scholarly journals Immune mediated necrotizing myopathy: A rare complication of statin therapy

2020 ◽  
Vol 10 (2) ◽  
Author(s):  
Shady Piedra Abusharar ◽  
Prashanth Moku ◽  
Sharon Banks ◽  
Fahad M. Khalid ◽  
Charles S. Specht ◽  
...  
Keyword(s):  
Creatine Phosphokinase ◽  
Rare Complication ◽  
Elevated Serum ◽  
Inflammatory Myopathies ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Serum Creatine Phosphokinase ◽  
Elevated Creatinine ◽  
Coa Reductase ◽  
Proximal Myopathy

Immune mediated necrotizing myopathy (IMNM) is part of the inflammatory myopathies group of diseases and presents with muscle weakness, myalgias and elevated serum creatine phosphokinase (CPK). Statin-induced IMNM is a rare complication. We present a patient with IMNM secondary to simvastatin use. The patient presented with proximal myopathy, dysphagia, and elevated creatinine kinase levels, and was subsequently found to have anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) autoantibodies with a necrotizing process on muscle biopsy. This patient’s case was further complicated by sequelae of multiple disease processes, ultimately leading to deterioration of his health.

scholarly journals Neuroleptic Malignant Syndrome with Very High Serum Creatine Phosphokinase: A Case Report

2012 ◽  
Vol 2 (1) ◽  
pp. 56-59
Author(s):  
Kaniz Fatema ◽  
Mohammad Omar Faruq ◽  
ASM Areef Ahsan ◽  
Rownak Jahan Tamanna ◽  
Fatema Ahmed ◽  
...  
Keyword(s):  
Neuroleptic Malignant Syndrome ◽  
Creatine Phosphokinase ◽  
Elevated Serum ◽  
High Serum ◽  
Altered Level ◽  
Serum Creatine Phosphokinase ◽  
Level Of Consciousness ◽  
Creatine Phosphokinase Level ◽  
Elevated Creatinine ◽  
High Index Of Suspicion

The neuroleptic malignant syndrome (NMS), characterized by muscular rigidity, altered level of consciousness, dysautonomias and an elevated creatinine phosphokinase level, is a potentially lethal consequence of treatment with neuroleptics. Although it occurs most frequently with conventional anti psychotics, it may also occur with newer anti psychotic agents. Physicians need to have a high index of suspicion with regard to diagnosing NMS in patients taking neuroleptics regardless of duration or dose and presenting with hyperthermia. It is under diagnosed in critical care settings though various neuroleptics are frequently used in intensive care units for various purpose. We report a case involving a 55-yr old man with psychiatric disorder who presented with NMS with extremely elevated serum creatine phosphokinase level and acute renal failure. DOI: http://dx.doi.org/10.3329/birdem.v2i1.12363 Birdem Med J 2012; 2(1) 56-59

scholarly journals Immune checkpoint inhibitor-associated myopathy: a clinicoseropathologically distinct myopathy

2020 ◽  
Vol 2 (2) ◽  
Author(s):  
Shahar Shelly ◽  
James D Triplett ◽  
Marcus V Pinto ◽  
Margherita Milone ◽  
Felix E Diehn ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Immune Checkpoint ◽  
Immune Checkpoint Inhibitor ◽  
Checkpoint Inhibitor ◽  
Signal Recognition Particle ◽  
Ocular Involvement ◽  
Signal Recognition ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Coa Reductase

