scholarly journals Diagnosis of Statin-Induced Necrotizing Myopathy: Contribution of Anti-HMGCR Antibodies

2021 ◽  
Vol 34 (13) ◽  
Author(s):  
Cláudia Fidalgo ◽  
Alice Mendes ◽  
Rosário Cunha ◽  
Fernando Rodrigues
Keyword(s):  
Muscle Biopsy ◽  
Indirect Immunofluorescence ◽  
Gold Standard ◽  
Case Reports ◽  
Antibody Detection ◽  
Fluorescence Pattern ◽  
Tissue Sections ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Essential Contribution

Over the last few years, several cases of statin-induced necrotizing myopathy have been described. This myopathy is characterized by the necrosis of muscle fibers and the presence of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibodies. Although the diagnosis of myopathies relies on muscle biopsy, which is considered the gold-standard, the search for autoantibodies has proved to be an essential contribution to the diagnosis of immune-mediated myopathies. The detection of anti-HMGCR antibodies in the patient’s serum can be performed by enzyme immunoassays, and more recently, by imunofluorescence. As for the latter, the detection of anti-HMGCR antibodies is performed on tissue sections by indirect immunofluorescence and is characterized by a typical fluorescence pattern called “HMGCR Associated Liver IFL Pattern”. The authors present two case reports that show the importance of diagnosing statin-induced necrotizing myopathy as quickly as possible and the contribution of anti-HMGCR antibody detection for the diagnosis.

scholarly journals Rituximab in the treatment of immune-mediated necrotizing myopathy: a review of case reports and case series

2021 ◽  
Vol 14 ◽  
pp. 175628642199891
Author(s):  
Anji Xiong ◽  
Guancui Yang ◽  
Zhuoyao Song ◽  
Chen Xiong ◽  
Deng Liu ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Case Reports ◽  
Signal Recognition Particle ◽  
High Dose ◽  
Signal Recognition ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Coa Reductase

Immune-mediated necrotizing myopathy (IMNM) is a group of immune-related myopathies characterized by progressive proximal muscle weakness, extremely high serum creatine kinase (CK) levels, and necrotic muscle fibers with a relative lack of inflammation. Treatment of IMNM is challenging, with most cases refractory to high-dose steroids in combination with multiple immunotherapies. The role of rituximab (RTX) for IMNM has been explored in isolated case reports and small series. The aim of this article was to perform a literature review of patients with IMNM treated with RTX and to evaluate RTX efficacy and safety. A total of 34 patients with IMNM were reviewed: 52.9% (18/34) with anti-signal recognition particle (SRP) antibodies and 47.1% (16/34) with anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibodies. Patient age at onset varied from 11 years to 81 years (mean 41 years). The majority of patients presented as a severe proximal muscle weakness and the peak level of CK varied from 3900 IU/L to 56,000 IU/L (mean 18,440 IU/L). Prior to RTX administration, all patients were treated with high-dose steroids and most were treated with multiple immunotherapies. The reason for initiating RTX was that 64.7% (22/34) of patients showed no improvement after previous treatments, and 35.3% (12/34) of patients relapsed when attempting to wean steroids or other immunosuppressive agents. With regard to RTX efficacy, 61.8% (21/34) of patients presented a response to RTX. Our data may support the use of RTX as an effective treatment strategy against IMNM resistant to steroids and multiple immunotherapies. Meanwhile, RTX as a first-line therapy could be a choice in IMNM, particularly in African Americans with anti-SRP antibody-positive subsets. ANA, antinuclear antibody; CK, creatine kinase; HMGCR, 3-hydroxy-3-methylglutaryl-CoA reductase; IMNM, immune-mediated necrotizing myopathy; MAC, membrane attack complex; MHC-I, major histocompatibility complex-I; RTX, rituximab; SRP, signal recognition particle.

scholarly journals Immunopathological Characterization of Muscle Biopsy Samples from Immune-Mediated Necrotizing Myopathy Patients

