Disease Progression in Systemic Sclerosis-Overlap Syndrome Is
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Clinical Reasoning
RESIDENT & FELLOW SECTION Clinical Reasoning: Section Editor A 49-year-old woman with progressive Mitchell S.V. Elkind, MD, MS motor deficit Ana Monteiro, MD SECTION 1 strength, and difficulty protruding the tongue, without Amélia Mendes, MD A previously healthy 49-year-old woman presented with fasciculations or atrophy. Symmetrical tetraparesis Fernando Silveira, MD progressive motor deficit. The complaints started the (proximal-greater-than-distal weakness) and increased Lígia Castro, MD year before with weakness of the right arm. Over the tone were noted, with severe pain upon mobilization Goreti Nadais, MD subsequent months, she developed weakness in the left and palpation of joints and muscles. Deep tendon reflexes arm, followed by both legs, and, finally, difficulty speak- were brisk and symmetric, with bilateral flexor plantar ing, with nasal voice, and swallowing. It was increasingly responses. There was atrophy of the interosseous muscles Correspondence to difficult to attend to her chores, and, by the time she of the hands and shoulder girdle muscle wasting. Dr. Monteiro: sought medical attention, she needed help with all daily [email protected] Questions for consideration: activities. In the last few weeks, she also complained of diffuse joint and muscle pain. Medical and family history 1. How do you localize the symptoms: upper motor were unremarkable. neuron (UMN) or lower motor neuron (LMN), Neurologic examination showed bilateral facial weak- neuromuscular junction (NMJ), peripheral nerve, ness, severe dysarthria, dysphonia and dysphagia (nau- or muscle? What is the broad differential? seous reflex preserved), decreased shoulder elevation 2. What findings on examination would be helpful? GO TO SECTION 2 Supplemental data at Neurology.org From the Departments of Neurology (A. -
A Rare Case Report of Polyangiitis Overlap Syndrome: Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis Michele V
Quan et al. BMC Pulmonary Medicine (2018) 18:181 https://doi.org/10.1186/s12890-018-0733-2 CASE REPORT Open Access A rare case report of polyangiitis overlap syndrome: granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Michele V. Quan1* , Stephen K. Frankel2, Mehrnaz Maleki-Fischbach3 and Laren D. Tan1* Abstract Background: Granulomatosis with polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic granulomatosis with polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Several polyangiitis overlap syndromes have been identified, however, there are very few case reports of an overlap syndrome involving both GPA and EGPA in the medical literature. Case presentation: We conducted a PUBMED literature review using key words ‘granulomatosis with polyangiitis,’ ‘Wegener’s,’‘GPA,’‘eosinophilic granulomatosis with polyangiitis,’‘Churg-Strauss,’‘EGPA,’‘overlap syndrome,’‘Wegener’s with eosinophilia,’ and ‘GPA with eosinophilia’ in English only journals from 1986 to 2017. Relevant case reports and review articles of overlap syndromes of GPA and EGPA were identified. We aim to report a unique case of GPA and EGPA overlap syndrome and review the cases that have been previously described. Between 1986 and 2017, we identified 15 cases that represent an overlap syndrome with compelling features of both GPA and EGPA. -
Understanding and Managing Scleroderma
Understanding and Managing Scleroderma A publication of Scleroderma Foundation 300 Rosewood Drive, Suite 105 Danvers, MA 01923 Maureen D. Mayes, M.D., M.P.H. Understanding and Understanding My notes and Managing Scleroderma Managing Scleroderma This booklet is intended to help people with scleroderma, their families and others interested ________________________ in learning more about the disease to better understand what scleroderma is, what effects ________________________ it may have, and what those with scleroderma can do to help themselves and their physicians ________________________ manage the disease. It answers some of the most frequently asked questions about ________________________ A publication of Maureen D. Mayes, M.D., M.P.H. Scleroderma Foundation 300 Rosewood Drive, Suite 105 scleroderma. Danvers, MA 01923 800-722-HOPE (4673) www.scleroderma.org www.facebook.com/sclerodermaUS www.twitter.com/scleroderma ________________________ Disclaimer The Scleroderma Foundation does not provide medical advice nor does it ________________________ endorse any drug or treatment mentioned herein. ________________________ The material contained in this booklet is presented for general information only. It is not intended to provide medical advice, to answer questions specific to the condition or problems of particular individuals, nor in ________________________ any way to substitute for the professional advice and care of qualified physicians. Mention of particular drugs and/or treatments is for ________________________ information purposes only and does not constitute an endorsement of said drugs and/or treatments. ________________________ Thanks! ________________________ The Scleroderma Foundation expresses its deep appreciation to the many ________________________ physicians whose efforts have led to this booklet. Special thanks are owed to Maureen D. Mayes, M.D., M.P.H., of the ________________________ University of Texas McGovern Medical School, Houston. -
The Voice of the Patient
The Voice of the Patient A series of reports from the U.S. Food and Drug Administration’s (FDA’s) Patient-Focused Drug Development Initiative Systemic Sclerosis Public Meeting: October 13, 2020 Report Date: June 30, 2021 Center for Drug Evaluation and Research (CDER) U.S. Food and Drug Administration (FDA) 1 Table of Contents Introduction ............................................................................................................................ 3 Overview of Systemic Sclerosis ................................................................................................................. 3 Meeting Overview ..................................................................................................................................... 3 Report Overview and Key Themes ............................................................................................................ 5 Topic 1: Disease Symptoms and Daily Impacts That Matter Most to Patients .......................... 6 Perspectives on Most Significant Symptoms ............................................................................................ 6 Overall Impact of Systemic Sclerosis on Daily Life .................................................................................... 9 Topic 2: Patient Perspectives on Treatments for Systemic Sclerosis ....................................... 11 Perspectives on Current Treatments ...................................................................................................... 11 Perspectives on Ideal -
Rheumatology?
WHAT IS RHEUMATOLOGY? diseases affect nearly 50 million Americans and can cause joint and organ destruction, severe pain, disability and even death. Inflammatory rheumatic diseases with arthritis cause more disability in America than heart disease, cancer or diabetes. How can a Rheumatologist help? Most rheumatologic conditions previously led to severe disability and even death in many patients. Evidence-based medical treatment of rheumatological disorders is currently helping patients with rheumatism lead a near-normal life. Medications such as Methotrexate and Tumor necrosis factor inhibitors have had a significant impact on patients with Rheumatoid Arthritis (RA), and today patients with RA can lead a pain free and productive life. Rheumatology facts by numbers: • Over 7 million American adults suffer from inflam- matory rheumatic diseases; 1.3 million adults have rheumatoid arthritis; and 161,000 to 322,000 adults have lupus. • 8.4 percent of women will develop a rheumatic dis- heumatology is a rapidly ease during their lifetime. Women are 2 to 3 times R evolving subspecialty in more likely to be diagnosed with RA, and 10 times internal medicine and more likely to develop lupus than men. pediatrics which includes the pathogenesis, diagnosis, and • 5 percent of men in the U.S. will develop a rheu- management of over 100 complex inflammatory and matic disease during their lifetime. connective tissue diseases. Rheumatologists care for a wide • Osteoporosis and low bone mass are currently esti- array of patients – from children to senior citizens, see mated to be a major public health threat for almost diseases like Rheumatoid Arthritis, Systemic Lupus 44 million U.S. -
