Serum Transaminases Are Frequently Elevated at Time of Diagnosis of Idiopathic Inflammatory Myopathy and Normalize With Creatine Kinase

2014 ◽  
Vol 20 (3) ◽  
pp. 130-132 ◽  
Author(s):  
Tanisha Mathur ◽  
Augustine M. Manadan ◽  
Saravanan Thiagarajan ◽  
Bala Hota ◽  
Joel A. Block
2020 ◽  
Vol 8 ◽  
pp. 2050313X2098412
Author(s):  
Darosa Lim ◽  
Océane Landon-Cardinal ◽  
Benjamin Ellezam ◽  
Annie Belisle ◽  
Annie Genois ◽  
...  

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary. This entity is not commonly known among dermatologists as there are usually no extramuscular manifestations. We report a rare case of statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like cutaneous features. The possibility of anti-HMGCR immune-mediated necrotizing myopathy should be considered in patients with cutaneous dermatomyositis-like features associated with severe proximal muscle weakness, highly elevated creatine kinase levels and possible statin exposure. This indicates the importance of muscle biopsy and specific autoantibody testing for accurate diagnosis, as well as significant therapeutic implications.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 714.2-714
Author(s):  
M. Qiu ◽  
X. Sun ◽  
F. Lu ◽  
Q. Wang ◽  
L. Zhou

Background:Cardiac involvement is a serious complication of idiopathic inflammatory myopathy (IIM). Early diagnosis and intervention can improve prognosis. At present, myocardial biopsy is the gold standard for its diagnosis, but it is not commonly used because of its invasiveness. Biomarkers can be invoked as a non-invasive and convenient choice. The traditional markers of myocardial injury, as troponin and creatine kinase are lack specificity in inflammatory myopathy, so the novel biomarkers are getting attention.GDF-15 can predict the risk of cardiovascular disease and the prognosis of coronary atherosclerosis, heart failure and other diseases.Objectives:This article was intended to investigate the diagnostic value of GDF-15 for myocardial involvement in inflammatory myopathy.Methods:This retrospective study included 54 patients with inflammatory myopathy from May 2018 to October 2019.Of these,30 patients underwent cardiac magnetic resonance examination due to increased myocardial markers, excluding 1 case of severe lung infection. 33 patients with systemic lupus erythematosus (SLE),16 normal patients were used as the control group.The concentration of GDF-15 in the serum of all groups of patients was measured by ELISA.Results:1. There were significantly differences in GDF-15 levels in patients with inflammatory myopathy, systemic lupus erythematosus and normal subjects (H =39.870, P <0.001).2. 29 patients with cardiac magnetic resonance on the basis of the delayed enhancement (LGE) and ECV results were divided into two groups in which 19 patients with myocardial injury group and 10 patients without myocardial injury. The best cut-off value was calculated by ROC curve,and comparing GDF-15 and CKMB with the optimum cut-off values in predicting cardiac involvement in IIM.GDF-15 levels were statistically significant between the myocardial injury group (1765.868±1068.549 pg/ml) and the group without myocardial injury(689.967±458.12 pg/ml)(p =0.0011).At the same time, the creatine kinase isoenzyme (CKMB)(158.583±119.389 U/L vs 57.96±52.673 U/L, p =0.005) was statistically different between the two groups.3.GDF-15≥1005.3650pg/ml (AUC =0.853,95% CI 0.694-1.000) predicted myocardial involvement in inflammatory diseases with a sensitivity of 0.765 and specificity of 0.900.The AUC of the ROC curve for the joint detection of GDF-15 and CKMB was 0.888,95% CI0.757-1.000,with the predicted probability cut-off value in 0.3895, the sensitivity 0.941 and the specificity 0.800.The combined detection of the two increased the sensitivity of myocardial damage detection in IIM patients. 5. After adjusted for age, renal function, the risk of myocardial injury in IIM patients increased by an average of 0.3% per unit of GDF-15(OR =1.003,95% CI 1.000–1.005).Conclusion:GDF-15 can predict myocardial injury in patients with inflammatory myopathy which have high specificity.The prediction sensitivity can be improved by combining with the traditional myocardial enzyme CKMB.More further studies are needed to confirm the specific mechanism of GDF-15 for myocardial involvement to assess the prognosis of such patients and guide further treatment.References:[1]Sultan SM, Ioannou Y, Moss K, Isenberg DA. Outcome in patients with idiopathic inflflammatory myositis: morbidity and mortality. Rheumatology (Oxford) 2002;41:22–6.[2]Lundberg IE, de Visser M, Werth VP. Classification of myositis. Nat Rev Rheumatol. 2018 May;14(5):269-278.[3]Zhang L, Wang GC, Ma L, Zu N (2012) Cardiac involvement in adult polymyositis or dermatomyositis: a systematic review. Clin Cardiol 35(11):686–691.[4]Chen F,Peng Y,Chen M. Diagnostic approach to cardiac involvement in idiopathic inflammatory myopathies.A strategy combining cardiac troponin I but not T assay with other methods[J].Int Heart J,2018;59:256-262Disclosure of Interests:None declared


