Differences in the Pattern of Muscular and Extramuscular Involvement In
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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. -
Azathioprine in Connective Tissue Disease-Associated Interstitial Lung Diseases
Disease ng s u & Aldehaim et al., Lung Dis Treat 2019, 5:1 L T f r o e a l t DOI: 10.4172/2472-1018.1000132 a m n r e u n o t J Journal of Lung Diseases & Treatment ISSN: 2472-1018 Research Article Open Access Azathioprine in Connective Tissue Disease-Associated Interstitial Lung Diseases. How Valuable? Aldehaim AY1*, AboAbat A1,2 and Alshabanat A1,3 1Department of Medicine, King Saud University, Riyadh, Saudi Arabia 2Department of Medicine, University of Toronto, Toronto, Canada 3Department of Medicine, McMaster University, Hamilton, Canada Abstract Objective: To systematically review the use of azathioprine as a treatment for connective tissue disease- associated interstitial lung disease (CTD-ILD) in terms of effectiveness and safety. Materials and methods: A literature search was performed using the PubMed, EMBASE, CINAHL, Cochrane, and Scopus databases. The search was restricted to articles published in English from 1950 to March 2018 that examined the use of azathioprine in patients with CTD-ILD and determined its effects on a primary or secondary endpoint. This review included studies that measured the impacts of azathioprine in terms of effectiveness and safety. Results: The search identified 15 studies with a total of 424 subjects. Two hundred twenty patients received azathioprine. A majority of the studies failed to provide clear evidence for the effectiveness of azathioprine. The reported adverse events were: death 4.5% (n=10), infection 1.3% (n=3), myelosuppression 0.9% (n=2), and malignancy 0.45% (n=1). The rate of azathioprine discontinuation due to treatment failure was 2.7% (n=6). -
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. -
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. -
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. -
Pulmonary Hypertension in Antisynthetase Syndrome: Prevalence, Aetiology and Survival
ORIGINAL ARTICLE PULMONARY VASCULAR DISEASE Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival Baptiste Hervier1, Alain Meyer2,Ce´line Dieval3, Yurdagul Uzunhan4,5, Herve´ Devilliers6, David Launay7, Matthieu Canuet8, Laurent Teˆtu9, Christian Agard10, Jean Sibilia2, Mohamed Hamidou10, Zahir Amoura1, Hilario Nunes4,5, Olivier Benveniste11, Philippe Grenier12, David Montani13,14 and Eric Hachulla7,14 Affiliations: 1Internal Medicine Dept 2 and INSERM UMRS-945, French Reference Center for Lupus, Hoˆpital Pitie´-Salpeˆtrie`re, APHP, University of Paris VI Pierre and Marie Curie, Paris, 2Rheumatology Dept, French Reference Center for Systemic Rare Diseases, Strasbourg University Hospital, Strasbourg, 3Internal Medicine and Infectious Diseases Dept, St-Andre´ Hospital, University of Bordeaux, Bordeaux, 4University of Paris 13, Sorbonne Paris Cite´, EA 2363, Paris, 5Dept of Pneumology, AP-HP, Avicenne Hospital, Bobigny, 6Internal Medicine and Systemic Disease Dept, University Hospital of Dijon, Dijon, 7Internal Medicine Dept, French National Center for Rare Systemic Auto-Immune Diseases (Scleroderma), Claude Huriez Hospital, Lille 2 University, Lille, 8Pneumology Dept, Strasbourg University Hospital, Strasbourg, 9Pneumology Dept, Larrey Hospital, Paul Sabatier University, Toulouse, 10Internal Medicine Dept, Hoˆtel Dieu, Nantes University, Nantes, 11Internal Medicine Dept 1, French Reference Center for Neuromuscular Disorders, Hoˆpital Pitie´-Salpeˆtrie`re, APHP, University of Paris VI Pierre and Marie Curie, Paris, 12Radiology Dept, Hoˆpital Pitie´-Salpeˆtrie`re, APHP, University of Paris VI Pierre and Marie Curie, Paris, and 13Pneumology Dept, APHP, DHU Thorax Innovation, INSERM UMRS-999, Centre de Re´fe´rence de l’Hypertension Pulmonaire Se´ve`re, Hoˆpital Universitaire de Biceˆtre, Le Kremlin-Biceˆtre, Paris, France. 14These authors contributed equally to this work. Correspondence: B. Hervier, Service de Me´decine Interne 2, Centre National de re´fe´rence du Lupus, 47–83 boulevard de l’hoˆpital, 75651 Paris cedex 13, France. -
