Publications by authors named "Werner Stenzel"
Article Synopsis
- Antisynthetase syndrome is a subtype of idiopathic inflammatory myopathies, characterized by symptoms like muscle inflammation, lung issues, arthritis, rashes, and fever.
- Muscle biopsies reveal unique morphological features that help differentiate Antisynthetase syndrome from other myositis types.
- The workshop aims to establish a consensus on the syndrome's definition, discuss clinical and laboratory profiles, and provide management strategies and classification criteria.
Article Synopsis
- The peripheral nervous system and skeletal muscle are commonly affected in systemic vasculitis, leading to symptoms like myalgia, muscle weakness, and sensory deficits.
- Vasculitic myopathy (VM) is marked by severe muscle pain but normal creatine kinase levels, and can be identified by MRI, while vasculitic neuropathy (VN) causes motor and sensory deficits, especially in the lower limbs.
- Diagnosing these conditions typically involves histopathological examination of nerve and muscle biopsies, which is crucial for determining treatment strategies.
Article Synopsis
- - The article focuses on peripheral neuroimmunological diseases, discussing the varying mechanisms, manifestations, and treatment advancements for conditions like idiopathic inflammatory myopathies and chronic inflammatory demyelinating polyneuropathy.
- - It emphasizes the importance of muscle biopsies for accurate diagnoses, particularly in distinguishing between vasculitic myopathy and myositis, as well as identifying specific autoimmune characteristics through autoantibody testing.
- - The text highlights that ongoing research and clinical trials aim to enhance understanding and treatment strategies for these diverse diseases, potentially leading to significant advancements in patient care.
Article Synopsis
- - The study focuses on improving the diagnosis of idiopathic inflammatory myopathies (IIM) by quantifying key proteins (MHC-1, MHC-2, ICAM-1) using an automated method to reduce biases associated with traditional diagnostic approaches.
- - Researchers conducted double immunofluorescence staining and RNA sequencing on muscle biopsies from various IIM subtypes, finding distinct immunological profiles and confirming the effectiveness of their method through RNA-Seq results.
- - Results indicate that ICAM-1 is particularly significant for diagnosis, showing high sensitivity and specificity in differentiating IIM subtypes, and the study suggests that automated analysis can enhance the understanding of these diseases.
Article Synopsis
- * A study used advanced techniques to analyze muscle biopsy samples from 26 patients, revealing common features such as heightened immune response markers, myofiber damage, and significant protein accumulation related to cell stress.
- * Findings indicated that anti-Ku myositis displays unique histopathological characteristics, distinguishing it from other conditions like inclusion body myositis and immune-mediated necrotizing myopathy, while also showing connections to specific syndromes like systemic sclerosis.
Objectives: Autoantibodies targeting intracellular proteins are common in various autoimmune diseases. In the context of myositis, the pathologic significance of these autoantibodies has been questioned due to the assumption that autoantibodies cannot enter living muscle cells. This study aims to investigate the validity of this assumption.
View Article and Find Full Text PDFMyasthenia gravis is a chronic antibody-mediated autoimmune disease disrupting neuromuscular synaptic transmission. Informative biomarkers remain an unmet need to stratify patients with active disease requiring intensified monitoring and therapy; their identification is the primary objective of this study. We applied mass spectrometry-based proteomic serum profiling for biomarker discovery.
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- Inclusion body myositis (IBM) is a common inflammatory muscle disease in older adults that currently has no effective treatment and presents a mix of inflammatory and degenerative characteristics.
- Researchers used advanced sequencing techniques to analyze muscle biopsies from IBM patients, finding unique patterns of cellular changes compared to other muscle diseases and non-inflammatory muscles.
- Key findings reveal a loss of specific muscle fibers, increased immune cell presence, and markers of cell stress and protein degradation, highlighting potential mechanisms behind muscle degeneration in IBM and pointing to vulnerabilities in type 2 muscle fibers.
Background: This study investigates the association between the mean arterial blood pressure (MAP), vasopressor requirement, and severity of hypoxic-ischemic encephalopathy (HIE) after cardiac arrest (CA).
Methods: Between 2008 and 2017, we retrospectively analyzed the MAP 200 h after CA and quantified the vasopressor requirements using the cumulative vasopressor index (CVI). Through a postmortem brain autopsy in non-survivors, the severity of the HIE was histopathologically dichotomized into no/mild and severe HIE.
Objective: Characteristics of myositis with anti-Ku antibodies are poorly understood. The purpose of this study was to elucidate the pathologic features of myositis associated with anti-Ku antibodies, compared with immune-mediated necrotizing myopathy (IMNM) with anti-signal recognition particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies, in muscle biopsy-oriented registration cohorts in Japan and Germany.
Methods: We performed a retrospective pathology review of patients with anti-Ku myositis samples diagnosed in the Japanese and German cohorts.
Neurological symptoms, including cognitive impairment and fatigue, can occur in both the acute infection phase of coronavirus disease 2019 (COVID-19) and at later stages, yet the mechanisms that contribute to this remain unclear. Here we profiled single-nucleus transcriptomes and proteomes of brainstem tissue from deceased individuals at various stages of COVID-19. We detected an inflammatory type I interferon response in acute COVID-19 cases, which resolves in the late disease phase.
View Article and Find Full Text PDFAnti-MDA5-positive dermatomyositis (MDA5-DM) often presents with extramuscular, especially pulmonary and skin manifestations, and apparent clinical signs of frank myositis can be missing (so called amyopathic DM). We hereby present two male patients who died from respiratory failure during the course of MDA5-DM. While overt signs of myositis or any skin involvement were absent at admission to hospital we noticed conspicuous inflammatory alterations in various skeletal muscles morphologically, showing different degrees of affection.
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- The rise of cancer immunotherapy, particularly immune checkpoint inhibitors (ICIs), has led to improved survival rates for various cancers, but it also brings significant risks of autoimmunity, including severe neurological side effects.
- The study focuses on understanding the role of neuronal autoantibodies in ICI-treated patients, exploring their prevalence and potential correlation with adverse neurological effects.
- This research is the first comprehensive comparison of neuronal autoantibody profiles between ICI-treated cancer patients with and without neurological immune-related adverse events, offering important insights for future clinical and research applications.
Article Synopsis
- Myositis is an autoimmune muscle disease characterized by autoantibodies that target proteins within muscle cells, but their impact on disease development was previously unclear.* -
- This study utilized confocal microscopy and bulk RNA sequencing on muscle biopsies to investigate the presence and effects of these autoantibodies, revealing they accumulate in muscle fibers and disrupt the normal function of their target proteins.* -
- Findings showed that the internalization of these autoantibodies led to significant changes in gene expression and function, indicating that they play a crucial role in causing the pathology associated with myositis.*
Article Synopsis
- * The review highlights the importance of major histocompatibility complex (MHC) and complement patterns in immunohistochemical analysis to aid in diagnosis, especially for idiopathic inflammatory myopathies.
- * A proposed diagnostic algorithm aims to streamline the diagnostic process using a limited set of stains while noting the need for more extensive studies to establish effective and standardized diagnostic protocols.
: To explore a possible connection between active viral infections and manifestation of dermatomyositis (DM). Skeletal muscle biopsies were analyzed from patients diagnosed with juvenile (n=10) and adult (n=12) DM. Adult DM patients harbored autoantibodies against either TIF-1γ (n=7) or MDA5 (n=5).
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