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    1462 results match your criteria Oral Manifestations of Systemic Diseases

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    Systemic mastocytosis with KIT V560G mutation presenting as recurrent episodes of vascular collapse: response to disodium cromoglycate and disease outcome.
    Allergy Asthma Clin Immunol 2017 24;13:21. Epub 2017 Apr 24.
    Consulta Multidisciplinar de Linfomas Cutâneos e Mastocitoses (CMLC), Hospital de Santo António (HSA), Centro Hospitalar do Porto (CHP), Porto, Portugal.
    Background: Mastocytosis are rare diseases characterized by an accumulation of clonal mast cells (MCs) in one or multiple organs or tissues. Patients with systemic mastocytosis (SM), whose MCs frequently arbor the activating D816V KIT mutation, may have indolent to aggressive diseases, and they may experience MC mediator related symptoms. Indolent SM with recurrent anaphylaxis or vascular collapse in the absence of skin lesions, ISMs(-), is a specific subtype indolent SM (ISM), and this clonal MC activation disorder represents a significant fraction of all MC activation syndromes. Read More

    Innate immunity in Sjögren's syndrome.
    Clin Immunol 2017 Apr 8. Epub 2017 Apr 8.
    Department of Oral Biology, School of Dental Medicine, State University of New York at Buffalo, Buffalo, NY 14214, United States; Department of Oral Diagnostic Sciences, School of Dental Medicine, State University of New York at Buffalo, Buffalo, NY 14214, United States. Electronic address:
    Sjögren's syndrome (SS) is an autoimmune disease of exocrine tissue that primarily affects women. Although patients typically experience xerostomia and xerophthalmia, numerous systemic disease manifestations are seen. Innate immune hyperactivity is integral to many autoimmune diseases, including SS. Read More

    Oral ulcers associated with bone destruction as the primary manifestation of histoplasmosis in an immunocompetent patient.
    J Eur Acad Dermatol Venereol 2017 Apr 5. Epub 2017 Apr 5.
    Oral Oncology Center and Department of Pathology and Clinical Propedeutics, São Paulo State University (Unesp), Araçatuba Dental School, Araçatuba, São Paulo, Brazil.
    Histoplasmosis is a systemic fungal infection caused by Histoplasmacapsulatum, a saprophyte fungus commonly found in enriched soils with bat and bird droppings.[1] Oral manifestations of histoplasmosis are associated with the disseminated form of the disease, occurring in general associated with pulmonary infection.[2] We report a rare case of oral histoplasmosis with periodontal bone destruction in an immunocompetent patient without underlying pulmonary disease. Read More

    Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment.
    Int J Med Sci 2017 23;14(3):191-200. Epub 2017 Feb 23.
    Department of Internal Medicine, Division of Clinical Immunology;; Department of Immunology, Erasmus MC, Rotterdam, The Netherlands.
    Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx and/or vagina. Extraglandular manifestations may also be prevalent in patients with pSS, including cutaneous, musculoskeletal, pulmonary, renal, hematological and neurological involvement. Read More

    HIV Infection and Compromised Mucosal Immunity: Oral Manifestations and Systemic Inflammation.
    Front Immunol 2017 7;8:241. Epub 2017 Mar 7.
    Faculty of Medicine and Dentistry, Department of Dentistry, University of Alberta, Edmonton, AB, Canada; Faculty of Medicine and Dentistry, Department of Medical Microbiology and Immunology, University of Alberta, Edmonton, AB, Canada.
    Mucosal surfaces account for the vast majority of HIV transmission. In adults, HIV transmission occurs mainly by vaginal and rectal routes but rarely via oral route. By contrast, pediatric HIV infections could be as the result of oral route by breastfeeding. Read More

    Oral Viral Infections: Diagnosis and Management.
    Dent Clin North Am 2017 Apr;61(2):351-363
    Department of Oral and Maxillofacial Surgery, Woodhull Medical Center, 760 Broadway, Room 2C-320, Brooklyn, NY 11206, USA.
    This article focuses on common viral infections in the oral cavity with associated systemic manifestations. Discussed are the clinical features, histopathology, diagnosis, treatment, and prevention of viral infections in oral cavity. This will be a useful aid for general practitioners and other dental personnel wanting to expand their pathologic knowledge. Read More

