Amyloid myopathy mimicked with idiopathic inflammatory myopathy diagnosed using Congo red staining: a case report.

Amyloid myopathy often occurs in the context of systemic amyloidosis, as a rare manifestation of "light chain" (AL) amyloidosis, accounting for 1% of its incidence. A 58-year-old man with two years history of weakness and edema of lower extremity, elevated creatine kinase (CK), and inflammatory lesions from muscle biopsy which was misdiagnosed as inflammatory myopathy. After immunotherapy, the original symptoms worsened.

Telitacicept add-on therapy in refractory idiopathic inflammatory myopathy: insights from a pilot study.

Objectives: This study aimed to evaluate the therapeutic efficacy of telitacicept based on the conventional treatment in adults with idiopathic inflammatory myopathy (IIM), focusing on its impact on clinical manifestations.

Methods: IIM patients who had been treated with telitacicept for at least 3 months based on the conventional treatment from January 2023 to January 2024 were included in this study. The clinical response to telitacicept was determined based on the ACR/EULAR criteria for minimal, moderate, and major improvement in the total improvement score (TIS).

Primary intramedullary spinal cord lymphoma misdiagnosed as longitudinally extensive transverse myelitis: a case report and literature review.

Background: Primary intramedullary spinal cord lymphoma (PISCL) is rare and easily misdiagnosed with the lack of typical clinical features and non-specific imaging manifestations.

Case Presentation: A 49-year-old man was admitted to our hospital because of persistent limbs numbness, pinprick-like pain in the posterior neck and unsteady gaits. He has brisk tendon reflexes and positive Babinski's sign.

Clinical Features, Treatment, and Prognostic Factors of Childhood-Onset Myasthenia Gravis in a Large Chinese Cohort.

Background: To describe the clinical features of patients with childhood-onset myasthenia gravis (MG) (CMG) and explore predictors affecting the treatment outcomes.

Methods: A retrospective observational cohort analysis of 859 patients with CMG with disease onset before age 14 years was performed at Tongji Hospital.

Results: Patients in the pubertal-onset group (n = 148) had a worse disease course than those in the prepubertal group (n = 711), including a higher incidence of generalized MG (GMG) at presentation, generalization of ocular MG (OMG), and more severe Myasthenia Gravis Foundation of America (MGFA) classification.

The diagnostic value of serum YKL-40 for myocardial involvement in immune-mediated necrotising myopathy.

Objectives: This study aimed to determine the diagnostic value of YKL-40 for myocardial involvement in immune-mediated necrotising myopathy (IMNM).

Methods: We retrospectively analysed the data of patients with IMNM admitted to the Neurology Department at Tongji Hospital between April 2013 and August 2022. Clinical data including patients' demographics, clinical characteristics (disease duration, muscle strength, atrophy, rash, dysphagia, dyspnoea, and myalgia) and laboratory test results were collected from the electronic medical record system.

Increased expression of cell adhesion molecules in myofasciitis.

Background: Myofasciitis is a heterogeneous group of diseases pathologically characterized by inflammatory cell infiltration into the fascia. Endothelial activation plays a critical role in the pathogenesis of the inflammatory response. However, the expression of cellular adhesion molecules (CAMs) in myofasciitis has not been investigated.

Anti-signal recognition particle positive necrotizing myopathy-sjogren's syndrome overlap syndrome: a descriptive study on clinical and myopathology features.

Background And Objective: The aim of this study was to elucidate the clinical and myopathological characteristics of patients with anti-signal recognition particle (SRP) positive immune-mediated necrotizing myopathy (IMNM) overlap Sjogren's syndrome (SS).

Materials And Methods: We retrospectively analyzed the data of anti-SRP positive IMNM patients admitted in the Neurology Department of Tongji Hospital between January 2011 to December 2020. Patients were divided into two groups: anti-SRP IMNM overlap SS group and anti-SRP IMNM control group.

TWEAK and Fn14 are overexpressed in immune-mediated necrotizing myopathy: implications for muscle damage and repair.

Objectives: TNF-like weak inducer of apoptosis (TWEAK) and its sole receptor fibroblast growth factor-inducible 14 (Fn14) are involved in various inflammatory conditions. This study was performed to investigate the potential role of TWEAK/Fn14 in immune-mediated necrotizing myopathy (IMNM).

Methods: Muscle biopsies from patients with IMNM (n = 37) and controls (n = 11) were collected.