2017 American College of Rheumatology/American Association
Arthritis Care & Research Vol. 69, No. 8, August 2017, pp 1111–1124 DOI 10.1002/acr.23274 VC 2017, American College of Rheumatology SPECIAL ARTICLE 2017 American College of Rheumatology/ American Association of Hip and Knee Surgeons Guideline for the Perioperative Management of Antirheumatic Medication in Patients With Rheumatic Diseases Undergoing Elective Total Hip or Total Knee Arthroplasty SUSAN M. GOODMAN,1 BRYAN SPRINGER,2 GORDON GUYATT,3 MATTHEW P. ABDEL,4 VINOD DASA,5 MICHAEL GEORGE,6 ORA GEWURZ-SINGER,7 JON T. GILES,8 BEVERLY JOHNSON,9 STEVE LEE,10 LISA A. MANDL,1 MICHAEL A. MONT,11 PETER SCULCO,1 SCOTT SPORER,12 LOUIS STRYKER,13 MARAT TURGUNBAEV,14 BARRY BRAUSE,1 ANTONIA F. CHEN,15 JEREMY GILILLAND,16 MARK GOODMAN,17 ARLENE HURLEY-ROSENBLATT,18 KYRIAKOS KIROU,1 ELENA LOSINA,19 RONALD MacKENZIE,1 KALEB MICHAUD,20 TED MIKULS,21 LINDA RUSSELL,1 22 14 23 17 ALEXANDER SAH, AMY S. MILLER, JASVINDER A. SINGH, AND ADOLPH YATES Guidelines and recommendations developed and/or endorsed by the American College of Rheumatology (ACR) are intended to provide guidance for particular patterns of practice and not to dictate the care of a particular patient. The ACR considers adherence to the recommendations within this guideline to be volun- tary, with the ultimate determination regarding their application to be made by the physician in light of each patient’s individual circumstances. Guidelines and recommendations are intended to promote benefi- cial or desirable outcomes but cannot guarantee any specific outcome. Guidelines and recommendations developed and endorsed by the ACR are subject to periodic revision as warranted by the evolution of medi- cal knowledge, technology, and practice. -
Gether with Anti -Synthetase, Ro52 and Jo-1-Double Positive: High Rate of Malignancies, Poorer E.G
Journal of Neuromuscular Diseases 5 (2018) 109–129 109 DOI 10.3233/JND-180308 IOS Press Review Current Classification and Management of Inflammatory Myopathies Jens Schmidt∗ Department of Neurology, Muscle Immunobiology Group, Neuromuscular Center, University Medical Center G¨ottingen, G¨ottingen, Germany Abstract. Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with myositis (overlap myositis, OM) including anti-synthetase syndrome (ASS), and inclusion body myositis (IBM). Whereas DM occurs in children and adults, all other forms of myositis mostly develop in middle aged individuals. Apart from a slowly progressive, chronic disease course in IBM, patients with myositis typically present with a subacute onset of weakness of arms and legs, often associated with pain and clearly elevated creatine kinase in the serum. PM, DM and most patients with NM and OM usually respond to immunosuppressive therapy, whereas IBM is largely refractory to treatment. The diagnosis of myositis requires careful and combinatorial assessment of (1) clinical symptoms including pattern of weakness and paraclinical tests such as MRI of the muscle and electromyogra- phy (EMG), (2) broad analysis of auto-antibodies associated with myositis, and (3) detailed histopathological work-up of a skeletal muscle biopsy. This review provides a comprehensive overview of the current classification, diagnostic pathway, treatment regimen and pathomechanistic understanding of myositis. Keywords: Skeletal muscle, muscle inflammation, myositis, immunosuppression, neuroinflammation, autoimmunity INTRODUCTION requires testing of auto-antibodies, histological eval- uation of a skeletal muscle biopsy and further tests Inflammatory myopathies (synonym: idiopathic including muscle MRI and EMG. Novel diagnostic inflammatory myopathy, IIM) –in short: myositis– criteria have recently been established, but an update are rare conditions that can affect multiple organs will be required (see below for details). -
An Overview of Various Diagnostic Methods to Detect Antinuclear Antibodies of Connective Tissue Diseases