    Treatment of Renal Angiomyolipoma and Other Hamartomas in Patients with Tuberous Sclerosis Complex.
    Clin J Am Soc Nephrol 2017 Mar 16. Epub 2017 Mar 16.
    Renal Disease and Hypertension, Pediatric Nephrology and Hypertension, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, Texas
    Tuberous sclerosis complex is an autosomal dominant genetic disease characterized by growth of benign tumors (hamartomas) in multiple organs, especially the kidneys, brain, heart, lungs, and skin. Tuberous sclerosis complex is usually caused by a mutation in either the tuberous sclerosis complex 1 or tuberous sclerosis complex 2 gene, resulting in constitutive activation of mammalian target of rapamycin signaling. Currently, mammalian target of rapamycin inhibitors are recommended in adult patients with tuberous sclerosis complex for the treatment of asymptomatic, growing renal angiomyolipoma that are >3 cm in diameter and pediatric or adult patients with brain lesions (subependymal giant cell astrocytoma) that either are growing or are not amenable to surgical resection. Read More

    An oral lesion as the primary clinical manifestation of sarcoidosis.
    Ann R Coll Surg Engl 2017 Mar 16:e1-e2. Epub 2017 Mar 16.
    Mid Essex Hospital Services NHS Trust , UK.
    An oral lesion as the first clinical presentation of sarcoidosis is extremely rare. We present the case of a 39-year-old woman who was referred to the oral and maxillofacial surgery department with a persistent asymptomatic nodular lesion in the hard palate. This was located adjacent to a grossly carious upper first molar and a provisional diagnosis of chronic periapical granuloma was made. Read More

    Amyloidosis and Anesthesia.
    Anesth Essays Res 2017 Jan-Mar;11(1):233-237
    Department of Anaesthesiology and Critical Care, NIMS Medical College and Hospital, Jaipur, Rajasthan, India.
    Aim: The aim of this article is to provide a view of amyloidosis and discuss implications for the anesthetic management of patients with this condition.

    Material And Method: Urine samples from patients with plasma cell dyscrasias were obtained from a urine bank that gathers urine samples from patients who gave research use consent for specimens that would otherwise be considered waste.

    Results: Patients with amyloidosis may present to the anesthesiologist for procedures relating to diagnosis, surgery relating to the underlying condition (e. Read More

    Comparison of Disease Characteristics, Organ Damage, and Survival in Patients with Juvenile-onset and Adult-onset Systemic Lupus Erythematosus in a Combined Cohort from 2 Tertiary Centers in Turkey.
    J Rheumatol 2017 Mar 15. Epub 2017 Mar 15.
    From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yıldız Technical University; Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. B. Artim-Esen, MD, Associate Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; S. Şahin, MD, Fellow, Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; E. Çene, Research Assistant, Department of Statistics, Faculty of Arts and Sciences, Yıldız Technical University; Y. Şahinkaya*, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; K. Barut, MD, Fellow, Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; A. Adrovic, MD, Fellow, Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine; Y. Özlük, PhD, Associate professor, Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, I. Kılıçaslan, PhD, Professor, Department of Pathology, Istanbul Faculty of Medicine, Istanbul University; A. Omma**, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; A. Gül, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine; L. Öcal, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Ö. Kasapçopur, MD, Professor, Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; M. İnanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University. *Current position: Division of Rheumatology, Department of Internal Medicine, Marmara Faculty of Medicine, Marmara University, Istanbul, Turkey. **Current position: Department of Rheumatology, Ankara Numune Training and Research Hospital, Ankara, Turkey. Address correspondence to Dr. B. Artim-Esen, Department of Internal Medicine, Division of Rheumatology, Istanbul University, Capa, Fatih, Istanbul 34093, Turkey. E-mail: Accepted for publication January 6, 2017.
    Objective: Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE).

    Methods: For the study, 719 patients (76. Read More

    Severe Systemic Lead Toxicity Resulting From Extra-Articular Retained Shrapnel Presenting as Jaundice and Hepatitis: A Case Report and Review of the Literature.
    Mil Med 2017 Mar;182(3):e1843-e1848
    Department of Preventive Medicine and Public Health, Blanchfield Army Community Hospital, 2576 23rd Street, Fort Campbell, KY 42223.
    Introduction: Despite greater than 60,000 nonfatal firearm injuries per year in the United States, retained shrapnel is a relatively rare cause of systemic lead toxicity with less than 100 cases reported in the medical literature since 1867. While intra-articular retained shrapnel as a cause of lead toxicity is well-described, extra-articular fragments are less well known to cause symptomatic disease.

