19,012 results match your criteria organ involvement

Pulmonary mucormycosis in immunocompetent hosts diagnosed by bronchioalveolar lavage.

BMJ Case Rep 2021 Apr 12;14(4). Epub 2021 Apr 12.

Pathology, KS Hegde Medical Academy, Mangalore, India

Mucormycosis is a rare infection caused by Mucorales fungi belonging to Zygomycetes class. It can present with spectrum of symptoms and signs based of organ involvement. Common forms of mucormycosis includes rhinocerebral, cutaneous, gastrointestinal, pulmonary, disseminated and miscellaneous forms involving bones, breast, kidney and central nervous system. Read More

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The neurology of IGG4-related disease.

J Neurol Sci 2021 Mar 27:117420. Epub 2021 Mar 27.

Division of Neuro-Ophthalmology, Department of Ophthalmology, Massachusetts Eye & Ear Infirmary/Harvard Medical School, Boston, MA, USA; Department of Neurology, Massachusetts General Hospital/Harvard Medical School, Boston, MA, USA. Electronic address:

Purpose Of Review: IgG4-related disease (IgG4-RD) is emerging as a fibro-inflammatory entity affecting multiple organs, including manifold neurologic manifestations. This review discusses general characteristics of IgG4-RD neurologic disease including epidemiology, histology, clinical picture and treatment approaches.

Recent Findings: IgG4-RD is increasingly recognized as an important underlying pathophysiology in multiple disorders of neurologic interest, including orbital inflammation, infundibulo-hypophysitis, hypertrophic pachymeningitis, and even in rare cases CNS parenchymal disease and cranial vascular involvement. Read More

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Elevated Ambulatory Blood Pressure Measurements are Associated with a Progressive Form of Fabry Disease.

High Blood Press Cardiovasc Prev 2021 Apr 12. Epub 2021 Apr 12.

Department of Medicine and Surgery, University of Milano-Bicocca, Via G.B. Pergolesi, 33, 20900, Monza, Italy.

 INTRODUCTION: Published data on hypertension incidence and management in Anderson-Fabry disease are scant and the contribution of elevated blood pressure to organ damage is not well recognized.

Aim: Therefore, we have assessed blood pressure values and their possible correlations with clinical findings in a well described cohort of Fabry patients.

Methods: Between January 2015 and May 2019, all adult Fabry patients (n = 24 females, n = 8 males) referred to our institute were prospectively enrolled. Read More

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Epidemiology of Idiopathic Inflammatory Myopathies in Africa: A Contemporary Systematic Review.

J Clin Rheumatol 2021 Apr 9. Epub 2021 Apr 9.

From the Department of Internal Medicine and Specialties, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Yaoundé, Cameroon Centre for Heart Rhythm Disorders, University of Adelaide and Royal Adelaide Hospital, Adelaide, Australia Rheumatology Unit, Yaoundé Central Hospital, Yaoundé, Cameroon Department of Internal Medicine and Clinical Immunology, CHRU Lille, Referral Centre for rare systemic autoimmune diseases North and Northwest of France, Univ. Lille, INSERM U995, LIRIC-Lille Inflammation Research International Centre, Lille, France.

Background: The epidemiology of idiopathic inflammatory myopathies (IIMs) has been extensively studied in America, Europe, and Asia, but remains unclear in Africa.

Objective: The aim of this review was to summarize available data on the epidemiology of IIMs in Africa.

Methods: We searched MEDLINE, EMBASE, and African Journals Online for studies published up to December 30, 2020, and reporting epidemiological data on IIMs in Africa. Read More

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Advances in cutaneous vasculitis research and clinical care.

Ann Transl Med 2021 Mar;9(5):439

Departments of Dermatology and Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.

Vasculitis is characterized by inflammation and destruction of blood vessels, resulting in downstream ischemic tissue damage. Diagnosis of vasculitis is a careful exercise in clinical-pathologic correlation, depending upon the clinical manifestations, organs involved, the size of affected blood vessels, imaging, and laboratory findings. While some vasculitis subtypes may be confined to the skin, serious internal organ involvement or underlying disease states may also occur. Read More

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Systemic sclerosis (scleroderma): remaining challenges.

