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Turk Neurosurg 2018 Jun 13. Epub 2018 Jun 13.

the Affiliated Hospital of Southwest Medical University,Luzhou,Sichuan,China.

Objective This study aimed to investigate the clinical manifestations and radiologic characteristics in diagnosing and treating hemorrhagic cerebral paragonimiasis. Methods The study retrospectively analyzed the data of three cases of hemorrhagic paragonimiasis who received treatment in the hospital from January 2014 to March 2017. All three patients were diagnosed with paragonimiasis by positive detection of paragonimiasis antibody. Read More

Front Immunol 2019 29;10:665. Epub 2019 Mar 29.

Department of Ophthalmology, The Second Hospital of Jilin University, Changchun, China.

Behcet's disease (BD) is a chronic systemic inflammatory vasculitis of unknown etiology characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, and other manifestations. Although the pathogenesis of BD is unclear, some studies have shown that immunological aberrations play an important role in the development and progression of BD. Infection-related trigger factors, including antigens and autoantigens, are believed to mediate the development of BD in patients with a genetic predisposition and subsequently activate the innate and adaptive immune systems, resulting in the production of numerous cytokines and chemokines to combat the infection-related factors. Read More

Int J Rheum Dis 2019 Apr 8. Epub 2019 Apr 8.

Department of Nephrology and Rheumatology, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Aim: Since there are only a few reports on pediatric systemic lupus erythematosus (pSLE) in Chinese populations, therefore we retrospectively report the clinical and immunological features as well as renal outcome in Chinese pSLE.

Methods: Patients diagnosed with pSLE at Shanghai Children's Medical Center between 2001 and 2016 were evaluated and clinical data were retrospectively collected.

Results: A total of 102 pSLE patients were analyzed. Read More

Semin Arthritis Rheum 2019 Mar 9. Epub 2019 Mar 9.

DOIM-Rheum, MSC 10 5550, University of New Mexico Health Sciences Center, 5th FL ACC, Albuquerque, NM 87131, United States.

Objective: The goal of this study was to determine the characteristics of Behçet's disease (BD) in the American Southwest.

Material And Methods: This was a cross-sectional study of BD patients clinically encountered during a 2-year period. All subjects fulfilled the International Study Group criteria (ISG) or International Criteria for Behcet's Disease (ICBD). Read More

BMJ Open 2019 Mar 30;9(3):e021458. Epub 2019 Mar 30.

Department of Prosthodontics and Implantology, King Faisal University, Al-Hasa, Saudi Arabia.

Objective: Gastro-oesophageal reflux disease (GORD) is a relatively common disorder and manifests with extraoesophageal symptoms, such as dental erosions (DE), cough, laryngitis, asthma, and oral soft- and hard-tissue pathologies. This study aimed (1) to identify oral soft and hard-tissue changes in patients with GORD and (2) to evaluate these oral changes as indices for assessing GORD and its severity.

Setting: This cross-sectional study was conducted at four major tertiary care government hospitals, in two metropolitan cities of Pakistan. Read More

Pediatr Dermatol 2019 Mar 28. Epub 2019 Mar 28.

Dermatology Service, KK Women's and Children's Hospital, Singapore, Singapore.

Background/objectives: Drug reaction with eosinophilia and systemic symptoms (DRESS) is rare but potentially fatal in children. Fever and rash, which are salient features of DRESS, may mimic other commonly encountered pediatric conditions. We profiled the DRESS cases in a tertiary children's hospital in Singapore. Read More

Clin Mol Allergy 2019 12;17. Epub 2019 Mar 12.

2Immunoallergology Unit, Department of Biomedicine, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy.

Background: Atopic dermatitis is a chronic inflammatory skin disorder, whose symptoms and severity grossly depend on individual trigger factors. The majority of patients are satisfactorily treated with emollients together with topical and systemic therapies. However, treatment failure or long-term side effects with conventional treatment options can be a significant clinical problem. Read More

J Gen Intern Med 2019 Mar 18. Epub 2019 Mar 18.

