underwent CI between 1990 and 2019 at a single tertiary care referral center. Patient demographics, tumor features, treatment parameters, and pre- and postimplantation audiometric and clinical outcomes are presented. Read More

Sci Rep 2021 07 8;11(1):14090. Epub 2021 Jul 8.

Department of Human Genetics, Research Branch, Sidra Medicine, 26999, Doha, Qatar.

MAIT cells have been shown to be activated upon several viral infections in a TCR-independent manner by responding to inflammatory cytokines secreted by antigen-presenting cells. Recently, a few studies have shown a similar activation of MAIT cells in response to severe acute respiratory coronavirus 2 (SARS-CoV-2) infection. In this study, we investigate the effect of SARS-CoV-2 infection on the frequency and phenotype of MAIT cells by flow cytometry, and we test in vitro stimulation conditions on the capacity to enhance or rescue the antiviral function of MAIT cells from patients with coronavirus disease 2019 (COVID-19). Read More

J Rheumatol 2021 Apr;48(4):616-617

Division of Rheumatology, Hôpital Maisonneuve-Rosemont and Université de Montreal, Montreal, Quebec, Canada.

J Rheumatol 2021 Mar;48(3):465-466

Julia A. Hunter, MB BCh BAO; Deborah M. Levy, MD, MS, FRCPC; Roberta A. Berard, MD, MSc, FRCPC, Division of Pediatric Rheumatology, Western University, London, Ontario, Canada.

Reumatol Clin 2021 Jul 3. Epub 2021 Jul 3.

Servicio de Reumatología, Hospital Universitario 12 de Octubre, Madrid, España.

We describe 4patients with Raynaud's phenomenon associated with systemic sclerosis, 3with ischaemic ulcers, successfully treated with hyperbaric oxygen. This therapy has been useful in the treatment of chronic wounds due to its anti-inflammatory, antimicrobial and angiogenic effects. Hyperbaric oxygen treatment could be a therapeutic option in patients with Raynaud's phenomenon refractory to conventional treatment. Read More

Best Pract Res Clin Rheumatol 2021 Jun 30:101695. Epub 2021 Jun 30.

New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, USA; Tulane University School of Medicine, New Orleans, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, USA; Louisiana State University School of Medicine, Section of Pulmonary Medicine, New Orleans, USA. Electronic address:

Systemic sclerosis (SSc) is a heterogeneous multisystem autoimmune disease whereby its main pathological drivers of disability and damage are vascular injury, inflammatory cell infiltration, and fibrosis. These mechanisms result in diffuse and diverse impairments arising from ischemic circulatory dysfunction leading to painful skin ulceration and calcinosis, neurovascular aberrations hindering gastrointestinal (GI) motility, progressive painful, incapacitating or immobilizing effects of inflammatory and fibrotic effects on the lungs, skin, articular and periarticular structures, and muscle. SSc-related impairments impede routine activities of daily living (ADLs) and disrupt three critical life areas: work, family, social/leisure, and also impact on psychological well-being. Read More

Cureus 2021 May 23;13(5):e15197. Epub 2021 May 23.

Internal Medicine, Southern Hills Hospital and Medical Center, Las Vegas, USA.

Systemic sclerosis (SS) is a heterogenous autoimmune disease that manifests itself with skin and internal organ involvement. The association of SS and malignancy is an emerging field of study with limited data in the literature. This report highlights the unique case of a patient with limited cutaneous SS (lcSS) found to have an intraductal papillary mucinous neoplasm (IPMN) of the pancreas. Read More

Prog Urol 2021 Jun 18. Epub 2021 Jun 18.

Service d'oncologie radiothérapie, unité de curiethérapie, CHU de Poitiers, 2, rue de la Miléterie, 86021 Poitiers, France.

Introduction: Biochemical recurrence of prostate cancer after radiation therapy occurs in 5 to 50% of cases depending on the radiation technique used. The diagnosis of recurrence of prostate adenocarcinoma must be made accurately. The aim of this study was to compare transperineal saturation and target biopsies to index lesion (IL) as defined on MRI and 18FCholine PET-CT (PETc) for detection of intra-prostatic recurrence after primary radiation therapy for prostate cancer. Read More

Rheumatol Int 2021 Aug 16;41(8):1485-1493. Epub 2021 Jun 16.

Department of Pediatric Cardiology and Rheumatology, Medical University of Lodz, Sporna 36/50, 91-738, Lodz, Poland.

Raynaud phenomenon (RP) may be the first manifestation of a systemic connective tissue disease (SCTD). Early detection of dysfunction of small vessels called microangiopathy is essential for the diagnostic process. The focus of this single-center, retrospective study was to investigate the potential dependencies between microvascular image and laboratory markers measured in children with RP. Read More

Adv Skin Wound Care 2021 Jul;34(7):348-354

General Purpose: To familiarize wound care practitioners with the differential diagnoses of chilblains-like lesions that could be associated with the complications of COVID-19.