2018 ◽  
Vol 24 ◽  
pp. 2189-2196 ◽  
Author(s):  
Qiong Wang ◽  
Yue Li ◽  
Suqiong Ji ◽  
Fang Feng ◽  
Bitao Bu
Keyword(s):  
Muscle Biopsy ◽  
Necrotizing Myopathy ◽  
Immune Mediated

scholarly journals A Case of Refractory Myositis

2019 ◽  
Vol 19 (02) ◽  
pp. 73-74
Author(s):  
Lucas Luk ◽  
Ho So
Keyword(s):  
Muscle Biopsy ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Exposure History

We report here a statin naive lady who had refractory myositis for years. The diagnosis was revised to be anti-HMGCR related immune mediated necrotizing myopathy after repeating the muscle biopsy and checking the autoantibody. This report serves as a diagnostic alert that anti-HMGCR related immune mediated necrotizing myopathy should be considered in refractory myositis cases even though there is no statin exposure history, as both the treatment and prognosis may differ.

Case Report: Delayed Alemtuzumab-Induced Concurrent Neutropenia and Thrombocytopenia in Relapsing-Remitting Multiple Sclerosis

2021 ◽  
pp. 089719002110212
Author(s):  
Akaansha Ganju ◽  
James C. Stock ◽  
Kim Jordan
Keyword(s):  
Multiple Sclerosis ◽  
Case Reports ◽  
Severe Neutropenia ◽  
Cell Counts ◽  
Immune Mediated ◽  
Relapsing Remitting ◽  
Remitting Multiple Sclerosis ◽  
Study Participants ◽  
Relapsing Remitting Multiple Sclerosis

Alemtuzumab is an anti-CD52 monoclonal antibody used to treat relapsing-remitting multiple sclerosis following failure of second-line medications. It is administered intravenously in 2 treatment sequences 1 year apart. This drug is frequently associated with mild infusion reactions within days of administration, increased infection risk, and long term adverse events from secondary autoimmunity. Alemtuzumab-induced serious immune-mediated thrombocytopenia (ITP) is well-reported and occurred in 1.0-2.2% of participants in initial phase 2 and 3 trials for multiple sclerosis. Significant neutropenia, however, is rare and was only observed in 0.1% of study participants. Delayed neutropenia and/or ITP is thought to occur from secondary autoimmunity. Few case reports have described severe neutropenia occurring beyond 2 months of last alemtuzumab dose. We present an unusual case of delayed combined neutropenia and thrombocytopenia that occurred 15 months after the second infusion of alemtuzumab. The patient was asymptomatic and presented following discovery of neutropenia and thrombocytopenia during routine laboratory studies. The patient responded to steroids initially and was discharged, although outpatient cell counts subsequently revealed recurrent neutropenia and ITP. The adverse drug reaction probability (Naranjo) scale was completed and showed probable likelihood that the adverse event was alemtuzumab-related. Long term screening for delayed hematologic abnormalities, at least 4 years after initial dose, is necessary when using alemtuzumab. Greater research is needed to understand the mechanism of drug-associated neutropenia.

scholarly journals Local Chemotherapy as an Adjuvant Treatment in Unresectable Squamous Cell Carcinoma: What Do We Know So Far?

2021 ◽  
Vol 28 (4) ◽  
pp. 2317-2325
Author(s):  
Luigi Bennardo ◽  
Francesco Bennardo ◽  
Amerigo Giudice ◽  
Maria Passante ◽  
Stefano Dastoli ◽  
...  
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Gold Standard ◽  
Systemic Chemotherapy ◽  
Standard Treatment ◽  
Case Reports ◽  
Case Series ◽  
Surgical Removal ◽  
Local Chemotherapy