2021 ◽  
pp. 154-156
Author(s):  
Margherita Milone ◽  
Teerin Liewluck

A 47-year-old man with hypercholesterolemia sought care for a 4-month history of progressive, proximal upper limb weakness and myalgia, followed by dysphagia, difficulty climbing stairs, and facial rash. Discontinuation of atorvastatin was of no benefit. Neurologic examination showed moderate weakness of the neck flexor muscles, shoulder girdle muscles, and finger extensors, and mild weakness of hip flexor and ankle dorsiflexor muscles. He had a heliotrope rash and Gottron sign. Serum testing showed an increased creatine kinase level. Needle electromyography showed myopathic changes with fibrillation potentials in proximal and axial muscles. Biopsy of the deltoid demonstrated a perifascicular pathologic process, including muscle fiber atrophy, and perivascular inflammatory exudate in the perimysium. Immunocytochemical studies showed patchy loss of intramuscular capillaries, some of which had complement (C5b9) deposition, and sarcoplasmic expression of myxovirus resistance protein A, mainly in the perifascicular regions. Immunologic testing was positive for autoantibodies to nuclear matrix protein 2 and negative for 3-hydroxy-3-methylglutaryl–coenzyme A reductase antibodies. Video swallow studies showed oropharyngeal dysphagia. Pulmonary function tests indicated mildly decreased maximal respiratory pressures but normal diffusing lung capacity for carbon monoxide. The findings were consistent with a diagnosis of dermatomyositis. The patient was started on oral prednisone and azathioprine, after checking for adequate thiopurine methyltransferase activity. Liver function tests and complete blood cell count with differential were assessed to monitor for potential azathioprine toxicity. Intravenous immunoglobulin was given. Follow-up examination revealed mild weakness of the shoulder girdle muscles after immunotherapy, and normal strength and creatine kinase value while on azathioprine monotherapy. Dermatomyositis is an idiopathic inflammatory myopathy. Idiopathic inflammatory myopathy is a group of autoimmune muscle diseases that includes dermatomyositis, polymyositis, inclusion body myositis, immune-mediated necrotizing myopathy, and overlap myositis, including antisynthetase syndrome.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
E Bollache ◽  
AT Huber ◽  
J Lamy ◽  
E Afari ◽  
TM Bacoyannis ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background. Recent studies revealed the ability of MRI T1 mapping to characterize myocardial involvement in both idiopathic inflammatory myopathy (IIM) and acute viral myocarditis (AVM), as compared to healthy controls. However, neither myocardial T1 nor T2 maps were able to discriminate between IIM and AVM patients, when considering conventional myocardial mean values and derived indices such as lambda and extracellular volume. Purpose. To investigate the ability of T1 mapping-derived texture analysis to differentiate IIM from AVM. Methods. Forty patients, 20 with IIM (51 ± 17 years, 9 men) and 20 with AVM (34 ± 13 years, 16 men) underwent 1.5T MRI T1 mapping using a modified Look-Locker inversion-recovery sequence before and 15 minutes after injection of a gadolinium contrast agent. After manual delineation of endocardial and epicardial borders and co-registration of all inversion time images, native and post-contrast T1 maps were estimated. Myocardial texture analysis was performed on native T1 maps. Textural features such as: autocorrelation, contrast, dissimilarity, energy and sum entropy were used to build a least squares-based linear regression model. Finally, receiver operating characteristic (ROC) analysis was used to investigate the ability of such texture features score to classify IIM vs. AVM patients, compared to the performance of mean myocardial T1. A Wilcoxon rank-sum test was also used to test difference significance between groups. Results. Both native and post-contrast mean myocardial T1 values were comparable between IIM (native: 1022 ± 43 ms; post-contrast: 319 ± 44 ms) and AVM (1056 ± 59 ms, p = 0.07; 318 ± 35 ms, p = 0.90, respectively) groups. Results of ROC analyses are provided in the Table, indicating that a better discrimination between IIM and AVM patients was obtained when using texture features, with higher AUC and accuracy than mean T1 values (Figure). Conclusion. Texture analysis derived from MRI T1 maps without contrast agent injection was able to discriminate between IIM and AVM with higher accuracy, sensitivity and specificity than conventional T1 indices. Such analysis could provide a useful myocardial signature to help diagnose and manage cardiac alterations associated with IIM in patients presenting with myocarditis and primarily suspected of AVM. Table Area under curve (AUC) Accuracy Sensitivity Specificity Native T1 0.67 0.70 0.65 0.75 Post-contrast T1 0.49 0.60 0.25 0.95 Texture features score 0.85 0.82 0.90 0.75 ROC analyses for classification between IIM and AVM patients Abstract Figure


Author(s):  
Tatiana Cobo-Ibáñez ◽  
Carlos Sánchez-Piedra ◽  
Laura Nuño-Nuño ◽  
Iván Castellví ◽  
Irene Carrión-Barberà ◽  
...  

2002 ◽  
Vol 21 (5) ◽  
pp. 391-396 ◽  
Author(s):  
C.-S. Lee ◽  
T.-L. Chen ◽  
C.-Y. Tzen ◽  
F.-J. Lin ◽  
M.-J. Peng ◽  
...  

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