Conditions Related to Inflammatory Arthritis
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Is Palindromic Rheumatism Amongst Children a Benign Disease? Yonatan Butbul-Aviel1,2,3, Yosef Uziel4,5, Nofar Hezkelo4, Riva Brik1,2,3,4 and Gil Amarilyo4,6*
Butbul-Aviel et al. Pediatric Rheumatology (2018) 16:12 https://doi.org/10.1186/s12969-018-0227-z RESEARCHARTICLE Open Access Is palindromic rheumatism amongst children a benign disease? Yonatan Butbul-Aviel1,2,3, Yosef Uziel4,5, Nofar Hezkelo4, Riva Brik1,2,3,4 and Gil Amarilyo4,6* Abstract Background: Palindromic rheumatism is an idiopathic, periodic arthritis characterized by multiple, transient, recurring episodes. Palindromic rheumatism is well-characterized in adults, but has never been reported in pediatric populations. The aim of this study was to characterize the clinical features and outcomes of a series of pediatric patients with palindromic rheumatism. Methods: We defined clinical criteria for palindromic rheumatism and reviewed all clinical visits in three Pediatric Rheumatology centers in Israel from 2006through 2015, to identify patients with the disease. We collected retrospective clinical and laboratory data on patients who fulfilled the criteria, and reviewed their medical records in order to determine the proportion of patients who had developed juvenile idiopathic arthritis. Results: Overall, 10 patients were identified. Their mean age at diagnosis was 8.3 ± 4.5 years and the average follow-up was 3.8 ± 2.7 years. The mean duration of attacks was 12.2 ± 8.4 days. The most frequently involved joints were knees. Patients tested positive for rheumatoid factor in 20% of cases. One patient developed polyarticular juvenile idiopathic arthritis after three years of follow-up, six patients (60%) continued to have attacks at their last follow-up and only three children (30%) achieved long-term remission. Conclusions: Progression to juvenile idiopathic arthritis is rare amongst children with palindromic rheumatism and most patients continued to have attacks at their last follow-up. -
Vaccination and Autoimmune Disease: What Is the Evidence?
REVIEW Review Vaccination and autoimmune disease: what is the evidence? David C Wraith, Michel Goldman, Paul-Henri Lambert As many as one in 20 people in Europe and North America have some form of autoimmune disease. These diseases arise in genetically predisposed individuals but require an environmental trigger. Of the many potential environmental factors, infections are the most likely cause. Microbial antigens can induce cross-reactive immune responses against self-antigens, whereas infections can non-specifically enhance their presentation to the immune system. The immune system uses fail-safe mechanisms to suppress infection-associated tissue damage and thus limits autoimmune responses. The association between infection and autoimmune disease has, however, stimulated a debate as to whether such diseases might also be triggered by vaccines. Indeed there are numerous claims and counter claims relating to such a risk. Here we review the mechanisms involved in the induction of autoimmunity and assess the implications for vaccination in human beings. Autoimmune diseases affect about 5% of individuals in Autoimmune disease and infection developed countries.1 Although the prevalence of most Human beings have a highly complex immune system autoimmune diseases is quite low, their individual that evolved from the fairly simple system found in incidence has greatly increased over the past few years, as invertebrates. The so-called innate invertebrate immune documented for type 1 diabetes2,3 and multiple sclerosis.4 system responds non-specifically to infection, does not Several autoimmune disorders arise in individuals in age- involve lymphocytes, and hence does not display groups that are often selected as targets for vaccination memory. -
Eosinophilic Fasciitis: an Atypical Presentation of a Rare Disease