Background: Squamous cell carcinoma (SCC) is one of the most common cancers involving skin and oral mucosa. Although this condition’s gold-standard treatment is the surgical removal of the lesions, the physician must propose alternative treatments in some cases due to the patient’s ineligibility for surgery. Among the available alternative therapies, local chemotherapy may represent an initial treatment in combination with radiotherapy or systemic chemotherapy due to the low frequency of side-effects and the lack of necessity for expensive devices. Methods: In this paper, we review all available literature in various databases (PubMed, Scopus-Embase, Web of Science), proposing local chemotherapy as a treatment for cutaneous and oral SCC. Exclusion criteria included ocular lesions (where topical treatments are common), non-English language, and non-human studies. Results: We included 14 studies in this review. The majority were case reports and case series describing the treatment of non-resectable localized SCC with either imiquimod or 5-fluorouracil. We also analyzed small studies proposing combination treatments. Almost all studies reported an excellent clinical outcome, with a low risk of relapses in time. Conclusions: Resection of the lesion remains the gold-standard treatment for SCC. When this approach is not feasible, local chemotherapy may represent a treatment alternative, and it may also be associated with radiotherapy or systemic chemotherapy.

Immune-Mediated Necrotizing Myopathy (IMNM)

2019 ◽  
pp. 225-235
Author(s):  
Brittany Adler ◽  
Lisa Christopher-Stine
Keyword(s):  
Necrotizing Myopathy ◽  
Immune Mediated

Clinical and genetic associations of autoantibodies to 3-hydroxy-3-methyl-glutaryl-coenzyme a reductase in patients with immune-mediated myositis and necrotizing myopathy

2015 ◽  
Vol 52 (2) ◽  
pp. 196-203 ◽  
Author(s):  
Vidya Limaye ◽  
Chris Bundell ◽  
Peter Hollingsworth ◽  
Arada Rojana-Udomsart ◽  
Frank Mastaglia ◽  
...  
Keyword(s):  
Coenzyme A ◽  
Genetic Associations ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Coenzyme A Reductase

scholarly journals Immune-Mediated Necrotizing Myopathy: An Often Misdiagnosed Entity

2015 ◽  
Vol 2 (1) ◽  
Author(s):  
Joana Espírito Santo ◽  
Rui Garcia ◽  
Benilde Barbosa ◽  
Olinda Rebelo ◽  
José Pereira de Moura
Keyword(s):  
Necrotizing Myopathy ◽  
Immune Mediated

scholarly journals Evaluation of two new rapid diagnostic tests (RDTs) for the diagnosis of malaria

2013 ◽  
Vol 5 (2) ◽  
pp. 11-15 ◽  
Author(s):  
Fahmida Jahan ◽  
Rubayet Elahi ◽  
Md. Khaja Mohiuddin ◽  
Md. Gulam Musawwir Khan ◽  
Mohammad Shafiul Alam ◽  
...  
Keyword(s):  
Predictive Value ◽  
Gold Standard ◽  
Diagnostic Tests ◽  
Rapid Diagnostic Tests ◽  
Accurate Diagnosis ◽  
The Other ◽  
Antibody Detection ◽  
Resource Limited Settings ◽  
Malaria Rdts ◽  
Resource Limited

Rapid diagnostic tests (RDTs) address the need for accurate diagnosis of malaria, particularly in resource limited settings. In this study, two malaria RDTs were compared with gold standard microscopy: On Site Pf/Pv test detecting Plasmodium falciparum-specific histidine rich protein-2 (Pf HR P2) and P. vivax-specific parasitic lactate dehydrogenase (pLDH) antigens; and SD Bioline anti-Pf/Pv test detecting anti-HR P2 and anti-pL DH antibodies for the diagnosis of P. falciparum and P. vivax infections, respectively. For OnSite test, the overall sensitivity was found 96.2% , specificity 98.2% , positive predictive value (PPV ) 98.2% , negative predictive value (NPV ) 96.4% and agreement with microscopy was found to be 0.94. On the other hand SD Bioline test, the overall sensitivity was 75.4%, specificity 83.7%, PPV 84.3% , NPV 74.5% and agreement with microscopy was 0.59. These data revealed that the R DT based on antigen detection (Onsite test) was more reliable than that based on the antibody detection (SD Bioline test).DOI: http://dx.doi.org/10.3329/bjmm.v5i2.16931 Bangladesh J Med Microbiol 2011; 05 (02): 11-15

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