    Case Report: A 31-year-old man initially presented with abdominal pain, constipation, jaundice, and elevated liver transaminases approximately 3 weeks after suffering a left lower extremity injury during athletic activity. Read More

    Cytokine Signatures in Mucocutaneous and Ocular Behçet's Disease.
    Front Immunol 2017 27;8:200. Epub 2017 Feb 27.
    Department of Emergency and Organ Transplantation, Rheumatology Unit, University of Bari Aldo Moro , Bari , Italy.
    Behçet's disease (BD) is a multi-systemic inflammatory disorder consisting of recurrent oral aphthosis, genital ulcers, and chronic relapsing bilateral uveitis; however, many other organs may be affected. Several pro-inflammatory cytokines, mainly derived from Th1 and Th17 lymphocytes, seem to be involved in different pathogenic pathways leading to development of the clinical manifestations. On this basis, the primary aim of our study was to compare a core set of pro-inflammatory cytokines between patients with BD and healthy control (HC). Read More

    Oral Pyoderma Gangrenosum: Diagnosis, Treatment and Challenges: A Systematic Review.
    Head Neck Pathol 2017 Mar 8. Epub 2017 Mar 8.
    Oral and Maxillofacial Pathologist, Department of Stomatology, Faculty of Dentistry, Universite de Montreal, Montreal, QC, Canada.
    Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The condition is characterized by the appearance of one or more necrotic ulcers with a ragged undermined violaceous border and surrounding erythema. Lesions are often initiated by minor trauma. Read More

    A meta-analysis of avascular necrosis in systemic lupus erythematosus: prevalence and risk factors.
    Clin Exp Rheumatol 2017 Feb 24. Epub 2017 Feb 24.
    Division of Rheumatology, Department of Medicine, Joseph's Health Care, London, Canada.
    Objectives: To determine the prevalence of and risk factors for avascular necrosis (AVN) in systemic lupus erythematosus (SLE).

    Methods: MEDLINE, CINAHL, Web of Science, EMBASE and Cochrane Library were searched from inception to July, 2015 and a random effects model was used to combine frequencies; study quality was assessed using STROBE.

    Results: 2,041 citations identified 62 articles. Read More

    Contemporary Use of Corticosteroids in Rhinology.
    Curr Allergy Asthma Rep 2017 Feb;17(2):11
    Department of Otorhinolaryngology, School of Medicine, University of Crete, Heraklion, Crete, Greece.
    Purpose Of Review: Exogenously administered corticosteroids are widely used today in the field of rhinology. Allergic rhinitis (AR), non-allergic rhinitis (NAR), acute rhinosinusitis (ARS), chronic rhinosinusitis with (CRSwNP) and without (CRSsNP) nasal polyps, and autoimmune disorders with nasal manifestations are common diseases treated effectively with intranasal and oral glucocorticoids. We focus on physiological pathways, therapeutic benefits, indications, contra-indications, and side effects of glucocorticoid utilization in the treatment of rhinologic disorders such as AR, NAR, ARS, CRSsNP, and CRSwNP. Read More

    Tuberculosis 'The Great Imitator': A usual disease with unusual presentations.
    Indian J Tuberc 2017 Jan 4;64(1):54-59. Epub 2016 Jul 4.
    Lecturer, Dept. of Pathology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India.
    Background: A number of infectious diseases have been referred to by the phrase 'The Great Imitator', of which the oldest is syphilis; others include Lyme disease, nocardiosis, etc. Tuberculosis has been described as the second great imitator as it can imitate various other disease processes. An awareness of the atypical clinical manifestations of tuberculosis is important, especially in regions where tuberculosis continues to be a major public health problem, such as India. Read More

    Three Cases of Erdheim-Chester Disease With Intraocular Manifestations: Imaging and Histopathology Findings of a Rare Entity.
    Am J Ophthalmol 2017 Apr 30;176:141-147. Epub 2017 Jan 30.
    The Rotterdam Eye Hospital, Rotterdam, Netherlands.
    Purpose: To report intraocular manifestations of Erdheim-Chester Disease (ECD) with multimodal imaging.

    Design: A retrospective observational case series.