AT BED SIDE Eosinophilic fasciitis: an atypical presentation of a rare disease Catia Cabral1,2 António Novais1 David Araujo2 Ana Mosca2 Ana Lages2 Anna Knock2 1. Internal Medicine Service, Centro Hospitalar Tondela-Viseu, Viseu, Portugal 2. Internal Medicine Service, Hospital de Braga, Braga, Portugal http://dx.doi.org/10.1590/1806-9282.65.3.326 SUMMARY Eosinophilic fasciitis, or Shulman’s disease, is a rare disease of unknown etiology. It is characterized by peripheral eosinophilia, hyper- gammaglobulinemia, and high erythrocyte sedimentation rate. The diagnosis is confirmed by a deep biopsy of the skin. The first line of treatment is corticotherapy. We present a rare case of eosinophilic fasciitis in a 27-year-old woman with an atypical presentation with symmetrical peripheral ede- ma and a Groove sign. The patient responded well to treatment with corticosteroids at high doses and, in this context, was associated with hydroxychloroquine and azathioprine. After two and a half years, peripheral eosinophilia had increased, and more of her skin had hardened. At that time, the therapy was modified to include corticoids, methotrexate, and penicillamine. It is of great importance to publicize these cases that allow us to gather experience and better treat our patients. KEYWORDS: Fasciitis. Eosinophils. Eosinophilia. Edema/etiology. INTRODUCTION Eosinophilic fasciitis is a rare disease character- among individuals of 40-50 years old and no associa- ized by skin alterations such as scleroderma, pe- tions of race, risk factors, or family history.3 ripheral eosinophilia, hypergammaglobulinemia, The importance of this case is related to the rar- and high erythrocyte sedimentation rate.1 It more ity of the disease, its atypical presentation, and the frequently involves the inferior limbs. -
Assessment of Skin, Joint, Tendon and Muscle Involvement
Assessment of skin, joint, tendon and muscle involvement A. Akesson1, G. Fiori2, T. Krieg3, F.H.J. van den Hoogen4, J.R. Seibold5 1Lund University Hospital, Lund, Sweden; ABSTRACT The extent of skin involvement is also 2Istituto di Clinica Medica IV, Florence, This rep o rt makes re c o m m e n d at i o n s the prime clinical criterion for the sub- Italy; 3University of Cologne, Koln, for standardized techniques of data ga - classification of SSc into its two princi- 4 Germany; University Medical Centre t h e ring and collection rega rd i n g : 1 ) pal subsets – SSc with diffuse cuta- St. Raboud, Nijmegen, The Netherlands; 5UMDNJ Scleroderma Program, skin involvement 2) joint and tendon in - neous involvement (diffuse scleroder- New Brunswick, New Jersey, USA. volvement, and 3) involvement of the ma) and SSc with limited cutaneous Anita Akesson, MD, PhD; Ginevra Fiori, skeletal muscles. The recommendations i nvo l vement (limited scl e ro d e rma – MD; Thomas Krieg, MD; Frank H.J. van in this report derive from a critical re - p rev i o u s ly termed the “CREST syn- den Hoogen, MD, PhD; James R. Seibold, v i ew of the ava i l able literat u re and drome”) (3). By consensus and conven- MD. group discussion. Committee re c o m - t i o n , p atients with skin invo l ve m e n t Please address correspondence to: mendations are considered appropriate restricted to sites distal to the elbows James R. Seibold, MD, Professor and for descri p t ive clinical inve s t i gat i o n , and knees, exclusive of the face, are Director, UMDNJ Scleroderma Program, translational studies and as standards considered to have limited scleroderma MEB 556 51 French Street, New for clinical practice. -
Rotator Cuff Tendon Ruptures and Degeneration As the First Manifestation of Polymyalgia Rheumatica Disease - a Case Report
Open Access Austin Journal of Clinical Case Reports Case Report Rotator Cuff Tendon Ruptures and Degeneration as the First Manifestation of Polymyalgia Rheumatica Disease - A Case Report Bazoukis G1*, Michelongona P2, Papadatos SS1, Pagkalidou E1, Grigoropoulou P1, Fragkou A1 and Abstract Yalouris A1 Polymyalgia Rheumatica (PMR) is a common rheumatic disease of the 1Department of Internal Medicine, General Hospital of elderly. Although it is a well-established disease, its causes and pathophysiology Athens “Elpis”, Greece remain unclear. In our case report we present an 83-year-old female presented 2Department of Internal Medicine, General Hospital of at the emergency department because of fever and diarrhea. Her medical Korinthos, Greece history included a recent orthopedic surgery because of tendons rupture of the *Corresponding author: George Bazoukis, rotator cuff. Her blood exams showed increased inflammatory markers and a Department of Internal Medicine, General Hospital of three-digit ESR. The diagnosis of PMR was set after the exclusion of infectious Athens “Elpis”, Greece and other diseases that mimic PMR symptoms. To the best of our knowledge, it is the first time that rotator cuff tendons rupture and degeneration is the first Received: June 05, 2016; Accepted: August 02, 2016; manifestation of PMR disease. Clinicians should be aware of the degeneration Published: September 08, 2016 of the shoulder and hip extra-articular structures in PMR and they should keep in mind that it can be the first manifestation of the disease. Keywords: Polymyalgia rheumatica; Rotator cuff denegeration; Tendon rupture Introduction and infraspinatus muscles as well as significant tendinopathy of the subscapularis and long head of biceps muscles. -