Ann Transl Med 2021 Mar;9(5):438

Department of Dermatology, University of California, San Francisco, San Francisco, CA 94115, USA.

Despite progress in treating internal organ involvement in systemic sclerosis (scleroderma) (SSc), such as pulmonary disease, effective treatments for the hallmark of the disease, cutaneous fibrosis, remain elusive. None of the disease-modifying antirheumatic drugs (DMARDS) have shown proven efficacy for SSc skin fibrosis, and there remain no FDA-approved medications, all of which are off-label, for cutaneous fibrosis in SSc. This review article will briefly summarize conventional therapies, biologics and hematopoietic stem cell transplants and select ongoing clinical trials in SSc. Read More

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Polishing the crystal ball: mining multi-omics data in dermatomyositis.

Ann Transl Med 2021 Mar;9(5):435

Ronald O. Perelman Department of Dermatology, NYU Grossman School of Medicine, New York, NY, USA.

Precision medicine, which recognizes and upholds the uniqueness of each individual patient and the importance of discerning these inter-individual differences on a molecular scale in order to provide truly personalized medical care, is a revolutionary approach that relies on the discovery of clinically-relevant biomarkers derived from the massive amounts of data generated by epigenomic, genomic, transcriptomic, proteomic, microbiomic, and metabolomic studies, collectively known as multi-omics. If harnessed and mined appropriately with the help of ever-evolving computational and analytic methods, the collective data from omics studies has the potential to accelerate delivery of targeted medical treatment that maximizes benefit, minimizes harm, and eliminates the "fortune-telling" inextricably linked to the prevailing trial-and-error approach. For a disease such as dermatomyositis (DM), which is characterized by remarkable phenotypic heterogeneity and varying degrees of multi-organ involvement, an individualized approach that incorporates big data derived from multi-omics studies with the results of currently available serologic, histopathologic, radiologic, and electrophysiologic tests, and, most importantly, with clinical findings obtained from a thorough history and physical examination, has immense diagnostic, therapeutic, and prognostic value. Read More

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Thrombotic microangiopathies assessment: mind the complement.

Clin Kidney J 2021 Apr 6;14(4):1055-1066. Epub 2020 Nov 6.

Josep Carreras Leukaemia Research Institute, Hospital Clinic/University of Barcelona Campus, Barcelona, Spain.

When faced with microangiopathic haemolytic anaemia, thrombocytopenia and organ dysfunction, clinicians should suspect thrombotic microangiopathy (TMA). The endothelial damage that leads to this histological lesion can be triggered by several conditions or diseases, hindering an early diagnosis and aetiological treatment. However, due to systemic involvement in TMA and its low incidence, an accurate early diagnosis is often troublesome. Read More

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FoxO1 as a Regulator of Aquaporin 5 Expression in the Salivary Gland.

J Dent Res 2021 Apr 10:220345211003490. Epub 2021 Apr 10.

Department of Pharmacology, School of Dentistry, Kyungpook National University, Daegu, Republic of Korea.

Forkhead box O1 (FoxO1) is a multifunctional initiator, mediator, and repressor of autoimmune diseases in an organ- or disease-specific manner. However, the role of FoxO1 in the salivary gland has not yet been elucidated. In this study, we discovered that FoxO1 and aquaporin 5 (AQP5) are both significantly downregulated in the patients with primary Sjögren syndrome, an autoimmune disease accompanying salivary gland dysfunction. Read More

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Long-term outcomes of oligometastatic breast cancer patients treated with curative intent: an updated report.

Breast Cancer 2021 Apr 11. Epub 2021 Apr 11.

Division of Clinical Oncology and Hematology, The Jikei University School of Medicine, 3-25-8 Nishi-shimbashi, Minato-ku, Tokyo, Japan.

Background: Oligometastatic breast cancer (OMBC) is characterized by limited metastatic tumor numbers and sites. We have reported a 20-year overall survival (OS) rate and relapse-free rate (RFR) of 34.1% and 27. Read More

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Animal Models of COVID-19. I. Comparative Virology and Disease Pathogenesis.