Stanford Center for Undiagnosed Diseases, Stanford University School of Medicine, Stanford, CA, USA.

We discuss a challenging case of a 58-year-old Vietnamese-American woman who presented to her new primary care provider with an 8-year history of slowly progressive dysphagia, hoarseness, muscle weakness with associated frequent falls, and weight loss. She eventually reported dry eyes and dry mouth, and she was diagnosed with Sjogren's syndrome. Subsequently, she was additionally diagnosed with inclusion body myositis and gastric light-chain (AL) amyloidosis. Read More

J Oral Pathol Med 2019 Mar 12. Epub 2019 Mar 12.

Department of Oral Medicine, School of Dental Sciences, University of Newcastle upon Tyne, Newcastle upon Tyne, UK.

Background: Despite the frequency of oral involvement, there are unexpectedly few studies of either on the oral manifestations of pemphigus or their long-term management, and diagnostic delay in Dentistry is frequent.

Methods: We have examined outcome of patients presenting with predominantly oral pemphigus vulgaris (PV). Ninety-eight subjects were followed up for 85. Read More

Reumatologia 2019 28;57(1):37-44. Epub 2019 Feb 28.

Department of Otolaryngology, Maria Skłodowska-Curie District Hospital in Skarżysko-Kamienna, Poland.

Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands and a range of extra-glandular features. The most common and earliest symptoms are oral and ocular dryness. The aim of this study is to present the most common otolaryngological manifestations of SS, their pathomechanism and possible aetiology. Read More

Int J Environ Res Public Health 2019 Mar 9;16(5). Epub 2019 Mar 9.

Department of Oral Surgery and Dental Emergency Care, Faculty of Health, Witten/Herdecke University, Alfred-Herrhausen-Strasse 45, 58455 Witten, Germany.

: The object of this paper was to provide an overview of rare diseases (RDs) with periodontal manifestations and allocate them to relevant categories. : In ROMSE, a database for "Rare Diseases with Orofacial Involvement", all 541 entities were analyzed with respect to manifestations of periodontal relevance. Inclusion criteria were periodontally relevant changes to the oral cavity, in accordance with the 2018 version of the Classification of Periodontal and Peri-Implant Diseases and Conditions. Read More

Swiss Med Wkly 2019 02 10;149:w20022. Epub 2019 Mar 10.

Division of Rheumatology and Immunology, Department of Internal Medicine, Kantonsspital St. Gallen, St. Gallen, Switzerland / Division of Rheumatology and Clinical Immunology, Department of Internal Medicine IV, Ludwig Maximilian University of Munich, Munich, Germany.

Aims Of The Study: To describe patterns of systemic lupus erythematosus (SLE) care and the clinical effectiveness of belimumab plus standard of care therapy in a real-world clinical setting in Switzerland.

Methods: This multicentre, observational, retrospective cohort study included adults with SLE who initiated belimumab as part of their usual care at least six months before data analysis. The primary outcome was the overall clinical response, assessed by a physician on a Physician’s Global Assessment-like scale, to six months’ treatment with belimumab. Read More

Can J Ophthalmol 2019 Feb 21;54(1):33-39. Epub 2018 May 21.

Department of corneal and refractive surgery, Narayana Nethralaya, Bangalore.. Electronic address:

Objective: To present clinical manifestations of bilateral acute depigmentation of Iris (BADI) and bilateral acute iris transillumination (BAIT) and discuss its pathogenesis.

Design: Retrospective descriptive case study.

Participants: Twenty-two patients diagnosed with BADI or BAIT. Read More

J Fr Ophtalmol 2019 Apr 5;42(4):e133-e146. Epub 2019 Mar 5.

Service d'ophtalmologie, hôpital universitaire Fattouma Bourguiba, faculté de médecine, université de Monastir, 5019 Monastir, Tunisie. Electronic address:

Behçet's disease is a chronic multisystem disorder characterized by relapsing inflammation for which the underlying histopathology is an occlusive vasculitis. The disease has a high prevalence in the Mediterranean region, Far and Middle Eastern countries. It is clinically characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular involvement and numerous other systemic manifestations. Read More

Clin Epigenetics 2019 Mar 4;11(1):39. Epub 2019 Mar 4.