James Albers, MD Phd Kirsten Gruis, MD Revised 10/2010
James Albers, MD PhD Kirsten Gruis, MD Revised 10/2010 RADICULOPATHY I. Focal Radiculopathy A. Definitions: 1. Pathological process affecting dorsal (sensory) and/or ventral (motor) spinal roots 2. Clinically includes roots, DRG (dorsal root ganglion) and spinal nerves. B. Clinical Characteristics: 1. Pain may be out of proportion to objective deficit. 2. If chronic, radiculopathy can be asymptomatic. 3. Features favoring radiculopathy vs plexopathy/mononeuropathy a. Proximal pain (neck, low back) b. Pain with movement (tilting neck, lumbar extension) c. Pain with cough, sneeze, Valsalva C. Variables in localization: 1. Nerve damage varies in severity 2. Dermatomal and Myotomal distributions overlap: a. Masks objective deficits b. Enlarges positive phenomena (pain) 3. Pain may also be referred. 4. Involvement of multiple roots may confuse localization. 5. Variable anatomy, especially motor 4. If pain reproduced by palpation then higher suspicion for musculoskeletal disorder mimicking radiculopathy (see Table 1 and 2). However, pain to palpation does not exclude a radiculopathy or abnormal EDX test. 5. 32% of patients referred for EMG lab for lumbosacral radiculopathy have a musculoskeletal disorder. Page 1 of 11 Table 1 Musculoskeletal conditions that commonly mimic cervical radiculopathy Condition Clinical symptoms/signs Fibromyalgia syndrome Pain all over, female predominance, often sleep problems, tender to palpation in multiple areas Polymyalgia rheumatica >50 years old, pain and stiffness in neck, shoulder and hips, high erythrocyte -
Juvenile Spondyloarthropathies: Inflammation in Disguise
PP.qxd:06/15-2 Ped Perspectives 7/25/08 10:49 AM Page 2 APEDIATRIC Volume 17, Number 2 2008 Juvenile Spondyloarthropathieserspective Inflammation in DisguiseP by Evren Akin, M.D. The spondyloarthropathies are a group of inflammatory conditions that involve the spine (sacroiliitis and spondylitis), joints (asymmetric peripheral Case Study arthropathy) and tendons (enthesopathy). The clinical subsets of spondyloarthropathies constitute a wide spectrum, including: • Ankylosing spondylitis What does spondyloarthropathy • Psoriatic arthritis look like in a child? • Reactive arthritis • Inflammatory bowel disease associated with arthritis A 12-year-old boy is actively involved in sports. • Undifferentiated sacroiliitis When his right toe starts to hurt, overuse injury is Depending on the subtype, extra-articular manifestations might involve the eyes, thought to be the cause. The right toe eventually skin, lungs, gastrointestinal tract and heart. The most commonly accepted swells up, and he is referred to a rheumatologist to classification criteria for spondyloarthropathies are from the European evaluate for possible gout. Over the next few Spondyloarthropathy Study Group (ESSG). See Table 1. weeks, his right knee begins hurting as well. At the rheumatologist’s office, arthritis of the right second The juvenile spondyloarthropathies — which are the focus of this article — toe and the right knee is noted. Family history is might be defined as any spondyloarthropathy subtype that is diagnosed before remarkable for back stiffness in the father, which is age 17. It should be noted, however, that adult and juvenile spondyloar- reported as “due to sports participation.” thropathies exist on a continuum. In other words, many children diagnosed with a type of juvenile spondyloarthropathy will eventually fulfill criteria for Antinuclear antibody (ANA) and rheumatoid factor adult spondyloarthropathy. -
The Painful Heel Comparative Study in Rheumatoid Arthritis, Ankylosing Spondylitis, Reiter's Syndrome, and Generalized Osteoarthrosis