DOI: 10.7860/NJLM/2021/48042:2492 Review Article An Overview of Various Diagnostic Methods to Detect Antinuclear Antibodies of Microbiology Section Microbiology Connective Tissue Diseases SAIKEERTHANA DURAISAMY ABSTRACT Antinuclear Antibodies (ANA) are present in many autoimmune disorders and these disorders are collectively called as Connective Tissue Diseases (CTD). There are various CTDs which include Systemic Lupus Erythematosus (SLE), Sjogren’s syndrome, Systemic Sclerosis (SS), Inflammatory Myositis (IM), Mixed Connective Tissue Disorder (MCTD) and Rheumatoid Arthritis (RA). Detection of ANAs in these CTDs is highly sensitive and is of utmost importance. The ANAs specific to SLE includes antidouble stranded Deoxyribonucleic Acid (antidsDNA), single stranded DNA (ssDNA). Scleroderma or Systemic Sclerosis (SS) is an immune mediated rheumatic disease where autoantibodies like topoisomerase 1, Ribonucleic Acid (RNA) polymerase 1 and fibrillarin are useful in diagnosis. Idiopathic Inflammatory Myositis (IIM) such as polymyositis and dermatomyositis are characterised by the presence of autoantibodies like PM-scl (Polymyositis-Scleromyositis), Mi-1 (Myositis specific autoantibody found in idiopathic inflammatory myositis), Mi-2 and Ku (DNA Binding Protein in dermatomyositis). Antibody titres against polypeptides on the U1 Ribonucleoprotein (U1RNP) is useful in the detection mixed CTD. Sjogren’s syndrome is characterised by the presence of serum autoantibodies against two ribonucleoproteinic complexes like Ro/SSA (Extractable nuclear antigen found in Sjogren's syndrome related antigen A auto antibodies) and La/SSB (Extractable nuclear antigen found in Sjogren's syndrome or lupus erythematous). ANA analysis can be done by techniques like indirect immunofluorescence method, Enzyme Linked Immuno Sorbent Assay (ELISA), Immunoprecipitation in agar and western blotting. All these diagnostic methods give precise identification of these antibodies with high accuracy. -
Rheuma.Stamp Line&Box
Arthritis Mutilans: A Report from the GRAPPA 2012 Annual Meeting Vinod Chandran, Dafna D. Gladman, Philip S. Helliwell, and Björn Gudbjörnsson ABSTRACT. Arthritis mutilans is often described as the most severe form of psoriatic arthritis. However, a widely agreed on definition of the disease has not been developed. At the 2012 annual meeting of the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA), members hoped to agree on a definition of arthritis mutilans and thus facilitate clinical and molecular epidemiological research into the disease. Members discussed the clinical features of arthritis mutilans and defini- tions used by researchers to date; reviewed data from the ClASsification for Psoriatic ARthritis study, the Nordic psoriatic arthritis mutilans study, and the results of a premeeting survey; and participated in breakout group discussions. Through this exercise, GRAPPA members developed a broad consensus on the features of arthritis mutilans, which will help us develop a GRAPPA-endorsed definition of arthritis mutilans. (J Rheumatol 2013;40:1419–22; doi:10.3899/ jrheum.130453) Key Indexing Terms: OSTEOLYSIS ANKYLOSIS PENCIL-IN-CUP SUBLUXATION FLAIL JOINT ARTHRITIS MUTILANS Psoriatic arthritis (PsA) is an inflammatory musculoskeletal gists as a severe destructive form of PsA, a precise disease specifically associated with psoriasis. Moll and definition has not yet been universally accepted. The earliest Wright defined PsA as “psoriasis associated with inflam- definition of arthritis mutilans was provided -
Allergy and Immunology Milestones
Allergy and Immunology Milestones The Accreditation Council for Graduate Medical Education Second Revision: August 2019 First Revision: August 2013 Allergy and Immunology Milestones The Milestones are designed only for use in evaluation of residents in the context of their participation in ACGME-accredited residency or fellowship programs. The Milestones provide a framework for the assessment of the development of the resident in key dimensions of the elements of physician competency in a specialty or subspecialty. They neither represent the entirety of the dimensions of the six domains of physician competency, nor are they designed to be relevant in any other context. i Allergy and Immunology Milestones Work Group Amal Assa’ad, MD Evelyn Lomasney, MD Taylor Atchley, MD Aidan Long, MD T. Prescott Atkinson, MD, PhD Mike Nelson, MD Laura Edgar, EdD, CAE Princess Ogbogu, MD Beverly Huckman, BA* Kelly