scholarly journals Clinical applications of immunoglobulin in neuromuscular diseases: focus on inflammatory myopathies

2014 ◽  
Vol 72 (12) ◽  
pp. 966-971
Author(s):  
Paulo Victor Sgobbi de Souza ◽  
Wladimir Bocca Vieira de Rezende Pinto ◽  
Acary Souza Bulle Oliveira
Keyword(s):  
Intravenous Immunoglobulin ◽  
Mechanism Of Action ◽  
Neurological Diseases ◽  
Clinical Manifestations ◽  
Neuromuscular Diseases ◽  
Clinical Applications ◽  
Inflammatory Myopathies ◽  
Pathophysiological Mechanisms ◽  
Therapeutic Uses ◽  
Immune Mediated

During recent years, an increasing number of neuromuscular diseases have been recognized either to be caused primarily by autoimmune mechanisms, or to have important autoimmune components. The involved pathophysiological mechanisms and clinical manifestations have been better recognized and many of these disorders are potentially treatable by immunosuppression or by immunomodulation with intravenous immunoglobulin (IVIg). IVIg has been tried in a variety of immune-mediated neurological diseases, being target of widespread use in central and peripheral nervous systems diseases. Objective To give an overview of the main topics regarding the mechanism of action and different therapeutic uses of IVIg in neurological practice, mainly in neuromuscular diseases.

scholarly journals Non-paraneoplastic Lambert-Eaton myasthenic syndrome: a brief review of 10 cases

2010 ◽  
Vol 68 (6) ◽  
pp. 849-854 ◽  
Author(s):  
Paulo J. Lorenzoni ◽  
Rosana H. Scola ◽  
Cláudia S. Kamoi Kay ◽  
Sérgio F. Parolin ◽  
Lineu C. Werneck
Keyword(s):  
Plasma Exchange ◽  
Intravenous Immunoglobulin ◽  
Age At Onset ◽  
Immunosuppressive Treatment ◽  
Clinical Manifestations ◽  
Symptomatic Treatment ◽  
Laboratory Findings ◽  
Immune Mediated ◽  
Electrophysiological Studies ◽  
Myasthenic Syndrome

Lambert-Eaton myasthenic syndrome (LEMS) is an immune-mediated disorder of the presynaptic neuromuscular transmission, which more frequently occurs as the remote effect of a neoplasm, in the paraneoplastic form (P-LEMS), or in a non-paraneoplastic form (NP-LEMS); but few studies describe the clinical features of NP-LEMS. We analyzed the clinical manifestations, laboratory findings, electrophysiological studies, and treatment responses in ten Brazilian patients suffering from NP-LEMS. The mean age was 41.5 years. More often neurological findings were hyporeflexia or areflexia with a post-exercise improvement. Treatment response occurred with pyridostigmine, guanidine, prednisone, azathioprine, and cyclosporine; but not response was observed after intravenous immunoglobulin and plasma exchange. Age at onset, clinical manifestations, and electrophysiological abnormalities can help more in the diagnosis than serum antibodies; the symptomatic treatment with pyridostigmine was effective; and the immunosuppressive treatment with prednisone, azathioprine, or cyclosporine was more beneficial than plasma exchange or intravenous immunoglobulin treatment.

scholarly journals Anti-HMGCR Antibody-Positive Myopathy Shows Bcl-2-Positive Inflammation and Lymphocytic Accumulations

2020 ◽  
Vol 79 (4) ◽  
pp. 448-457
Author(s):  
Takashi Kurashige ◽  
Tomomi Murao ◽  
Naoko Mine ◽  
Tomohito Sugiura ◽  
Yukiko Inazuka ◽  
...  
Keyword(s):  
Cholesterol Biosynthesis ◽  
Low Density Lipoprotein ◽  
Clinical Manifestations ◽  
Density Lipoprotein ◽  
Inflammatory Myopathies ◽  
Cholesterol Levels ◽  
Immune Mediated ◽  
The Difference ◽  
Essential Enzyme ◽  
Chemokine Receptor 4