Centre for Research and Development of Medical Diagnostic Laboratories, Faculty of Associated Medical Sciences, Khon Kaen University, Khon Kaen, 40002, Thailand.

Background: Cholangiocarcinoma (CCA) is a fatal cancer of the bile duct epithelial cell lining. The misdiagnosis of CCA and other biliary diseases may occur due to the similarity of clinical manifestations and blood tests resulting in inappropriate or delayed treatment. Thus, an accurate and less-invasive method for differentiating CCA from other biliary diseases is inevitable. Read More

Med Oral Patol Oral Cir Bucal 2019 Mar 1;24(2):e217-e230. Epub 2019 Mar 1.

Charité - Universitaetsmedizin Berlin, Charité Centre 3 for Dental, Oral, and Maxillary Medicine, Department for Oral Medicine Dental Radiology, and Oral Surgery, Assmannshauser Str. 4-6, 14197 Berlin, Germany,

Background: To give an overview on implant survival rates in patients with oral manifestations of systemic autoimmune (oral Lichen planus (oLp), Pemphigus (Pe)), muco-cutaneous (Epidermolysis bullosa (EB)), autoimmune multisystemic rheumatic diseases (Sjögren's syndrome (SjS), systemic Lupus erythematosus (sLE), or systemic Sclerosis (sSc)).

Material And Methods: Systematic literature review (PubMed/Medline, Embase) using MESH and search term combinations, published between 1980 and August 2018 in English language reporting on dental implant-prosthetic rehabilitation of patients with oLp, Pe, EB, SjS, sLE, sSc, study design, age, gender, follow-up period (≥ 12 months), implant survival rate. Implant-related weighed mean values of implant survival rate (wmSR) were calculated. Read More

Medicine (Baltimore) 2019 Mar;98(9):e14668

Department of Ophthalmology & Visual Science, Tokyo Medical and Dental University Graduate School of Medicine.

Noninfectious uveitis (NIU), which pathogenesis is often autoimmune nature, occurs as a symptom of systemic syndromes or only in the eye. The standard treatment of NIU is local, topical, and oral administration of corticosteroids (CS) in combination with immunomodulatory therapy (IMT). However, additional therapeutic strategies involving topical and systemic administration of CS or others to treat relapse or exacerbation of ocular inflammation in NIU which present as various ocular manifestations have not been established. Read More

J Oral Maxillofac Surg 2019 Jan 28. Epub 2019 Jan 28.

Assistant Professor and Associate Program Director, Department of Oral and Maxillofacial Surgery, University of Rochester Medical Center, Eastman Institute for Oral Health, Rochester, NY.

Scleroderma and more generally systemic sclerosis comprise a group of autoimmune diseases characterized by increased collagen synthesis and deposition within connective tissue. The Scleroderma Foundation estimates that as many as 300,000 persons in the United States have scleroderma. Although the etiology of the disease is unknown, it is thought to be related to chronic inflammation secondary to autoimmune tissue damage. Read More

Int J Toxicol 2019 Mar/Apr;38(2):96-109. Epub 2019 Feb 21.

1 Landos Biopharma Inc, Blacksburg, VA, USA.

BT-11 is an orally active, gut-restricted investigational therapeutic targeting the lanthionine synthetase C-like 2 pathway with lead indications in ulcerative colitis (UC) and Crohn disease (CD), 2 manifestations of inflammatory bowel disease (IBD). In 5 mouse models of IBD, BT-11 is effective at oral doses of 8 mg/kg. BT-11 was also efficacious at nanomolar concentrations in primary human samples from patients with UC and CD. Read More

Ann Nutr Metab 2018 19;73 Suppl 4:18-28. Epub 2019 Feb 19.