Ann Rheum Dis: first published as 10.1136/ard.36.4.343 on 1 August 1977. Downloaded from Annals of the Rheumatic Diseases, 1977, 36, 343-348 The painful heel Comparative study in rheumatoid arthritis, ankylosing spondylitis, Reiter's syndrome, and generalized osteoarthrosis J. C. GERSTER, T. L. VISCHER, A. BENNANI, AND G. H. FALLET From the Department of Medicine, Division of Rheumatology, University Hospital, Geneva, Switzerland SUMMARY This study presents the frequency of severe and mild talalgias in unselected, consecutive patients with rheumatoid arthritis, ankylosing spondylitis, Reiter's syndrome, and generalized osteoarthosis. Achilles tendinitis and plantar fasciitis caused a severe talalgia and they were observed mainly in males with Reiter's syndrome or ankylosing spondylitis. On the other hand, sub-Achilles bursitis more frequently affected women with rheumatoid arthritis and rarely gave rise to severe talalgias. The simple calcaneal spur was associated with generalized osteoarthrosis and its frequency increased with age. This condition was not related to talalgias. Finally, clinical and radiological involvement of the subtalar and midtarsal joints were observed mainly in rheumatoid arthritis and occasionally caused apes valgoplanus. copyright. A 'painful heel' syndrome occurs at times in patients psoriasis, urethritis, conjunctivitis, or enterocolitis. with inflammatory rheumatic disease or osteo- The antigen HLA B27 was present in 29 patients arthrosis, causing significant clinical problems. Very (80%O). few studies have investigated the frequency and characteristics of this syndrome. Therefore we have RS 16 PATIENTS studied unselected groups of patients with rheuma- All of our patients had the complete triad (non- toid arthritis (RA), ankylosing spondylitis (AS), gonococcal urethritis, arthritis, and conjunctivitis). -
Psoriasis, a Systemic Disease Beyond the Skin, As Evidenced by Psoriatic Arthritis and Many Comorbities
1 Psoriasis, a Systemic Disease Beyond the Skin, as Evidenced by Psoriatic Arthritis and Many Comorbities – Clinical Remission with a Leishmania Amastigotes Vaccine, a Serendipity Finding J.A. O’Daly Astralis Ltd, Irvington, NJ USA 1. Introduction Psoriasis is a systemic chronic, relapsing inflammatory skin disorder, with worldwide distribution, affects 1–3% of the world population, prevalence varies according to race, geographic location, and environmental factors (Chandran & Raychaudhuri, 2010; Christophers & Mrowietz, 2003; Farber & Nall, 1974). In Germany, 33,981 from 1,344,071 continuously insured persons in 2005 were diagnosed with psoriasis; thus the one year prevalence was 2.53% in the study group. Up to the age of 80 years the prevalence rate (range: 3.99-4.18%) was increasing with increasing age and highest for the age groups from 50 to 79 years The total rate of psoriasis in children younger than 18 years was 0.71%. The prevalence rates increased in an approximately linear manner from 0.12% at the age of 1 year to 1.2% at the age of 18 years (Schäfer et al., 2011). In France, a case-control study in 6,887 persons, 356 cases were identified (5.16%), who declared having had psoriasis during the previous 12 months (Wolkenstein et al., 2009). The prevalence of psoriasis analyzed across Italy showed that 2.9% of Italians declared suffering from psoriasis (regional range: 0.8-4.5%) in a total of 4109 individuals (Saraceno et al., 2008). The overall rate of comorbidity in subjects with psoriasis aged less than 20 years was twice as high as in subjects without psoriasis. -
The Pathomechanics of Plantar Fasciitis
Sports Med 2006; 36 (7): 585-611 REVIEW ARTICLE 0112-1642/06/0007-0585/$39.95/0 2006 Adis Data Information BV. All rights reserved. The Pathomechanics of Plantar Fasciitis Scott C. Wearing,1 James E. Smeathers,1 Stephen R. Urry,1 Ewald M. Hennig2 and Andrew P. Hills1 1 Institute of Health and Biomedical Innovation, Queensland University of Technology, Kelvin Grove, Queensland, Australia 2 Biomechanik Labor, University Duisburg-Essen, Essen, Germany Contents Abstract ....................................................................................585 1. Anatomy of the Plantar Fascia ............................................................586 1.1 Gross Anatomy of the Plantar Fascia...................................................586 1.2 Histological Anatomy of the Plantar Fascia .............................................589 1.2.1 Neuroanatomy ................................................................590 1.2.2 Microcirculation ...............................................................590 1.2.3 The Enthesis ...................................................................590 2. Mechanical Properties of the Plantar Fascia ................................................592 2.1 Structural Properties ..................................................................593 2.2 Material Properties ...................................................................593 3. Mechanical Function of the Plantar Fascia .................................................594 3.1 Quasistatic Models of Plantar Fascial Function ..........................................594 -