Abstract Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) and antisignal recognition particle (SRP) antibodies are frequently associated with immune-mediated necrotizing myopathy (IMNM). However, the difference in clinical manifestations between anti-HMGCR and anti-SRP antibodies is unclear. HMGCR is an essential enzyme for cholesterol biosynthesis and is inhibited by statins that regulate apoptosis of Bcl-2-positive and beta chemokine receptor 4 (CCR4)-positive lymphoma cells. In this study, we aimed to clarify Bcl-2 and CCR4 expressions of lymphocytes in anti-HMGCR antibody-positive IMNM and explore the difference between anti-HMGCR antibody-positive myopathy and other inflammatory myopathies. We retrospectively examined Bcl-2- and CCR4-positive lymphocyte infiltrations in muscle and skin biopsy specimens from 19 anti-HMGCR antibody-positive patients and 75 other idiopathic inflammatory myopathies (IIMs) patients. A higher incidence of Bcl-2- and CCR4-positive lymphocytes was detected in the muscle and skin of anti-HMGCR antibody-positive IMNM patients (p < 0.001). In 5 patients with anti-HMGCR antibodies, Bcl-2-positive lymphocytes formed lymphocytic accumulations, which were not observed in other IIMs. Low-density lipoprotein cholesterol levels were not increased except for patients with Bcl-2-positive lymphocytic accumulations (p = 0.010). Bcl-2 and CCR4 lymphocyte infiltrations could be a pathological characteristic of anti-HMGCR antibody-positive IMNM.

scholarly journals Series of clinical cases of patients with idiopathic inflammatory myopathy and interstitial lung disease from the registry of idiopathic inflammatory myopathies of the Argentine Society of Rheumatology

2020 ◽  
pp. 12-17
Author(s):  
Y.M. Ponce ◽  
M.M. Zalazar ◽  
A.D. García Coello ◽  
O.L. Rillo
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inflammatory Myopathy ◽  
Clinical Manifestations ◽  
Idiopathic Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Disease Prognosis ◽  
Immune Mediated ◽  
Necrotizing Myositis

Idiopathic Inflammatory Myopathies (MII) are a heterogeneous group of diseases characterized by muscle weakness and inflammation underlying muscle biopsy. The main organs affected are muscle, skin and the lung can also be affected. They are distinguished within clinical subtypes such as Polymyositis (PM), Dermatomyositis (DM), DM with the variant Clinically Amiopathic Dermatomyositis (DMCA), the Syndrome Antisynthetase (SAS), Immune-mediated Necrotizing Myositis, Body Myositis Inclusion (MCI) and Neoplasia-Associated Myositis. The presence of certain specific and associated antibodies predisposes to the development of clinical manifestations, determining the disease prognosis. 4 patients from the Registry of MII of the Argentine Society of Rheumatology (SAR) are presented with these characteristics: one patient with PM and anti Jo-1 positive and three patients with DM (one with DMCA and anti-RO 52 and two patients with anti-PL7 and anti-TI-F1γ respectively).

Biological therapy in idiopathic inflammatory myopathies

2014 ◽  
Vol 155 (1) ◽  
pp. 3-10
Author(s):  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Andrea Péter ◽  
Csilla András ◽  
...  
Keyword(s):  
T Cells ◽  
Muscle Weakness ◽  
Biological Therapy ◽  
Therapeutic Options ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Progressive Muscle Weakness ◽  
Immune Mediated ◽  
Novel Therapeutic

Idiopathic inflammatory myopathies are systemic, immune-mediated diseases characterized by proximal, symmetrical, progressive muscle weakness. The aim of this work is to give an overview of the biological therapy used in the treatment of idiopathic inflammatory myopathies. The authors also focus on novel results in the therapy directed against the B- and T-cells. They emphasize the importance of new trials in these diseases which may lead to the introduction of novel therapeutic options in these disorders. Orv. Hetil., 2014, 155(1), 3–10.

scholarly journals Dual threat of comorbidity of celiac disease and systemic lupus erythematosus

2021 ◽  
Vol 49 (5) ◽  
pp. 030006052110122
Author(s):  
Yimin Ma ◽  
Duanming Zhuang ◽  
Zhenguo Qiao
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Celiac Disease ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Failure To Thrive ◽  
Severe Malnutrition ◽  
Systemic Lupus ◽  
Electrolyte Disorders ◽  
Diagnostic Challenge ◽  
Immune Mediated

Celiac disease (CD) is a chronic immune-mediated intestinal disease that is characterized by production of autoantibodies directed against the small intestine. The main clinical manifestations of CD are typically defined as those related to indigestion and malabsorption. These manifestations include unexplained diarrhea or constipation, abdominal pain, bloating, weight loss, anemia, failure-to-thrive in children, and decreased bone density. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous clinical manifestations, which may also involve the gastrointestinal tract. Comorbidity of CD and SLE is rare, and the overlapping symptoms and nonspecific clinical presentation may pose a diagnostic challenge to clinicians. We report here a case of SLE with CD, which mainly manifested as recurrent diarrhea, uncorrectable electrolyte disorders, and severe malnutrition. Through review, we hope to further improve our understanding and diagnostic level of this combination of diseases.