Autoantibodies in Systemic Autoimmune Diseases K
umschlag_neutral.qxd 03.10.2007 10:53 Seite 1 2nd edition Karsten Conrad, Werner Schößler, Falk Hiepe, Marvin J. Fritzler Autoantibodies are a very heterogeneous group of antibodies with respect to their specificity, induction, effects, and clinical signifi- cance. Testing for autoantibodies can be helpful or necessary for the diagnosis, differential diagnosis, prognostication, or monitoring of Autoantibodies in Systemic autoimmune diseases. In case of limited (forme fruste) disease or a single disease manifestation, the detection of serum autoantibodies can play an Autoimmune Diseases important role in raising the suspicion of evolving disease and forecasting prog- nosis. This book and reference guide is intended to assist the physician in under- A Diagnostic Reference standing and interpreting the variety of autoantibodies that are being used as diagnostic and prognostic tools for patients with systemic rheumatic diseases. Autoantibodies observed in systemic autoimmune diseases are described in alphabetical order in Part 1 of this reference guide. In Part 2, systemic autoim- mune disorders as well as symptoms that indicate the possible presence of an autoimmune disease are listed. Systemic manifestations of organ-specific autoim- mune diseases will not be covered in this volume. Guide marks were inserted to K. Conrad, W.K. Conrad, Hiepe, M. J. Fritzler F. Schößler, ensure fast and easy cross-reference between symptoms, a given autoimmune disease and associated autoantibodies. Although the landscape of autoantibody testing continues to change, this information will be a useful and valuable refer- ence for many years to come. AUTOANTIGENS, AUTOANTIBODIES, AUTOIMMUNITY Autoantibodies in Systemic Autoimmune Diseases Autoimmune in Systemic Autoantibodies Volume 2, second Edition – 2007 ISBN 978-3-89967-420-0 www.pabst-publishers.com PABST Autoantibodies in Systemic Autoimmune Diseases A Diagnostic Reference Karsten Conrad, Werner Schößler, Falk Hiepe, Marvin J. -
Hypersensitivity Reactions (Types I, II, III, IV)
Hypersensitivity Reactions (Types I, II, III, IV) April 15, 2009 Inflammatory response - local, eliminates antigen without extensively damaging the host’s tissue. Hypersensitivity - immune & inflammatory responses that are harmful to the host (von Pirquet, 1906) - Type I Produce effector molecules Capable of ingesting foreign Particles Association with parasite infection Modified from Abbas, Lichtman & Pillai, Table 19-1 Type I hypersensitivity response IgE VH V L Cε1 CL Binds to mast cell Normal serum level = 0.0003 mg/ml Binds Fc region of IgE Link Intracellular signal trans. Initiation of degranulation Larche et al. Nat. Rev. Immunol 6:761-771, 2006 Abbas, Lichtman & Pillai,19-8 Factors in the development of allergic diseases • Geographical distribution • Environmental factors - climate, air pollution, socioeconomic status • Genetic risk factors • “Hygiene hypothesis” – Older siblings, day care – Exposure to certain foods, farm animals – Exposure to antibiotics during infancy • Cytokine milieu Adapted from Bach, JF. N Engl J Med 347:911, 2002. Upham & Holt. Curr Opin Allergy Clin Immunol 5:167, 2005 Also: Papadopoulos and Kalobatsou. Curr Op Allergy Clin Immunol 7:91-95, 2007 IgE-mediated diseases in humans • Systemic (anaphylactic shock) •Asthma – Classification by immunopathological phenotype can be used to determine management strategies • Hay fever (allergic rhinitis) • Allergic conjunctivitis • Skin reactions • Food allergies Diseases in Humans (I) • Systemic anaphylaxis - potentially fatal - due to food ingestion (eggs, shellfish, -