    Methods: This was a multicenter case series of 3 patients with confirmed tissue diagnosis of ECD that showed intraocular manifestations and were imaged at baseline and follow-up visits. Read More

    Autoimmune Disease Manifestations in the Oral Cavity.
    Surg Pathol Clin 2017 Mar 28;10(1):57-88. Epub 2016 Dec 28.
    Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida, 1395 Center Drive, Gainesville, FL 32610, USA.
    Immune-related disorders of the oral cavity may occur as primary disease process, secondary to systemic disease or neoplasm, or as a reaction to medications and other agents. The entities represented within this group may vary significantly by severity, clinical presentation, microscopic presentation, and special testing results. The selected immune-related conditions of the oral cavity in this article are categorized and presented by their prototypical tissue reaction patterns: vesiculobullous, including acantholytic and subepithelial separation; psoriasiform; spongiotic; and lichenoid reaction patterns. Read More

    Evaluation of olfactory function in Behçet's disease.
    Eur J Rheumatol 2016 Dec 1;3(4):153-156. Epub 2016 Dec 1.
    Division of Rheumatology, Department of Internal Medicine, Ondokuz Mayıs University School of Medicine, Samsun, Turkey.
    Objective: Behçet's disease (BD) is a chronic, relapsing type of vasculitis of unknown etiology and is characterized by oral and urogenital ulcers and ocular inflammation with cutaneous, musculoskeletal, vascular, and nervous system manifestations. Few cases involving the nasal mucosa have been reported in the literature, and the true prevalence of BD remains unknown. Neurological involvement associated with BD might play a more important role in causing olfactory dysfunction than mucosal involvement, but sufficient clinical data are not available on the effect of BD on olfaction in adults. Read More

    [Fabry disease].
    Ann Dermatol Venereol 2017 Feb 16;144(2):137-146. Epub 2017 Jan 16.
    Département de dermatologie, Hôpital Hôtel-Dieu de France, Faculté de médecine, Université Saint-Joseph, Beyrouth, Liban.
    Fabry disease, also known as Anderson-Fabry disease or angiokeratoma corporis diffusum universale, is an X-linked recessive form of sphingolipidosis caused by total or partial deficiency of the lysosomal hydrolase, alpha-galactosidase A. From the youngest age, it results in a gradual ubiquitous build-up of glycosphingolipids that are not degraded by the missing enzyme. Cutaneous, neurological, nephrologic, cardiac, gastrointestinal, ophthalmological, respiratory, cochleovestibular and haematological involvement are responsible for increased mortality and significant impairment of quality of life in subjects affected by the disease. Read More

    Mechanisms and New Strategies for Primary Sjögren's Syndrome.
    Annu Rev Med 2017 Jan;68:331-343
    Department of Physiology, School of Medicine and Joint Academic Rheumatology Program, National and Kapodistrian University of Athens, Athens, Greece; email:
    Primary Sjögren's syndrome (SS) is a common chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, mainly salivary and lacrimal, resulting in oral and ocular dryness, although virtually any organ system can be affected. SS-related systemic manifestations are classified as either related to the presence of periepithelial infiltrates in exocrine and parenchymal organs or resulting from immunocomplex deposition due to B cell hyperactivity with increased risk for B cell lymphoma development. Activation of both innate and adaptive immune pathways contributes to disease pathogenesis, with prominent interferon (IFN) signatures identified in both peripheral blood and affected salivary gland tissues. Read More

    Oral mucous membrane pemphigoid and pemphigus vulgaris-a retrospective two-center cohort study.
    Oral Dis 2017 May 22;23(4):498-504. Epub 2017 Feb 22.
    Division of Oral Medicine and Dentistry, Brigham and Women's Hospital, Boston, MA, USA.
    Objectives: Few studies have compared oral mucous membrane pemphigoid (MMP) and pemphigus vulgaris (PV). Descriptive analysis of oral features, extent of extra-oral involvement, and management outcomes were performed.