ILAR J 2021 Apr 9. Epub 2021 Apr 9.

Department of Comparative Medicine, John Hopkins School of Medicine, Baltimore, Maryland, USA.

The Coronavirus Disease 2019 (COVID-19) pandemic has fueled unprecedented development of animal models to understand disease pathogenesis, test therapeutics, and support vaccine development. Models previously developed to study severe acute respiratory syndrome coronavirus (SARS-CoV) have been rapidly deployed to study SARS-CoV-2. However, it has become clear that despite the common use of ACE2 as a receptor for both viruses, the host range of the 2 viruses does not entirely overlap. Read More

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Thrombotic Microangiopathy Associated With Macrophage Activation Syndrome: A Multinational Study of 23 Patients.

J Pediatr 2021 Apr 6. Epub 2021 Apr 6.

IRCCS Istituto Giannina Gaslini, Genoa, Italy; Università degli Studi di Genova, Genoa, Italy; Sechenov First Moscow State Medical University, Moscow, Russian Federation.

Objective: To describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA).

Study Design: International pediatric rheumatologists were asked to collect retrospectively the data of patients with the co-occurrence of MAS and TMA. Clinical and laboratory features of patients with systemic juvenile idiopathic arthritis (sJIA)-associated MAS and TMA were compared with those of an historical cohort of patients with sJIA and MAS. Read More

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Clinical profile and evolution of patients with juvenile-onset Behçet's syndrome over a 25-year period: insights from the AIDA network.

Intern Emerg Med 2021 Apr 9. Epub 2021 Apr 9.

Research Center of Systemic Auto Inflammatory Diseases, Behçet's Disease and Rheumatology-Ophthalmology Collaborative Uveitis Center, Rheumatology Unit, Policlinico "Le Scotte", Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale Bracci 16, 53100, Siena, Italy.

Behçet's syndrome (BS) represents an understudied topic in pediatrics: the main aims of our study were to characterize demographic and clinical features of a cohort of BS patients with juvenile-onset managed in three tertiary referral centers in Italy, evaluate their evolution in the long-term, and detect any potential differences with BS patients having an adult-onset. Medical records of 64 juvenile-onset and 332 adult-onset BS followed-up over a 2-year period were retrospectively analyzed and compared. Mean age ± SD of first symptom-appearance was 10. Read More

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How will artificial intelligence and bioinformatics change our understanding of IgA in the next decade?

Semin Immunopathol 2021 Apr 9. Epub 2021 Apr 9.

Faculty of Medicine, Heidelberg University, Heidelberg, Germany.

IgA nephropathy (IgAN) is the most common glomerulonephritis. It is characterized by the deposition of immune complexes containing immunoglobulin A (IgA) in the kidney's glomeruli, triggering an inflammatory process. In many patients, the disease has a progressive course, eventually leading to end-stage kidney disease. Read More

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The utility of immunoglobulin A/complement 3 and immunoglobulin G/immunoglobulin M ratios in the assessment of disease activation in patients with Behçet disease.

Autoimmunity 2021 Apr 9:1-5. Epub 2021 Apr 9.

Department of Dermatology and Veneorology, Ufuk University, Ankara, Turkey.

Objective: Pathogenesis of Behçet disease (BD) has not yet been clearly revealed and there is no ideal test for the estimation of disease activation at present. This study aimed to assess the efficiencies of IgG/IgM and IgA/C3 ratios in determining activation of BD.

Method: This retrospective cohort study consisted of 140 patients with BD. Read More

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Atypical presentation of immunoglobulin G4-related disease as subglottic stenosis: a case-based review.

Rheumatol Int 2021 Apr 8. Epub 2021 Apr 8.

Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, Avda. Valdecilla s/n, 39008, Santander, Spain.

Background: Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory pathology that has been reported to affect principally the retroperitoneum, hepatobiliary system, salivary glands, orbital structures or lymph nodes. However, IgG4-RD with laryngeal involvement is a very rare entity. Our aims were to describe a case of subglottic stenosis as first and only manifestation of IgG4-RD and review the literature. Read More

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