Division on Gastroenterology, Hepatology and Nutrition, First Department of Pediatrics, University of Athens, Children's Hospital "Agia Sofia", Athens, Greece,

Eosinophilic gastrointestinal diseases (EGIDs) comprise a group of chronic, inflammatory diseases of the gastrointestinal (GI) tract, that are characterized, clinically, by symptoms related to the dysfunction of the involved segment(s) of the GI tract, and histologically, by dense eosinophilic inflammation, in the absence of an identifiable secondary cause. The group of EGIDs comprises eosinophilic esophagitis (EoE), eosinophilic gastritis (EG), eosinophilic gastroenteritis (EGE), and eosinophilic colitis (EC). EoE is the most common and the best described EGID compared to EG, EGE, and EC. Read More

J Oral Maxillofac Surg 2019 Jan 23. Epub 2019 Jan 23.

Associate Professor, Department of Pediatric Rheumatology, Children's of Alabama, Birmingham, AL.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that commonly affects the lungs, lymph nodes, and skin. The disease often presents in patients between the third and sixth decade and its pathology is defined by the presence of noncaseating granulomas within organs throughout the body. Oral and neurologic involvement of sarcoid is extremely rare and occurs in approximately 1% and 5% of patients with the disease, respectively. Read More

Quintessence Int 2019 ;50(3):208-213

Acute myeloid leukemia is a bone marrow malignancy in which blasts count increases by more than 20% in the bone marrow. Allogeneic hematopoietic stem cell transplantation (alloHCT) is a treatment option for these patients with high risk of graft versus host disease (GVHD) development. Chronic GVHD (cGVHD) often mimics a variety of autoimmune conditions such as systemic lupus erythematous or systemic sclerosis. Read More

Rheumatology (Oxford) 2019 Feb 15. Epub 2019 Feb 15.

National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre, University of Birmingham, Birmingham, UK.

Health-related quality of life (HRQoL) has an increasing role in medical decision-making. This review of the literature aims to provide an overview on HRQoL, costs, and work disability in SS, a disease characterized by focal lymphocytic infiltration of exocrine glands with no therapeutics of proven immunomodulatory potential. HRQoL is markedly reduced in SS in multiple studies across many countries when compared with HRQoL in healthy controls. Read More

Rheumatology (Oxford) 2019 Jan 30. Epub 2019 Jan 30.

Infection, Inflammation and Rheumatology Section, UCL Great Ormond Street Institute of Child Health, London, UK.

Objectives: Monogenic autoinflammatory disorders (AID) and primary immunodeficiencies can present early in life with features that may be mistaken for Behçet's disease (BD). We aimed to retrospectively describe the clinical and laboratory features of 11 paediatric cases referred for suspected BD who turned out to have an alternative, monogenic disease mimicking BD.

Methods: Retrospective, paediatric BD specialist multicentre case series. Read More

Dermatol Online J 2019 Jan 15;25(1). Epub 2019 Jan 15.

McGovern Medical School of University of Texas Houston Health Science Center, Houston, Texas.

The possible presentations of cytomegalovirus (CMV) are vast not only in its systemic manifestations, but also in the various cutaneous lesions that may result. Cutaneous cytomegalovirus is rarely reported in the literature because the clinical and pathologic features can be difficult to identify. Its identification, however, is vital as cutaneous human CMV infection can signal systemic disease and an unfavorable prognosis. Read More

Head Neck Pathol 2019 Mar 31;13(1):33-46. Epub 2019 Jan 31.

Virginia Commonwealth University School of Dentistry, Richmond, VA, USA.

When faced with an uncertain clinical pathosis in the oral cavity, identifying the color of the mucosal lesion helps to narrow down a differential diagnosis. Although less common than red and white lesions, yellow lesions encompass a small group of distinct mucosal pathologic entities. Adipose tissue, lymphoid tissue, and sebaceous glands are naturally occurring yellow constituents of the oral cavity and become apparent with associated developmental or neoplastic lesions. Read More

J Indian Soc Periodontol 2019 Jan-Feb;23(1):12-20

Department of Periodontics, A B Shetty Memorial Institute of Dental Sciences, Nitte University, Mangalore, Karnataka, India.