Abstract Immune checkpoint inhibitors have revolutionized the landscape of cancer treatment. Alongside their many advantages, they elicit immune-related adverse events, including myopathy, which potentially result in substantial morbidity if not recognized and treated promptly. Current knowledge of immune checkpoint inhibitor-associated myopathy is limited. We conducted a 5-year retrospective study of patients with immune checkpoint inhibitor-associated myopathy. Clinical features, survival and ancillary test findings were analysed and compared with those of immune-mediated necrotizing myopathy patients without immune checkpoint inhibitor exposure seen during the same time period. We identified 24 patients with immune checkpoint inhibitor-associated myopathy (median age 69 years; range 28–86) and 38 patients with immune-mediated necrotizing myopathy. Ocular involvement occurred in 9/24 patients with immune checkpoint inhibitor exposure, without electrodiagnostic evidence of neuromuscular transmission defect, and in none of the immune-mediated necrotizing myopathy patients (P < 0.001). Myocarditis occurred in eight immune checkpoint inhibitor-associated myopathy patients and in none of the immune-mediated necrotizing myopathy patients (P < 0.001). Median creatine kinase was 686 IU/l in the immune checkpoint inhibitor cohort (seven with normal creatine kinase) compared to 6456 IU/l in immune-mediated necrotizing myopathy cohort (P < 0.001). Lymphopenia was observed in 18 and 7 patients with and without immune checkpoint inhibitor exposure, respectively (P < 0.001). Myopathological findings were similar between patients with and without immune checkpoint inhibitor exposure, consisting of necrotic fibres with no or subtle inflammation. Necrotic fibres however arranged in clusters in 10/11 immune checkpoint inhibitor-associated myopathy patients but in none of the immune checkpoint inhibitor-naïve patients (P < 0.001). Despite the lower creatine kinase levels in immune checkpoint inhibitor-exposed patients, the number of necrotic fibres was similar in both groups. Immune checkpoint inhibitor-associated myopathy patients had a higher frequency of mitochondrial abnormalities and less number of regenerating fibres than immune-mediated necrotizing myopathy patients (P < 0.001). Anti-hydroxy-3-methylglutaryl-CoA reductase or signal recognition particle antibodies were absent in patients with immune checkpoint inhibitor exposure but positive in two-thirds of immune checkpoint inhibitor-naïve patients. Most patients with immune checkpoint inhibitor-associated myopathy responded favourably to immunomodulatory treatments, but four died from myopathy-related complications and one from myocarditis. Intubated patients had significantly shorter survival compared to non-intubated patients (median survival of 22 days; P = 0.004). In summary, immune checkpoint inhibitor-associated myopathy is a distinct, treatable immune-mediated myopathy with common ocular involvement, frequent lymphopenia and necrotizing histopathology, which contrary to immune-mediated necrotizing myopathy, is featured by clusters of necrotic fibres and not accompanied by anti-hydroxy-3-methylglutaryl-CoA reductase or signal recognition particle antibodies. Normal or mildly elevated creatine kinase level does not exclude the diagnosis.

ELEVATED SERUM CREATINE PHOSPHOKINASE ACTIVITY IN NON-CARDIAC SHOCK

1981 ◽  
Vol 9 (3) ◽  
pp. 210
Author(s):  
Osamu Kemmotsu ◽  
Kazui Soma ◽  
Kazuhiko Maekawa ◽  
Takashi Ohwada
Keyword(s):  
Creatine Phosphokinase ◽  
Elevated Serum ◽  
Serum Creatine Phosphokinase ◽  
Cardiac Shock

scholarly journals POS0883 DETECTION OF AUTOANTIBODIES AGAINST MUSCLE-SPECIFIC FOUR-AND-A-HALF-LIM DOMAIN 1 (FHL1) IN INFLAMMATORY MYOPATHIES: RESULTS FROM A SINGLE-CENTER COHORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 697.3-698
Author(s):  
A. S. Galindo-Feria ◽  
B. Horuluoglu ◽  
J. Day ◽  
C. Cerqueira ◽  
S. Proudman ◽  
...  
Keyword(s):  
Muscle Atrophy ◽  
Adelaide Hospital ◽  
Enzyme Linked Immunosorbent Assay ◽  
Royal Adelaide Hospital ◽  
Inflammatory Myopathies ◽  
Lim Domain ◽  
Research Support ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Scientific Adviser

Background:Autoantibodies targeting a muscle-specific autoantigen, four-and-a-half-LIM-domain 1 (FHL1), have been previously identified in patients with idiopathic inflammatory myopathies (IIM) (1).Objectives:The aim of this project was to determine the prevalence and associations of anti-FHL antibody in South Australian patients with histologically-confirmed IIM and in an autoimmune disease control (systemic sclerosis (SSc)).Methods:Sera from patients with IIM (n=267) from the South Australian Myositis Database (SAMD), and SSc (n=174) from the Australian Scleroderma Cohort Study (ASCS) followed at the Royal Adelaide Hospital, and healthy controls (HC, n=100) were analyzed for anti-FHL1 autoantibodies by Enzyme-Linked ImmunoSorbent Assay (ELISA). Clinical, serological and histological details were retrieved from the SAMD and the ASCS.Results:Autoantibodies to FHL1 were more frequent in patients with IIM (55/267, 20.5%) compared with SSc (18/174, 10%) (p<0.001) and HC (4/100, 4%) (p<0.001). Muscular vessel inflammation and atrophy were seen more frequently in IIM anti-FHL1+ patients compared with anti-FHL1- (p<0.01 and p<0.05). Dysphagia, marked muscle atrophy, and high CK levels were frequent in anti-FHL1+ patients with inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM). In 35/54 anti-FHL1+ patients, there were no other myositis-specific autoantibodies present. Anti-FHL1 autoantibodies in patients with SSc were associated with gastric antral vascular ectasia.Conclusion:Anti-FHL1 autoantibodies were detected in 20.5% of IIM patients. In IBM and IMNM, the presence of anti-FHL1-autoantibodies was associated with a severe myopathy as suggested by presence of dysphagia and muscle atrophy.References:[1]Albrecht I, Wick C, Hallgren A, Tjarnlund A, Nagaraju K, Andrade F, et al. Development of autoantibodies against muscle-specific FHL1 in severe inflammatory myopathies. J Clin Invest. 2015;125(12):4612-24.Disclosure of Interests:Angeles Shunashy Galindo-Feria: None declared, Begum Horuluoglu: None declared, Jessica Day: None declared, Catia Cerqueira: None declared, Susanna Proudman: None declared, Ingrid E. Lundberg Consultant of: Consulting fees from Corbus Pharmaceuticals, Inc, Grant/research support from: Research grants from Bristol Myers Squibb and Astra Zeneca, Vidya Limaye Consultant of: Scientific adviser for Actelion and Boehringer-Ingelheim, Grant/research support from: PI for clinical trials for Bayer, Boehringer-Ingelheim, Corbus, and CSL