Target Audience: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care.

Learning Objectives/outcomes: After participating in this educational activity, the participant will:1. Read More

Int Wound J 2021 Jun 4. Epub 2021 Jun 4.

Orthopedic Department, First Affiliated Hospital, Fujian Medical University, Fuzhou, China.

Sympathectomy of arteries has been adopted for the treatment of peripheral arterial disease and Raynaud's disease. However, the exact route for sympathetic axons to reach peripheral arteries awaits further investigation that could pave the way for development of new surgical strategies. In this study, saphenous neurovascular bundles from 10 neonatal Sprague-Dawley rats first were harvested for whole-mount immunostaining to show sympathetic innervation pattern of the artery. Read More

Cureus 2021 Apr 19;13(4):e14569. Epub 2021 Apr 19.

Department of Intedisciplinary Medicine, University of Bari Aldo Moro, Bari, ITA.

Necrotizing Raynaud's phenomenon is a vascular clinical syndrome characterized by vasospasm of distal resistance vessels, usually triggered by cold temperatures or by psychological conditions such as anxiety and stress. Pain is the first reported symptom, related to insufficient oxygen delivery to the extremities that leads to ischemia of the peripheral tissues. The initial treatment is conservative, but if the symptoms persist, necrosis and distal amputation can occur. Read More

J Pers Med 2021 May 2;11(5). Epub 2021 May 2.

Pediatrics Chair, University of Medicine and Pharmacy Carol Davila, 020021 Bucharest, Romania.

Background: Systemic sclerosis (Ssc) is an autoimmune disease with incomplete known physiopathology. There is a high number of candidate proteomic biomarkers for Ssc that have not yet been confirmed on independent Ssc cohorts. The aim of the study was to confirm circulating S100A6, calumenin, and cytohesin 2 as biomarkers for Ssc. Read More

Hand (N Y) 2021 May 31:15589447211017211. Epub 2021 May 31.

The Ohio State University Wexner Medical Center, Columbus, USA.

Systemic sclerosis (scleroderma, SSc) is an autoimmune disease that causes significant dysfunction to multiple organ systems, including the musculoskeletal system. It poses significant challenges to the hand surgeon, including calcinosis, ischemic changes, Raynaud phenomenon, tendinopathies, synovitis, and joint contractures. Patients with SSc also suffer from multiorgan dysfunction, which makes them high-risk surgical patients. Read More

Crit Care 2021 05 28;25(1):181. Epub 2021 May 28.

GPIP, Groupe de Pathologie Infectieuse Pédiatrique, Paris, France.

Arthritis Rheumatol 2021 May 27. Epub 2021 May 27.

Leiden University Medical Centre, Department of Rheumatology, Leiden, The Netherlands.

Objectives: Little is known on the disease course of very early systemic sclerosis (SSc). It is unknown whether anti-centromere antibody (ACA) isotype levels can serve as biomarkers for future SSc development and for organ involvement. We aim to evaluate whether ACA-IgG, -IgM and -IgA levels in ACA-IgG positive patients associate with disease severity and/or progression from very early SSc to definite SSc. Read More

BMC Infect Dis 2021 May 19;21(1):457. Epub 2021 May 19.

SSA (French Military Health Service), Bégin Military Teaching Hospital, Saint-Mandé, France.

Background: This study presents the methods and results of the investigation into a SARS-CoV-2 outbreak in a professional community. Due to the limited testing capacity available in France at the time, we elaborated a testing strategy according to pre-test probability.

Methods: The investigation design combined active case finding and contact tracing around each confirmed case with testing of at-risk contact persons who had any evocative symptoms (n = 88). Read More

Cochrane Database Syst Rev 2021 05 17;5:CD006687. Epub 2021 May 17.

Department of Rheumatology, Gold Coast University Hospital, Southport, Australia.

Background: Numerous agents have been suggested for the symptomatic treatment of primary Raynaud's phenomenon. Apart from calcium channel blockers, which are considered to be the drugs of choice, evidence of the effects of alternative pharmacological treatments is limited. This is an update of a review first published in 2008. Read More

Antimicrob Agents Chemother 2021 Jul 16;65(8):e0001121. Epub 2021 Jul 16.

Centre National de la Recherche Scientifique UMR 9004, Institut de Recherche en Infectiologie de Montpellier (IRIM), Université de Montpellier, Montpellier, France.

Tuberculosis remains a leading cause of mortality among infectious diseases worldwide, prompting the need to discover new drugs to fight this disease. We report here the design, synthesis, and antimycobacterial activity of isatin-mono/bis-isoniazid hybrids. Most of the compounds exhibited very high activity against Mycobacterium tuberculosis, with MICs in the range of 0. Read More