Stone, MD, PhD Bruce Lanser, MD The ACGME would like to thank the following organizations for their continued support in the development of the Milestones: American Board of Allergy and Immunology American Academy of Allergy, Asthma, and Immunology Review Committee for Allergy and Immunology *Acknowledgments: The Work Group and the ACGME would like to honor Beverly Huckman, for her contributions as the non-physician member of the milestones work group. She will be greatly missed. ii Understanding Milestone Levels and Reporting This document presents the Milestones, which programs use in a semi-annual review of resident performance, and then report to the ACGME. Milestones are knowledge, skills, attitudes, and other attributes for each of the ACGME Competencies organized in a developmental framework. -
Rheumatology Connections
IN THIS ISSUE Uncommon Manifestations of SLE 3 | PACNS or RCVS? 6 | Psychosocial Burden of PsA 9 Advances in IL-6 Biology 10 | AHCT for Systemic Sclerosis 12 | PET in Large Vessel Vasculitis 14 Rheumatology Connections An Update for Physicians | Summer 2018 112236_CCFBCH_18RHE1077_ACG.indd 1 7/10/18 2:06 PM Dear Colleagues, Welcome to another issue of Rheumatology Con- From the Chair of Rheumatic nections. I think you’ll find our title perfectly apt as and Immunologic Diseases you browse these articles, full of connections to an array of other specialties, to our patients and to our clinical, scientific and educational missions. Dr. Chatterjee’s collaboration with the Department of Medical Oncology and Hematology for treating severe systemic sclerosis is but one example of the relevance of our field across disciplines (p. 12). Cleveland Clinic’s Rheumatology This issue also features a study of psychosocial fac- Program is ranked among the top tors in psoriatic arthritis co-authored by Dr. Husni 2 in the nation in U.S. News & and a colleague in the Neurological Institute (p. 9). World Report’s “America’s Best Our connections to neurology become even clearer Hospitals” survey. as Drs. Calabrese and Hajj-Ali offer guidance on distinguishing between a neurological condition and the more fatal, less common rheumatologic one that it mimics (p. 6). Rheumatology Connections, published by We are always searching for ways to transcend Cleveland Clinic’s Department of Rheumatic disciplinary boundaries to provide better patient care. Our multidisciplinary style of caring for patients was and Immunologic Diseases, provides information critical in a case co-authored with a colleague in pathology and Drs. -
Graft-Versus-Host Disease Cells Suppresses Development Of
Adenosine A2A Receptor Agonist −Mediated Increase in Donor-Derived Regulatory T Cells Suppresses Development of Graft-versus-Host Disease This information is current as of September 28, 2021. Kyu Lee Han, Stephenie V. M. Thomas, Sherry M. Koontz, Cattlena M. Changpriroa, Seung-Kwon Ha, Harry L. Malech and Elizabeth M. Kang J Immunol 2013; 190:458-468; Prepublished online 7 December 2012; Downloaded from doi: 10.4049/jimmunol.1201325 http://www.jimmunol.org/content/190/1/458 http://www.jimmunol.org/ References This article cites 52 articles, 20 of which you can access for free at: http://www.jimmunol.org/content/190/1/458.full#ref-list-1 Why The JI? Submit online. • Rapid Reviews! 30 days* from submission to initial decision • No Triage! Every submission reviewed by practicing scientists by guest on September 28, 2021 • Fast Publication! 4 weeks from acceptance to publication *average Subscription Information about subscribing to The Journal of Immunology is online at: http://jimmunol.org/subscription Permissions Submit copyright permission requests at: http://www.aai.org/About/Publications/JI/copyright.html Email Alerts Receive free email-alerts when new articles cite this article. Sign up at: http://jimmunol.org/alerts The Journal of Immunology is published twice each month by The American Association of Immunologists, Inc., 1451 Rockville Pike, Suite 650, Rockville, MD 20852 All rights reserved. Print ISSN: 0022-1767 Online ISSN: 1550-6606. The Journal of Immunology Adenosine A2A Receptor Agonist–Mediated Increase in Donor-Derived Regulatory T Cells Suppresses Development of Graft-versus-Host Disease Kyu Lee Han,* Stephenie V. M. Thomas,* Sherry M.