The Myth of Elevated Serum Creatine Phosphokinase Level and Neuroleptic Malignant Syndrome

1991 ◽  
Vol 158 (5) ◽  
pp. 706-707 ◽  
Author(s):  
Adityanjee
Keyword(s):  
Case Report ◽  
Neuroleptic Malignant Syndrome ◽  
Creatine Phosphokinase ◽  
Case Reports ◽  
Elevated Serum ◽  
Original Description ◽  
Related Complication ◽  
Serum Creatine Phosphokinase ◽  
Creatine Phosphokinase Level ◽  
Recent Case Report

The 1980s witnessed the honeymoon between American psychiatry and an enigmatic and mysterious drug complication called neuroleptic malignant syndrome (NMS). I fear that the 1990s are witnessing the same in British psychiatry. Gone is the era when case reports in British literature described the syndrome without identifying it by name (Allan & White, 1972; Moyes, 1973), ostensibly owing to lack of awareness. Probably, no one had bothered to read the original description in the French-language literature (Delay et al, 1960). The pendulum has now swung to the other extreme. Every known drug-related complication is being labelled NMS. In the US, Addonizio et al (1986) suggested a ridiculously high incidence figure of 12.2%. The same trend for overdiagnosis is seen in the recent case report by Dalkin & Lee (1990). The authors of this case report seem to have forgotten that NMS is an idiosyncratic adverse drug reaction which is dose-independent. There is no reason to label every neuroleptic overdose as NMS on grounds of raised creatine phosphokinase (CPK) level.

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.

Elevated Serum-Creatine Phosphokinase Activity in a Family With Malignant Hyperpyrexia

1972 ◽  
Vol 51 (2) ◽  
pp. 220???225
Author(s):  
E. K. ZSIGMOND ◽  
W. H. STARKWEATHER ◽  
G. S. DUBOFF ◽  
K. FLYNN
Keyword(s):  
Creatine Phosphokinase ◽  
Elevated Serum ◽  
Malignant Hyperpyrexia ◽  
Serum Creatine Phosphokinase

Inflammatory myopathies

2020 ◽  
pp. 4537-4546
Author(s):  
Ingrid E. Lundberg ◽  
Hector Chinoy ◽  
Robert Cooper
Keyword(s):  
Gastrointestinal Tract ◽  
Connective Tissue ◽  
Muscle Fibre ◽  
Inclusion Body Myositis ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Heterogenous Group ◽  
Muscle Biopsies

The idiopathic inflammatory myopathies are a heterogenous group of disorders characterized by muscle weakness, inflammation in muscle tissue, and with frequent extramuscular involvement. Autoantibodies are common, supporting the notion of these disorders being autoimmune. Typically, inflammatory cell infiltrates are found in muscle biopsies. Other organs are frequently involved such as skin, lungs, joints, gastrointestinal tract, and the heart. These heterogenous disorders can be subclassified based on clinical and histopathological features, or by autoantibody specificities. The idiopathic inflammatory myopathies have traditionally comprised polymyositis (PM), dermatomyositis (DM), juvenile DM, PM/DM overlapping with another connective tissue disease, and inclusion body myositis. More recently a subgroup with similar clinical features but with no or scarce inflammation and with pronounced muscle fibre necrosis has been identified and termed immune-mediated necrotizing myopathy.

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