    Subjects And Methods: Patients with PV and MMP, the latter with exclusive oral involvement at first presentation, were included. Read More

    ANA-Negative Presentation of SLE in Man with Severe Autoimmune Neutropenia.
    Case Rep Med 2016 19;2016:6853936. Epub 2016 Dec 19.
    University of California San Diego School of Medicine, La Jolla, CA, USA.
    Background. Systemic lupus erythematosus (SLE) is a chronic, inflammatory, connective tissue disease that commonly affects the joints and a variety of organs due to an overactivation of the body's immune system. There is wide heterogeneity in presentation of SLE patients, including lung, central nervous system, skin, kidney, and hematologic manifestations. Read More

    Systemic alterations and their oral manifestations in pregnant women.
    J Obstet Gynaecol Res 2017 Jan;43(1):16-22
    Clinica Odontologica, Federal University of Maranhao, São Luís, Brazil.
    The aims of this literature review are: to depict the main oral diseases that are related to pregnancy; to clarify some of the possible systemic mechanisms that are associated with these changes; and to address issues about oral care during pregnancy. A woman's organs undergo various physiological, neurological, and hormonal changes during pregnancy. Such changes occur gradually and are essential for the development of the fetus, providing what is needed for tissue formation and establishment of reserves for uterine and fetal life. Read More

    Oral manifestations of gastrointestinal diseases in children. Part 4: Coeliac disease.
    Eur J Paediatr Dent 2016 Dec;17(4):332-334
    Department of Surgical sciences and Integrated Diagnostics, University of Genova, Genoa, Italy.
    Alterations within the oral cavity can be the first sign of systemic diseases and may thus allow for an early diagnosis and treatment. In particular, being the oral cavity part of the gastrointestinal system, oral alterations can be an expression of a gastrointestinal disease. Dental enamel hypoplasia and aphthous ulcers have been found to be more common in children with coeliac disease compared with the general population and to regress after the patient is started on a gluten free-diet. Read More

    Orofacial granulomatosis as early manifestation of Crohn's disease: report of a case in a paediatric patient.
    Eur J Paediatr Dent 2016 Dec;17(4):318-321
    1st Department of Pediatrics, Medical School, University of Athens, Aghia Sophia Children's Hospital, Athens, Greece.
    Background: Orofacial granulomatosis (OFG) is a controversial entity mainly characterised by recurrent or permanent soft tissue swelling of sudden onset in the orofacial area with a histologic appearance of granulomatous inflammation. Differential diagnosis includes local diseases and systemic conditions, such as Crohn's disease (CD). A case of OFG in a paediatric patient is reported here, focusing on the clinical features, diagnostic procedures, treatment and long-term outcome. Read More

    Steinert syndrome and repercussions in dental medicine.
    Arch Oral Biol 2017 Mar 23;75:37-47. Epub 2016 Dec 23.
    Health Sciences Faculty, Fernando Pessoa University, Porto, Portugal. Electronic address:
    Steinert syndrome, also called myotonic dystrophy type 1, is a genetic disorder with autosomal dominant transmission characterized by myotonia and a multisystemic clinical picture that affects several tissues of the human body. The most common systemic phenotypes are: muscular, cardiac, respiratory, CNS, ocular, gynecological, digestive, orthopedical, as well as cognitive and psychological symptoms (cognitive decline). Muscles involved in voluntary movement are highly affected by myotonia especially distal muscles of upper limbs. Read More

    Dental and Anaesthetic Challenges in a Patient with Dystrophic Epidermolysis Bullosa.
    Sultan Qaboos Univ Med J 2016 Nov 30;16(4):e495-e499. Epub 2016 Nov 30.
    Oral & Maxillofacial Surgery Residency Program, Oman Medical Specialty Board, Muscat, Oman.
    Epidermolysis bullosa is a group of rare genetic disorders characterised by skin and mucous membrane fragility and systemic manifestations of variable severity. We report a case of dystrophic epidermolysis bullosa in an 18-year-old male patient who presented to the Department of Oral Health at Sultan Qaboos University Hospital, Muscat, Oman, in 2015 with recurrent dental pain and infections. Due to the poor dental status of the patient and anticipated operative difficulties due to microstomia and limited mouth opening, the patient underwent full dental clearance under general anaesthesia. Read More

    Mucocutaneous manifestations of Cowden's syndrome.
    Indian Dermatol Online J 2016 Nov-Dec;7(6):512-515
    Department of Oral Pathology and Microbiology, Mamata Dental College and Hospital, Khammam, Telangana, India.
    Cowden's syndrome is an autosomal dominant genodermatosis with variable orofacial and systemic manifestations. Here we present one such classical case of Cowden's syndrome in a 45-year-old female patient with features such as multiple cutaneous papillomatosis, oral fibromas, and fibromas involving multiple organs such as gastrointestinal tract (multiple polyps), thyroid disorders, and breast cancer. Read More