Background: Micronutrients play an important part in metabolism and maintenance of tissue function the maintenance of the integrity and vitality of the periodontal tissues depends on the availability of adequate nutrients, and it is possible that deficiencies can produce pathological alterations in the periodontal tissues. However, the association between nutritional factors and periodontal health is not fully understood. The essential micronutrients for maintaining health include zinc, copper, selenium, and iron. Read More

BMJ Case Rep 2019 Jan 22;12(1). Epub 2019 Jan 22.

Oral Health Sciences Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Idiopathic gingival fibromatosis (GF), also known as gingivomatosis, is a rare condition in childhood, with an unknown aetiology. The oral manifestations of the condition are varied and depend on the severity and age of involvement. This paper describe the case of a 5-year-old male child with extensive gingival enlargement covering almost all the maxillary and mandibular teeth resulted in difficulty with speech, mastication and poor aesthetics. Read More

J Immunol Res 2018 20;2018:1246818. Epub 2018 Dec 20.

Department of Oral Biology, School of Dental Medicine, State University of New York at Buffalo, Buffalo, NY 14214, USA.

While the importance of Toll-like receptor (TLR) signaling is well established in many autoimmune diseases, the role of TLR activation in Sjögren's syndrome (SS) is poorly understood. Studies in mice and humans reveal that TLRs are potent mediators of inflammation in SS. TLRs are expressed and functional in salivary tissue, and TLRs in peripheral blood cells of SS patients are also upregulated and hyperresponsive to ligation. Read More

Ned Tijdschr Tandheelkd 2019 01;126(1):23-28

Dental professionals will be confronted more frequently with medically compromised patients due to an increasing life expectancy in Western countries. The patients' systemic diseases and medication usage can both have consequences for oral health and dental treatment. It is often impossible to have all medical-dental interactions handy in the dental practice. Read More

J Indian Soc Periodontol 2018 Nov-Dec;22(6):551-554

Department of Periodontology, Mahatma Gandhi Postgraduate Institute of Dental Sciences, Puducherry, India.

Pemphigus is an autoimmune disease affecting the skin and mucosae. Oral lesions are common and sometimes are the only manifestations of the disease. The clinical presentations of pemphigus might mimic other vesiculobullous lesions of the oral cavity. Read More

Curr Opin Rheumatol 2019 Mar;31(2):201-207

Department of Immunobiology, Yale University School of Medicine, New Haven, Connecticut, USA.

Purpose Of Review: The resident bacterial communities and the host immune system have coevolved for millennia. However, recent changes in modern societies have disrupted this coevolutionary homeostasis and contributed to a rise in immune-mediated conditions. The purpose of this review is to provide an overview of recently elucidated mechanisms of how certain taxa within the bacterial microbiome propagate autoimmunity. Read More

J Drugs Dermatol 2018 12;17(12):1328-1329

Behçet’s syndrome is an inflammatory disease characterized by aphthous oral ulcers and several systemic manifestations, which include genital ulcers, ocular disease, skin lesions, arthritis, and vascular disease Read More

J Clin Med 2018 Dec 25;8(1). Epub 2018 Dec 25.

Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Internal Medicine, IRCCS Fondazione Ca' Granda, Ospedale Maggiore Policlinico, 20122 Milano, Italy.

Oral manifestations are frequent in patients with rheumatic diseases. The aim of this review is to offer readers practical advice concerning the onset, diagnosis and treatment of the main oral manifestations encountered in rheumatological and dental clinics. Signs and symptoms such as oral hyposalivation, xerostomia, temporomandibular joint disorders, periodontal disease, and dysphagia may be the first expression of a number of rheumatic diseases. Read More

Clin Exp Rheumatol 2018 Nov-Dec;36(6 Suppl 115):110-115. Epub 2018 Dec 12.

Research Centre of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Centre, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy.