scholarly journals How the Heart Was Involved in COVID-19 during the First Pandemic Phase: A Review

Epidemiologia ◽  
2021 ◽  
Vol 2 (1) ◽  
pp. 124-139
Author(s):  
Andrea Canalella ◽  
Ermanno Vitale ◽  
Francesca Vella ◽  
Paola Senia ◽  
Emanuele Cannizzaro ◽  
...  
Keyword(s):  
Public Health ◽  
Diabetes Mellitus ◽  
Scientific Community ◽  
Respiratory Virus ◽  
Clinical Manifestations ◽  
Hubei Province ◽  
Public Health Emergency ◽  
Converting Enzyme ◽  
Pathophysiological Mechanisms ◽  
Cardiovascular Involvement

Coronavirus disease (COVID-19) was first observed in Wuhan, Hubei Province (China) in December 2019, resulting in an acute respiratory syndrome. Only later was COVID-19 considered a public health emergency of international concern and, on 11 March 2020, the WHO classified it as pandemic. Despite being a respiratory virus, the clinical manifestations are also characterized by cardiological involvement, especially in patients suffering from previous comorbidities such as hypertension and diabetes mellitus, its complications being potentially serious or fatal. Despite the efforts made by the scientific community to identify pathophysiological mechanisms, they still remain unclear. A fundamental role is played by the angiotensin 2 converting enzyme, known for its effects at the cardiovascular level and for its involvement in COVID-19 pathogenesis. The goal of this paper was to highlight the mechanisms and knowledge related to cardiovascular involvement during the first pandemic phase, as well as to emphasize the main cardiological complications in infected patients.

scholarly journals Inflammatory Skin Lesions in Three SARS-CoV-2 Swab-Negative Adolescents: A Possible COVID-19 Sneaky Manifestation?

2021 ◽  
Vol 13 (2) ◽  
pp. 181-188
Author(s):  
Giuseppe Ingravallo ◽  
Francesco Mazzotta ◽  
Leonardo Resta ◽  
Sara Sablone ◽  
Gerardo Cazzato ◽  
...  
Keyword(s):  
Electron Microscopy ◽  
Respiratory Symptoms ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Sweat Glands ◽  
Histological Findings ◽  
Immune Mediated ◽  
Nodular Lesions ◽  
Immunohistochemical Evidence ◽  
Eccrine Sweat Glands

Coronavirus disease 19 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is associated with various clinical manifestations, including skin lesions. In particular, during the COVID-19 pandemic lock-down period numerous chilblain-like lesions, mainly located on the feet, were observed in adolescents. The latter were often asymptomatic or associated with very mild respiratory symptoms. Here, we report three cases of acral nodular lesions in SARS-CoV-2 swab-negative adolescents with histological findings of chronic immune-mediated inflammation and immunohistochemical evidence of SARS-CoV-2 spike glycoproteins in endothelial cells and eccrine sweat glands. In one of these cases, the virus presence was confirmed by electron microscopy.

Treatment of Heparin-Induced Thrombocytopenia

2002 ◽  
Vol 36 (3) ◽  
pp. 489-503 ◽  
Author(s):  
William E Dager ◽  
Richard H White
Keyword(s):  
Adverse Reaction ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Low Molecular Weight ◽  
National Library ◽  
Severe Adverse Reaction ◽  
Heparin Induced Thrombocytopenia ◽  
Immune Mediated ◽  
Study Selection ◽  
Clinical Conditions

OBJECTIVE: To describe heparin-induced thrombocytopenia (HIT or HIT-2), an immune-mediated adverse reaction to heparin or low-molecular-weight heparin. Available treatment options and considerations in developing a therapy approach are discussed. DATA SOURCES: A search of the National Library of Medicine (1992–June 2001) was done to identify pertinent literature. Additional references were reviewed from selected articles. STUDY SELECTION: Articles related to laboratory recognition and treatment options of HIT, including the use of agents in selected clinical conditions, were reviewed and included. CONCLUSIONS: HIT is a rare but potentially severe adverse reaction to heparin that was, until recently, poorly understood and had limited treatment options. Recent advances describing the recognition and clinical manifestations of immune-mediated HIT, including recently available antithrombotic treatment options, have dramatically changed outcomes for patients having this syndrome.

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