    Kawasaki disease for dermatologists.
    Indian Dermatol Online J 2016 Nov-Dec;7(6):461-470
    Allergy Immunology Unit, Department of Pediatrics, PGIMER, Chandigarh, India.
    Kawasaki disease (KD) is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. Read More

    Unusual maxillary osteoblastic and osteolytic lesions presenting as an initial manifestation of childhood acute myeloid leukemia: A case report.
    Quintessence Int 2017 ;48(2):149-153
    Changes in facial bones may represent a manifestation of systemic disease. Dentists play an important role in the early detection of these manifestations of complex systemic diseases. A case of unusual maxillary mixed (osteoblastic and osteolytic) lesions as an initial manifestation of childhood acute myeloid leukemia (AML) is presented. Read More

    Orofacial manifestations in patients with inflammatory rheumatic diseases.
    Best Pract Res Clin Rheumatol 2016 Oct 18;30(5):826-850. Epub 2016 Nov 18.
    Birmingham Behçet's Syndrome Centre of Excellence, Sheldon Block, Birmingham and Midland Eye Centre, City Hospital, Dudley Road, Birmingham, B18 7QH, UK. Electronic address:
    The main orofacial manifestation of the inflammatory rheumatic diseases is that of Sjögren's syndrome. In addition, there is a constellation of orofacial manifestations of the inflammatory rheumatic diseases, many of which are extra-articular with some constituting presenting signs of the underlying rheumatic disease. This review will discuss the orofacial manifestations in a variety of connective tissue diseases and will also allude to the oral adverse drug reactions that may occur as a consequence of therapy. Read More

    Sjögren Syndrome and Other Causes of Sicca in Older Adults.
    Clin Geriatr Med 2017 Feb;33(1):87-103
    National Institute of Dental and Craniofacial Research, National Institutes of Health, 10 Center Drive, Bethesda, MD 20892, USA.
    Dry eye and dry mouth symptoms are each reported by up to 30% of persons more than 65 years of age, particularly in women. Medication side effects are the most common contributing factors. The evaluation of these symptoms requires measures of ocular and oral dryness. Read More

    [Systemic lupus erythematosus : Unusual cutaneous manifestations].
    Hautarzt 2016 Dec;67(12):970-981
    Abteilung Dermatologie und Venerologie, Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Wien, Österreich.
    Background: Various different mucocutaneous symptoms may affect up to 80 % of systemic lupus erythematosus (SLE) patients.

    Objectives: To investigate, various unspecific, but otherwise typical clinical symptoms of skin and mucous membranes that arise in SLE patients other than those defined as SLE criteria such as butterfly rash, chronic cutaneous lupus erythematosus, oral ulcers, and increased photosensitivity.

    Materials And Methods: Extensive search of peer-reviewed scientific articles was performed, medical histories of several SLE patients seen in our department were analyzed, and the rare disease courses in three SLE patients are presented. Read More

    Adult-onset Still's disease-like manifestation accompanied by the cancer recurrence after long-term resting state.
    Mod Rheumatol 2016 Dec 9:1-5. Epub 2016 Dec 9.
    a The First Department of Internal Medicine, Division of Rheumatology and Nephrology , Kyorin University School of Medicine , Tokyo , Japan.
    A 72-year-old woman presented 9 months ago with skin rash on her bilateral forearms, which was followed by intermittent high fever, and stiffness and swelling of her bilateral fingers. She was diagnosed with seronegative rheumatoid arthritis (RA). She had a past history of breast cancer and had undergone breast preservation surgery 13 years previously. Read More

    Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.
    Lupus 2017 May 12;26(6):646-649. Epub 2016 Nov 12.
    2 Department of Pediatrics, Duke University Medical Center, Durham, USA.
    Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Read More

    First Real-World Insights into Belimumab Use and Outcomes in Routine Clinical Care of Systemic Lupus Erythematosus in Germany: Results from the OBSErve Germany Study.
    Rheumatol Ther 2016 Dec 1;3(2):271-290. Epub 2016 Nov 1.
    Medical Department, Immunology and Rare Diseases Business Unit, GlaxoSmithKline, Hamburg, Germany.
    OBSErve Germany was the first observational study of belimumab as add-on treatment for systemic lupus erythematosus (SLE) in routine clinical care in Germany, retrospectively collecting data from 102 SLE patients, 6 months before and after belimumab initiation. Most patients had moderate or severe SLE and several SLE manifestations. After 6 months of belimumab treatment, 78% of patients showed an improvement in overall disease activity of at least 20% in their physician's judgment and for 42% of patients the improvement was at least 50%. Read More