Objectives: Behçet's disease (BD) is an autoinflammatory disorders mainly characterised by recurrent oral aphthosis, genital ulcers, and uveitis. The involvement of immunoglobulin D (IgD) in BD physiopathology is still unclear. The aim of our study was to assess the role of IgD in BD by comparing circulating levels of IgD in a cohort of BD patients and healthy controls (HC), as well as by correlating IgD levels with BD activity and different clinical presentations. Read More

Med Oral Patol Oral Cir Bucal 2019 Jan 1;24(1):e1-e7. Epub 2019 Jan 1.

Bellvitge University Campus, Department of Dental Medicine - School of Dentistry, Pabellón de Gobierno, C/. Feixa LLarga s/n, 08907 L'Hospitalet de Llobregat, Barcelona - Spain,

Background: Lichen planus (LP) is a chronic autoimmune disease that affects the oral mucosa as well as the skin, genital mucosa and other sites.

Objective: to evaluate the correlation between oral, genital and cutaneous lichen planus, in a sample of LP patients.

Material And Methods: This descriptive study reviewed 274 clinical histories of patients, who all presented histological confirmation of lichen planus verified by a pathologist, attending research centers in Barcelona. Read More

J Rheumatol 2018 Dec 15. Epub 2018 Dec 15.

From the Division of Rheumatology, Department of Medicine, Toronto Western Hospital, University of Toronto, Toronto; Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, Toronto; University Health Network, and Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada; Division of Rheumatology, Department of Medicine, University of Michigan, Ann Arbor, Michigan; University of California, Los Angeles, Los Angeles; University of California, San Francisco, California; Brigham and Women's Hospital, Boston; Harvard Medical School, Boston, Massachusetts; Medical University of South Carolina, Charleston, South Carolina; Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA; Hospital Clínic, Barcelona, Spain; AP-HP, Cochin Hospital, Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares d'île de France, Paris; Université Paris Descartes-Sorbonne Paris Cité, Paris; INSERM U 1153, Center for Epidemiology and Statistics Sorbonne Paris Cité (CRESS), Paris, France; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin; Berlin Institute of Health, Department of Rheumatology and Clinical Immunology, Berlin; University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany; Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico; National and Kapodestrian University of Athens, and Biomedical Research Foundation of the Athens Academy, Athens, Greece; Department of Medicine, University of Cambridge, Cambridge, UK; University of Pisa, Pisa, Italy; Medical University of Vienna, Vienna, Austria. This study was jointly supported by the European League Against Rheumatism and the American College of Rheumatology. Dr. Johnson is supported by a Canadian Institutes of Health Research New Investigator Award. Dr. Khanna was supported by a grant from the US National Institutes of Health/ National Institute of Arthritis and Musculoskeletal and Skin Diseases K24 AR 063120. S.R. Johnson, MD, PhD, FRCPC, Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Mount Sinai Hospital, and Institute of Health Policy, Management and Evaluation, University of Toronto; D. Khanna, MD, MS, Division of Rheumatology, Department of Medicine, University of Michigan; R. Cervera, MD, PhD, FRCP, Hospital Clínic, Barcelona; N. Costedoat-Chalumeau, MD, PhD, AP-HP, Cochin Hospital, Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares d'île de France, and Université Paris Descartes-Sorbonne Paris Cité, and INSERM U 1153, CRESS; D.D. Gladman, MD, FRCPC, Division of Rheumatology, Department of Medicine, Toronto Western Hospital, University of Toronto; B.H. Hahn, MD, University of California, Los Angeles; F. Hiepe, MD, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Rheumatology and Clinical Immunology; J. Sánchez-Guerrero, MD, MSc, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital/University Health Network, University of Toronto, and Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán; E. Massarotti, MD, Brigham and Women's Hospital, and Harvard Medical School; D.T. Boumpas, MD, FACP, FACR, National and Kapodestrian University of Athens, and Biomedical Research Foundation of the Athens Academy; K.H. Costenbader, MD, MPH, Brigham and Women's Hospital, and Harvard Medical School; D. Daikh, MD, University of California, San Francisco; D. Jayne, MD FRCP, FRCPE, FMedSci, Department of Medicine, University of Cambridge; T. Dörner, MD, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Rheumatology and Clinical Immunology; D.L. Kamen, MD, MSCR, Medical University of South Carolina; M. Mosca, MD, PhD, University of Pisa; R. Ramsey-Goldman, MD, DrPH, Northwestern University Feinberg School of Medicine; J.S. Smolen, MD, Medical University of Vienna; D. Wofsy, MD, University of California, San Francisco; M. Aringer, MD, University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden. Address correspondence to Dr. S.R. Johnson, Division of Rheumatology, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst St., Toronto, Ontario M5T 2S8, Canada. E-mail: Accepted for publication November 30, 2018.