Arthritis and Coeliac Disease
Ann Rheum Dis: first published as 10.1136/ard.44.9.592 on 1 September 1985. Downloaded from Annals of the Rheumatic Diseases 1985, 44, 592-598 Arthritis and coeliac disease J T BOURNE,' P KUMAR,2 E C HUSKISSON,' R MAGEED 3 D J UNSWORTH,3 AND J A WOJTULEWSKI4 From the Departments of 'Rheumatology and 2Gastroenterology, St Bartholomew's Hospital, West Smithfield, London ECIA 7BE; the 3Bone and Joint Research Unit, London Hospital Medical College, London El; and 4St Mary's Hospital, Eastbourne SUMMARY We report six patients with coeliac disease in whom arthritis was prominent at diagnosis and who improved with dietary therapy. Joint pain preceded diagnosis by up to three years in five patients and 15 years in one patient. Joints most commonly involved were lumbar spine, hips, and knees (four cases). In three cases there were no bowel symptoms. All were seronegative. X-rays were abnormal in two cases. HLA-type Al, B8, DR3 was present in five and B27 in two patients. Circulating immune complexes showed no consistent pattern before or after treatment. Coeliac disease was diagnosed in all patients by jejunal biopsy, and joint symptoms in all responded to a gluten-free diet. Gluten challenge (for up to three weeks) failed to provoke arthritis in three patients tested. In a separate study of 160 treated coeliac patients attending regular follow up no arthritis attributable to coeliac disease and no ankylosing was in a group spondylitis identified, though control of 100 patients with Crohn's disease thecopyright. expected incidence of seronegative polyarthritis (23%) and ankylosing spondylitis (5%) was found (p<0.01). -
Palindromic Rheumatism Or When Do You Decide to Treat an Asymptomatic Seropositive RA Patient? What Is This?
Palindromic Rheumatism or When do you decide to treat an asymptomatic seropositive RA patient? What Is This? • 11.15.15 • 56 yo man comes for 2nd opinion for bouts of severe large joint monoarthritis lasting 24 hours or longer. • Vague about duration “10-15 years.” Had wrist synovectomy 2005 after “trauma.” • Saw rheumatologist 2012: ACPA>500, RF 60. • Loss of shoulder motion in all planes. • At the conclusion of this presentation, the participant should be able to: – Appreciate the relationship of Palindromic Rheumatism (PR) and progression to RA – Understand the biology of intercritical PR – Define the utility of prevention strategies – Comprehend the yield of imaging in PR and how it informs PR pathophysiology • Should we try to prevent? How? Annual transition to RA is greater than 15% in which of the following ACPA+ pts? A. Arthralgia B. Arthralgia + Imaging + CRP C. Palindromic Rheumatism D. Asymptomatic Twin E. Interstitial Lung Disease Rheumatoid Arthritis Pathogenesis Tolerance broken-AutoAb appear Adaptive Immune Response Locus and Trigger? Systemic Nature? “Amplification” Synovial Targeting with variable kinetics? Innate vs Adaptive Immunity? Joint Targeting ACPA-IC deposit or are formed de novo in joint? Or something else? Tissue Injury Rheumatoid Arthritis Persistence of the Systemic Trigger? Systemic autoimmunity & inflammation T cells/B Cells Immune Complexes TNF, IL-6, GM-CSF No treatment shown to eliminate systemic process Where does MTX work? Joint Inflammation MF, FLS, Cartilage, Bone RA Centers in Synovium, Destroying All Around It? Why Is Palindromic Rheumatism Palindromic? Systemic inflammation Followed by resolution? e.g. like gout? Why does it resolve? Why does it stop resolving? Single Joint Inflammation Palindromic Rheumatism (PR) • How frequent is PR as an initial presentation of RA? • What is the mechanism of PR? • Is synovitis present during its intercritical phase? • What is the frequency of progression to RA in 5 years? Treatment? Is Palindromic Rheumatism a Common Presentation? Frequency relative to new onset RA is: A.