    Cranial Nerve-VI Palsy as the Main Clinical Manifestation of Neurosarcoidosis.
    Neurologist 2016 Nov;21(6):109-111
    *Associated Specialists Inc., United Hospital Center, Bridgeport, WV †Center for Advanced Health Care, Lehigh Valley Health Network, Allentown, PA ‡Neurological Institute of New Jersey, Rutgers, the State University of New Jersey, Newark, NJ.
    Introduction: Sarcoidosis is a chronic, systemic, inflammatory disorder that is characterized by the formation of noncaseating granulomas. Patients may present with cranial nerve palsy, paresthesia, paresis, pyramidal signs, progressive cognitive decline, urinary retention, seizures, or hypothalamic-pituitary syndrome. Although the diagnosis of neurosarcoidosis can be challenging, neurological manifestations of sarcoidosis occur more frequently than previously described. Read More

    Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
    Eur Respir J 2016 Nov 1;48(5):1429-1441. Epub 2016 Sep 1.
    Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre de référence des maladies orphelines pulmonaires; Université de Lyon, Université Lyon I; UCBL-INRA-ENVL-EPHE, UMR754; IFR128; Lyon, France.
    The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49. Read More

    Review of orofacial considerations of systemic sclerosis or scleroderma with report of analysis of 3 cases.
    Indian J Dent 2016 Sep;7(3):134-139
    Department of Oral Medicine and Radiology, SP Dental College, Wardha, Maharashtra, India.
    Scleroderma (skleros; hard, and derma; skin), is currently known as systemic sclerosis due to its progressive nature and widespread tissue involvement. It is a rare connective tissue disorder with a wide range of oral manifestations. Thickening of the skin is the hallmark of the disease. Read More

    Juvenile systemic lupus erythematosus in Nigeria.
    Lupus 2017 Mar 23;26(3):329-333. Epub 2016 Oct 23.
    3 Department of Medicine, University of Ilorin Teaching Hospital, Ilorin, Nigeria.
    Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Read More

    Orofacial manifestations of scleroderma. A literature review.
    Rev Stomatol Chir Maxillofac Chir Orale 2016 Nov 27;117(5):322-326. Epub 2016 Jul 27.
    Service de chirurgie maxillo-faciale, Hôpital de la Timone, 264, rue Saint-Pierre, 13385 Marseille, France; Aix-Marseille Université, Jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France; Aix Marseille Université, CNRS, LPL UMR 7309, 13100 Aix-en-Provence, France.
    Introduction: Scleroderma is a rare disease of the connective tissue (50 to 200 patients/1 million people; 60,000 patients in France). We conducted a literature review about the orofacial manifestations of scleroderma that have been little studied.

    Material And Methods: The 45 articles found in 6 different databases by using the keywords "scleroderma", "systemic sclerosis", "oral medicine", "face" and published between 1944 and 2016 were selected, for a total of 328 patients. Read More

    Concomitant herpes simplex virus colitis and hepatitis in a man with ulcerative colitis: Case report and review of the literature.
    Medicine (Baltimore) 2016 Oct;95(42):e5082
    aDivision of Infectious Diseases, Emory University School of Medicine bDepartment of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital cDivision of Digestive Diseases, Emory University School of Medicine dEmory Transplant Center, Atlanta, GA.
    Background: Herpesvirus infections often complicate the clinical course of patients with inflammatory bowel disease; however, invasive disease due to herpes simplex virus is distinctly uncommon.