Objective: Given the complexity and heterogeneity of systemic lupus erythematosus (SLE), high-performing classification criteria are critical to advancing research and clinical care. A collaborative effort by the European League Against Rheumatism and the American College of Rheumatology was undertaken to generate candidate criteria, and then to reduce them to a smaller set. The objective of the current study was to select a set of criteria that maximizes the likelihood of accurate classification of SLE, particularly early disease. Read More

Lupus 2019 Jan 6;28(1):104-113. Epub 2018 Dec 6.

1 Rheumatology, Clinical Immunology and Allergy, University of Crete School of Medicine, Iraklio, Greece.

Background: Examining urban-rural differences can provide insights into susceptibility or modifying factors of complex diseases, yet limited data exist on systemic lupus erythematosus (SLE).

Objective: To study SLE risk, manifestations and severity in relation to urban versus rural residence.

Methodology: Cross-sectional analysis of the Crete Lupus Registry. Read More

Arq Gastroenterol 2018 Jul-Sep;55(3):290-295

Universidade do Estado da Bahia, Departamento de Ciências da Vida, Salvador, BA, Brasil.

Background: The diagnosis of inflammatory bowel disease is often delayed because of the lack of an ability to recognize its major clinical manifestations.

Objective: Our study aimed to describe the onset of clinical manifestations in inflammatory bowel disease patients.

Methods: A cross-sectional study. Read More

Case Rep Dermatol 2018 Sep-Dec;10(3):226-230. Epub 2018 Oct 18.

Department of Dermatology, Nara Medical University School of Medicine, Kashihara, Japan.

We report the case of a 45-year-old female with generalized morphea (GM), who exhibited positivity for the anti-centromere antibody (Ab). She frequently developed multiple sclerotic skin lesions, whose histological findings were compatible with morphea. She demonstrated favorable responses to topical and oral steroids. Read More

Pediatr Dermatol 2019 Jan 26;36(1):e27-e30. Epub 2018 Nov 26.

Department of Oral Surgery and Pathology, Faculty of Dentistry, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.

Drug-induced reactions are complications associated with high mortality and significant morbidity. Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are examples of these conditions, which are characterized by skin and mucous lesions. Here, we report a case of a 9-year-old girl who presented with blisters associated with an extensive vesicular rash and multiple ulcerations on the lips and oral cavity. Read More

Joint Bone Spine 2018 Nov 22. Epub 2018 Nov 22.

Centre national de référence des maladies auto-inflammatoires et de l'amylose inflammatoire (CEREMAIA), 94270 Le Kremlin-Bicêtre, France; Cellules souches, plasticité cellulaire, médecine régénératrice et immunothérapies, Inserm, Université de Montpellier, 34090 Montpellier, France; Département de génétique médicale, maladies rares et médecine personnalisée, CHU de Montpellier, 34295 Montpellier, France.

The spectrum of factors known to mediate autoinflammation has broadened recently to include not only interleukin-1 (IL-1) and interferon, but also abnormalities that impair NF-κB pathway negative regulation. The NF-κB pathway is activated upon contact of a ligand with tumor necrosis factor receptor 1 (TNFR1) and plays a pivotal role in triggering the inflammatory process by producing major cytokines such as IL-1, IL-6, and TNF. Negative regulation of the NF-κB pathway, which is essential to stop the inflammatory process, depends on the level of ubiquitination of the proteins associated with TNFR1 and of other intermediate compounds. Read More

Ther Umsch 2018 Nov;75(4):235-239

1 Internistischer Dienst, Psychiatrische Universitätsklinik Zürich.

Hemochromatosis related Arthropathy Abstract. Hemochromatosis is an autosomal recessive disease that is caused by an HFE gene mutation (High Iron Fe) in most patients. Pathophysiologically, the effect of the mutation is an increased iron absorption in the gut. Read More

J Craniofac Surg 2018 Nov 15. Epub 2018 Nov 15.