Antisynthetase Syndrome and Rheumatoid Arthritis: a Rare Overlapping Disease
Case Report Annals of Clinical Case Reports Published: 15 Jul, 2020 Antisynthetase Syndrome and Rheumatoid Arthritis: A Rare Overlapping Disease Makhlouf Yasmine*, Miladi Saoussen, Fazaa Alia, Sallemi Mariem, Ouenniche Kmar, Leila Souebni, Kassab Selma, Chekili Selma, Zakraoui Leith, Ben Abdelghani Kawther and Laatar Ahmed Department of Rheumatology, Mongi Slim Hospital, Tunisia Abstract The association between Antisynthetase Syndrome (ASS) and rheumatoid arthritis is extremely rare. In this case report, we are describing a 16 years long standing history of seropositive RA before its uncommon association to an ASS. A 55-year-old female patient presented at the first visit with symmetric polyarthritis and active synovitis affecting both hands and ankles. Laboratory investigations showed positive rheumatoid factors, positive anti-CCP antibodies and negative ANA. The X-rays were consistent with typical erosive in hands. Thus, the patient fulfilled the ACR 1987 criteria of RA in 2000 at the age of 37. Methotrexate was firstly prescribed. However, it was ineffective after 4 years. Then, the patient did well with Leflunomide until January 2017, when she developed exertional dyspnea. High-resolution CT of the lung revealed Nonspecific Interstitial Pneumonia (NSIP). Autoantibodies against extractable nuclear antigens were screened and showed positive results for anti-Jo1 autoantibodies. She was diagnosed with ASS complicating the course of RA. Keywords: Antisynthetase syndrome; Rheumatoid arthritis; Overlap syndrome; Nonspecific interstitial pneumonia Key Points • Antisynthetase Syndrome should be considered as a clinical manifestation of overlap syndromes, particularly in active RA patients with pulmonary signs and anti-Jo-1 antibody. OPEN ACCESS • An early diagnosis of antisynthetase syndrome in an overlap syndrome is important, as *Correspondence: treatment may need adjustment. -
What You Need to Know About the New Guidelines for the Diagnosis and Management of Food Allergy in the U.S
Allergy guidelines insert_Layout 1 9/26/11 1:36 PM Page 1 What you need to know about the new guidelines for the diagnosis and management of food allergy in the U.S. V OLUME 126, N O . 6 D ECEMBER 2010 • Tests for food-specific IgE are recom- Overview www.jacionline.org • The Guidelines, sponsored by the NIH Supplement to mended to assist in diagnosis, but should (NIAID), are based upon expert opinion THE JOURNAL OF not be relied upon as a sole means to di- Allergy ANDClinical and a comprehensive literature review. Immunology agnose food allergy. The medical history/ AAP had input on the document.1,2 exam are recommended to aid in diag- nosis. A medically monitored feeding Guidelines for the Diagnosis and Management Definitions of Food Allergy in the United States: Report of the (food challenge) is considered the most NIAID-Sponsored Expert Panel • Food allergy was defined as an adverse definitive test for food allergy. health effect arising from a specific im- • Food-specific IgE testing has numerous mune response. limitations; positive tests are not intrin- • Food allergies result in IgE-mediated sically diagnostic and reactions