    Methods: We present a case of herpes simplex virus colitis and hepatitis, review all the previously published cases of herpes simplex virus colitis, and discuss common clinical features and outcomes. We also discuss the epidemiology, clinical manifestations, diagnosis, and management of herpes simplex virus infections, focusing specifically on patients with inflammatory bowel disease. Read More

    Oral manifestations of gastrointestinal diseases in children. Part 3: Ulcerative colitis and gastro-oesophageal reflux disease.
    Eur J Paediatr Dent 2016 Sep;17(3):248-250
    Department of Paediatrics, University of Milan, Ospedale dei Bambini Vittore Buzzi, Milan, Italy.
    Background: Alterations of the oral cavity are common in children: 22% of children aged less than 4 years and 44% of those aged more than 12 develop dental erosion, 9-95% of children in Europe and in North America develop gingivitis, with adolescents showing a prevalence of more than 60% [Italian Ministry of Health, Guidelines 2013]. Alterations within the oral cavity can be the first sign of systemic diseases and may thus allow for an early diagnosis and treatment. In particular, being the oral cavity a part of the gastrointestinal system, oral alterations can be an expression of a gastrointestinal disease. Read More

    The impact of hepatitis C virus outside the liver: Evidence from Asia.
    Liver Int 2017 Feb 10;37(2):159-172. Epub 2016 Nov 10.
    Department of Internal Medicine and Therapeutics, University of Pavia, Pavia, Italy.
    Between 80 and 115 million people worldwide are chronically infected with hepatitis C virus, with 60%-90% of these being undiagnosed. Untreated chronic hepatitis C (CHC) is associated with progressive liver disease, cirrhosis, hepatocellular carcinoma and liver-related mortality. A number of extrahepatic manifestations are also reported in CHC patients, further adding to the burden of the disease. Read More

    Multiple Roles for B-Lymphocytes in Sjogren's Syndrome.
    J Clin Med 2016 Oct 8;5(10). Epub 2016 Oct 8.
    College of Veterinary Medicine University of Florida, Gainesville, FL 32608, USA.
    Sjogren's syndrome (SS) is a complex heterogeneous autoimmune disease resulting in loss of salivary gland and lacrimal gland function that may include multiple systemic manifestations including lymphoma. Multiple cell types participate in disease pathogenesis. This review discusses evidence for abnormal B cell subpopulations in patients with SS, critical roles of B cells in SS and the status of B cell-directed therapies in the management of patients with SS. Read More

    Management of patients with ocular manifestations in vesiculobullous disorders affecting the mouth.
    Oral Dis 2016 Oct 7. Epub 2016 Oct 7.
    Department of Ophthalmology, Rigshospitalet, University Hospital of Copenhagen, Copenhagen, Denmark.
    Pemphigoid and pemphigus diseases as well as Stevens-Johnson syndrome present as vesiculobullous disorders of the skin and may additionally involve both the oral cavity and the ocular surface. Ocular involvement ranges from mild irritation and dry eye disease to chronic conjunctivitis, symblepharon, eyelid malposition, ocular surface scarring and severe visual loss. In addition to diagnostic assessments, ophthalmologists must treat the dry eye and meibomian gland dysfunction components of these diseases using a stepladder approach, including eyelid hygiene and lubricants. Read More

    What rheumatologists should know about orofacial manifestations of autoimmune rheumatic diseases.
    Rev Bras Reumatol Engl Ed 2016 Sep - Oct;56(5):441-450. Epub 2016 Mar 16.
    Programa de Pós-Graduação em Ciências Médicas, Faculdade de Medicina, Universidade de Brasília (UnB), Brasília, DF, Brazil.
    Orofacial manifestations occur frequently in rheumatic diseases and usually represent early signs of disease or of its activity that are still neglected in clinical practice. Among the autoimmune rheumatic diseases with potential for oral manifestations, rheumatoid arthritis (RA), inflammatory myopathies (IM), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), relapsing polychondritis (RP) and Sjögren's syndrome (SS) can be cited. Signs and symptoms such as oral hyposalivation, xerostomia, temporomandibular joint disorders, lesions of the oral mucosa, periodontal disease, dysphagia, and dysphonia may be the first expression of these rheumatic diseases. Read More

    Orofacial manifestations of systemic sclerosis.
    Br Dent J 2016 Sep;221(6):305-10
    Royal National Hospital for Rheumatic Diseases, Bath and Visiting Senior Lecturer, Department of Pharmacy and Pharmacology, University of Bath.
    Systemic sclerosis (SSc) is a multisystem disease of unknown aetiology characterised by microangiopathy, dysregulated immune function and tissue remodelling, which commonly involves the oral cavity. Orofacial manifestations of SSc contribute greatly to overall disease burden and yet are regularly overlooked and under-treated. This may reflect a pre-occupation amongst rheumatology clinicians on potentially life-threatening internal organ involvement, but is also a consequence of insufficient engagement between rheumatologists and dental professionals. Read More

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