Department of Oral and Maxillofacial Surgery II, Osaka University Graduate School of Dentistry, Osaka, Japan.

Gingival enlargement, although frequently encountered in clinical settings, is rarely associated with systemic diseases or syndromes. Among the diverse pathological conditions of neurofibromatosis type 1 (NF-1), minor manifestations in the orofacial region are occasionally overlooked. Herein, the authors present an unusual case of gingival neurofibroma in a patient with NF-1 associated with characteristic osseous defects in the alveolus in the long-term course of 17 years from the first examination. Read More

J Prosthodont 2018 Oct 25. Epub 2018 Oct 25.

Oral Medicine, Diagnosis, Radiology, and Periodontology Department, Faculty of Dentistry, Tanta University, Tanta, Egypt.

Purpose: To evaluate marginal bone loss (MBL) of dental implants inserted in active lichen planus patients.

Materials And Methods: The study included 59 subjects divided into 3 groups depending on their lichen planus diagnosis and administration of a low dose of corticosteroids: 17 healthy individuals, 20 controlled lichen planus patients controlled using low doses of systemic corticosteroids, and 22 noncontrolled lichen planus patients. During 4-year follow-up sessions MBL was evaluated, and biopsies were collected from lichen planus patients and examined. Read More

J Dent Child (Chic) 2018 May;85(2):75-78

ALYN Hospital Pediatric and Adolescent Rehabilitation Center, in Jerusalem, Israel.

Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). LCH may manifest orally with single or multiple lesions of the alveolar or basal bone. Ulcerated mucosal lesions may be accompanied by periodontal lesions that present with gingival inflammation, bleeding, recession, necrosis, toothache, dental hypermobility, tooth development abnormalities, and premature tooth loss. Read More

Dtsch Arztebl Int 2018 Sep;155(37):599-605

Institute and Outpatient Clinic for Occupational, Social and Environmental Medicine, Comprehensive Pneumology Center Munich, German Center for Lung Research (DZL), Munich, Germany; Department of Medicine, Pulmonary, Critical Care and Sleep Medicine, University Hospitals of Gießen and Marburg, Marburg site, German Center for Lung Research (DZL); Department of Respiratory Medicine, Allergology and Sleep Medicine, General Hospital Nürnberg, Paracelsus Medical University, Nürnberg, Germany.

Background: Chronic obstructive pulmonary disease (COPD) is common around the world and carries a high morbidity and mortality. Symptom- and risk-oriented drug treatment is recommended, both in Germany and in other countries. It is not yet known to what extent the treatment that is actually delivered in Germany corresponds to the current recommendations in the guidelines. Read More

Acta Dermatovenerol Alp Pannonica Adriat 2018 Sep;27(3):153-154

Department of Dermatology, Şırnak State Hospital, Şırnak, Turkey.

Behçet's disease is a rare disorder of unknown etiology that is classified as a systemic vasculitis. The prevalence of the disease is high in countries in the Far East, Mediterranean Basin, and East Asia. Thus, it is also known as the Silk Road Disease. Read More

J Gastrointestin Liver Dis 2018 Sep;27(3):331-336

Gastroenterology and Digestive Endoscopy, 'Nuovo Regina Margherita' Hospital, Rome, Italy.

Whipple's disease (WD) is known as an infrequent, systemic, chronic infection caused by the actinomycete Tropherima whipplei (T. whipplei). The disease is frequently characterized by a long prodromal and protean extra-intestinal phase, which often causes misdiagnosis and inappropriate treatments. Read More