The accumulation of muscle RING finger-1 in regenerating myofibers: Implications for muscle repair in immune-mediated necrotizing myopathy.

Background: Muscle RING finger-1 (MuRF-1) plays a key role in the degradation of skeletal muscle proteins. We hypothesize the involvement of MuRF-1 in immune-mediated necrotizing myopathy (IMNM).

Methods: Muscle biopsies from patients with IMNM ( = 37) were analyzed and compared to biopsies from patients with dermatomyositis (DM, = 13), dysferlinopathy ( = 9) and controls ( = 7) using immunostaining.

Dynamic nomogram for predicting generalized conversion in adult-onset ocular myasthenia gravis.

Purpose: To explore the factors and risk mapping model of progression from ocular myasthenia gravis (OMG) to generalized myasthenia gravis (GMG) in adult-onset patients.

Methods: A retrospective, observational cohort study was performed for 435 OMG patients with onset age older than 14 years old. Multivariate Cox regression was used to identify the independent factors affecting generalized conversions that then were incorporated into the construction of the nomogram.

The Clinicopathological Distinction between Immune-Mediated Necrotizing Myopathy and Limb-Girdle Muscular Dystrophy R2: Key Points to Prevent Misdiagnosis.

Background: Limb−girdle muscular dystrophy R2 (LGMD R2) is most frequently misdiagnosed as immune-mediated necrotizing myopathy (IMNM). This study aimed to compare the clinicopathological data of IMNM and LGMD R2 to find distinguishing features. Methods: We retrospectively reassessed the medical data of patients with IMNM (n = 41) and LGMD R2 (n = 8) treated at Tongji Hospital from January 2017 to December 2021.

Remission and relapses of myasthenia gravis on long-term tacrolimus: a retrospective cross-sectional study of a Chinese cohort.

Objective: To identify the factors that predict the remission and relapses in myasthenia gravis (MG) patients improved by prednisone and tacrolimus treatment.

Methods: A retrospective, observational cohort analysis of MG patients who achieved remission after receiving prednisone and tacrolimus were performed at Tongji Hospital. The main outcome measures were the time to remission, prednisone discontinuation, tacrolimus reduction-associated relapse, and treatment outcome.

Application of kNN and SVM to predict the prognosis of advanced schistosomiasis.

Predictive models for prognosis of small sample advanced schistosomiasis patients have not been well studied. We aimed to construct prognostic predictive models of small sample advanced schistosomiasis patients using two machine learning algorithms, k nearest neighbour (kNN) and support vector machine (SVM) utilising routinely available data under the government medical assistance programme. The predictive models were derived from 229 patients from Xiantao and externally validated by 77 patients of Jiayu, two county-level cities in Hubei province, China.

Tacrolimus Combined with Corticosteroids Improved the Outcome of CIDP Patients with Autoantibodies Against Paranodal Proteins.

Purpose: To investigate the response of tacrolimus to chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies against paranodal proteins, including neurofascin-155 (NF155), contactin-1 (CNTN1) and contactin-associated protein 1 (Caspr1).

Methods: We retrospectively reviewed all CIDP patients who carried anti-NF155, CNTN1 and Caspr1 antibodies and were treated with tacrolimus at Tongji hospital from Jan 2018 to Apr 2021.

Results: There were 58 patients with CIDP and only 9 patients had autoantibodies against paranodal proteins (17.

Using multi-parametric quantitative MRI to screen for cardiac involvement in patients with idiopathic inflammatory myopathy.

Idiopathic inflammatory myopathies (IIM) is a group of heterogeneous autoimmune systemic diseases, which not only involve skeletal muscle but also myocardium. Cardiac involvement in IIM, which eventually develops into heart failure, is difficult to identify by conventional examinations at early stage. The aim of this study was to investigate if multi-parametric cardiac magnetic resonance (CMR) imaging can screen for early cardiac involvement in IIM, compared with clinical score (Myositis Disease Activity Assessment Tool, MDAAT).

Endoplasmic Reticulum Stress Is Involved in Muscular Pathogenesis in Idiopathic Inflammatory Myopathies.

Endoplasmic reticulum (ER) stress plays pivotal roles in the regulation of skeletal muscle damage and dysfunction in multiple disease conditions. We postulate the activation of ER stress in idiopathic inflammatory myopathies (IIM). Thirty-seven patients with immune-mediated necrotizing myopathy (IMNM), 21 patients with dermatomyositis (DM), 6 patients with anti-synthetase syndrome (ASS), and 10 controls were enrolled.