some- immediate reactions (e.g., anaphylaxis) OFFICIAL JOURNAL OF times occur with negative tests. These and several chronic diseases (e.g., ente- Supported by the Food Allergy Initiative issues are also reviewed in an AAP Clini - rocolitis syndromes, eosinophilic esopha - cal Report.3 Testing “food panels” with- gitis, etc), in which IgE may not play an important role. out considering history is often mis - leading. Tests selected to evaluate food allergy should be Epidemiology and Natural History based on the patient’s medical history and not comprise • Food allergy is more common in children than adults, large general panels of food allergens. -
That Are Not Lielu Uutuullittu
THAT ARE NOT LIELUUS009987356B2 UUTUULLITTU (12 ) United States Patent ( 10 ) Patent No. : US 9 ,987 , 356 B2 Reimann et al. ( 45) Date of Patent : Jun . 5 , 2018 (54 ) ANTI -CD40 ANTIBODIES AND METHODS 762 A 12 / 1997 Queen et al. 5 , 801, 227 A 9 / 1998 Fanslow , III et al. OF ADMINISTERING THEREOF 5 , 874 ,082 A 2 / 1999 de Boer 6 ,004 , 552 A 12 / 1999 de Boer et al. ( 75 ) Inventors: Keith A . Reimann , Marblehead , MA 6 ,051 ,228 A 4 / 2000 Aruffo et al. (US ) ; Rijian Wang , Saugus, MA (US ) ; 6 ,054 ,297 A 4 / 2000 Carter et al . Christian P . Larsen , Atlanta , GA (US ) 6 ,056 , 959 A 5 /2000 de Boer et al. 6 , 132 , 978 A 10 /2000 Gelfand et al. 6 , 280 , 957 B18 / 2001 Sayegh et al. ( 73) Assignees : Beth Israel Deaconess Medical 6 , 312 ,693 B1 11/ 2001 Aruffo et al. Center , Inc ., Boston , MA (US ) ; Emory 6 , 315 , 998 B111 / 2001 de Boer et al. University , Atlanta , GA (US ) 6 , 413 ,514 B1 7 / 2002 Aruffo et al. 6 , 632, 927 B2 10 /2003 Adair et al. ( * ) Notice : Subject to any disclaimer , the term of this 7 ,063 , 845 B2 6 / 2006 Mikayama et al. patent is extended or adjusted under 35 7 , 193 , 064 B2 3 / 2007 Mikayama et al. 7 , 288 , 251 B2 10 / 2007 Bedian et al . U . S . C . 154 ( b ) by 464 days . 7 , 361 , 345 B2 4 /2008 de Boer et al. 7 , 445 , 780 B2 11/ 2008 Chu et al. (21 ) Appl . No. -
What Is the Difference Between Allergy, Sensitivity & Intolerance?
What Is the Difference Between Allergy, Sensitivity & Intolerance? The primary difference between an allergy, a sensitivity, and an intolerance is that an allergy is characterized by an immune system reaction to a substance, a sensitivity involves no immune response and an intolerance is characterized by the body lacking a chemical or enzyme needed to digest certain food. All, however, can be quite serious, and a range of symptoms can be caused by allergies, sensitivities, and intolerances. For this reason, it is a good idea to see a doctor about symptoms which appear to be linked to exposure to certain substances, to figure out precisely what is going on. Allergy: Although the word "Allergy" is commonly used to describe any unpleasant reaction to a drug, food, insect sting or chemical, this can be misleading. The word should only really be used to describe a reaction produced when the body meets a normally harmless substance, which has been “remembered" from a previous exposure and subsequently produces the "IgE" antibody. In the case of an allergy, the immune system learns to attack a particular substance for an unknown reason. In order for an allergy to develop, someone must be exposed to the substance at least once before the allergy will manifest. A classic example of an allergy is a peanut allergy, in which the immune system regards peanuts as harmful, and goes into overdrive when someone consumes peanuts or is exposed to peanut products. Some common symptoms linked with allergies are dermatological symptoms like eczema and hives, respiratory problems, anaphylaxis, rhinitis, and shock.