Contribution of relapse-associated worsening to overall disability accrual in patients with relapsing-onset multiple sclerosis: A mediation analysis.

Objective: The neurological disability accumulation in patients with relapsing-onset multiple sclerosis (MS) is commonly attributed to relapse-associated worsening (RAW) and progression independent of relapse activity (PIRA). Using a mediation model, this research aimed to investigate and quantify the contributions of RAW and PIRA to the overall disability accrual.

Methods: Clinical data, containing Expanded Disability Status Scale (EDSS) scores, duration, attack number, and demographics, were collected from 121 patients with relapsing-onset MS in China and included in the mediation model.

Efficacy of tacrolimus as long-term immunotherapy for neuronal surface antibody-mediated autoimmune encephalitis.

Aims: We aimed to verify the efficacy and safety of tacrolimus as long-term immunotherapy for the treatment of neuronal surface antibody-mediated autoimmune encephalitis (AE) during the first attack.

Methods: In this retrospective observational cohort study, patients with neuronal surface antibody-mediated AE who experienced the first attack were enrolled. We compared the outcomes of 17 patients who received tacrolimus with those of 47 patients treated without tacrolimus.

Distinct Immunological Features of Inflammatory Demyelinating Diseases of the Central Nervous System.

Objective: The immunological features between neuromyelitis optica spectrum disorder (NMOSD), multiple sclerosis (MS), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), lacked systemic comparisons. Accordingly, we aimed to investigate immunological differences between NMOSD, MS, and MOGAD.

Methods: Patients with MOGAD, MS, and NMOSD who received immunological tests including cytokine profiles and cytometry analysis of the lymphocyte subgroups were retrospectively reviewed and divided into training and validation sets.

Overlapping syndrome mimicking infectious meningoencephalitis in a patient with MOG and GFAP IgG.

Background: Central nervous system overlapping autoimmune syndromes are uncommon, especially with the coexistence of MOG-IgG and GFAP-IgG.

Case Presentation: A 23-year-old woman presented with transient convulsions, a loss of consciousness, persistent fever, headache, and vomiting. Cerebrospinal fluid (CSF) analysis revealed elevated cellularity, and magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement.

Characteristics of immune and inflammatory responses among different age groups of pediatric patients with COVID-19 in China.

Background: Severe cases of coronavirus disease 2019 (COVID-19) among pediatric patients are more common in children less than 1 year of age. Our aim is to address the underlying role of immunity and inflammation conditions among different age groups of pediatric patients.

Methods: We recruited pediatric patients confirmed of moderate COVID-19 symptoms, admitted to Wuhan Children's Hospital from January 28th to April 1st in 2020.

The Clinicopathological Distinction Between Seropositive and Seronegative Immune-Mediated Necrotizing Myopathy in China.

The present study aimed to compare the clinicopathological features of patients with seronegative immune-mediated necrotizing myopathy (IMNM) and those positive for anti-signal recognition particle (SRP) or anti-3-hydroxy-3-methylglutarylcoenzyme-a reductase (HMGCR) antibodies. We retrospectively analyzed the data of patients with IMNM treated in the Neurology Department of Tongji Hospital from January 1, 2013, to December 31, 2019. Among the 117 patients with IMNM, 30.

Modified models to distinguish central nervous system demyelinating diseases with brain lesions.

Background: Brain lesions in patients with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are indistinguishable from those with relapsing-remitting multiple sclerosis (RRMS) and aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-Ab NMOSD).

Methods: Patients with MOGAD, RRMS, and AQP4-Ab NMOSD with abnormal brain lesions were retrospectively reviewed and divided into training and validation sets. Discriminatory models using brain images and demographics were generated to identify optimal predictors using orthogonal partial least square discriminant analysis after principal component analysis (PCA) of clinico-radiological data without a diagnosis.

Tacrolimus as a therapeutic option in patients with acquired neuromyotonia.

Objective: To analyze the clinical characteristics and outcomes of patients diagnosed with acquired neuromyotonia and who were treated with tacrolimus.

Methods: A single center, retrospective study was performed on patients with acquired meuromyotonia whose treatment included tacrolimus. The clinical information, antibody tests, and electromyography results were reviewed.