scholarly journals Necrotizing myopathy presenting as congestive heart failure and life-threatening ventricular arrhythmias: a case report

2021 ◽  
Vol 5 (6) ◽  
Author(s):  
Kyunghee Lim ◽  
Jong Sung Park ◽  
Byeol-A Yoon ◽  
Song-Hee Han

Abstract Background Necrotizing autoimmune myopathy is a rare subtype of idiopathic inflammatory myopathy; however, it can be associated with fatal cardiac manifestations. Case summary A 58-year-old female patient was referred for congestive heart failure with dysrhythmia. Electrocardiograms showed ventricular arrhythmias of various QRS complex morphologies and coupling intervals with beat-to-beat differences. Despite optimal medical therapy for heart failure, the patient was admitted for the progression of dyspnoea and generalized motor weakness. The burden of non-sustained ventricular tachycardia gradually increased, and ventricular fibrillation eventually occurred. In view of a differential diagnosis of an inflammatory myocardial diseases such as sarcoidosis, a cardiac biopsy was performed. However, pathologic examinations revealed only necrotic muscle fibres without granuloma. Further examinations revealed proximal dominant motor weakness, an elevated serum creatinine-phosphokinase level, myogenic potentials on needle electromyography, and biceps muscle biopsy findings that were compatible with necrotizing autoimmune myopathy. High-dose steroid therapy improved the patient’s motor weakness, including her respiratory impairment, and successfully suppressed ventricular arrhythmias. Discussion This case suggests that intensive immunosuppressive therapy with high-dose steroid could be useful in the necrotizing autoimmune myopathy manifested as congestive heart failure and life-threatening ventricular arrhythmias.

2019 ◽  
Vol 12 (5) ◽  
pp. e230213 ◽  
Author(s):  
Mitchell Pitlick ◽  
Floranne Ernste

Necrotising autoimmune myopathy (NAM) is an immune-mediated myopathy that may be associated with statin use, malignancy or an autoimmune connective tissue disease, but it can also be idiopathic. Anti-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) myopathy is an extremely rare side effect of statin use, occurring in approximately 2–3 out of every 100 000 patients who use statins. Patients typically present with subacute proximal muscle weakness and creatine kinase levels >10 times the upper limit of normal. The diagnosis is suggested by muscle biopsy showing necrotic fibres with minimal inflammation along with positive anti-HMGCR antibodies. Treatment nearly always requires multiple immunosuppressive agents, the earlier use of which is associated with improved outcomes. Reports of statin-induced NAM leading to heart failure are limited. We present the case of a 69-year-old woman with statin-induced NAM who presented with acute systolic heart failure. Early initiation of high-dose corticosteroids and IVIG resulted in significant improvement in her symptoms.


2009 ◽  
Vol 32 (9) ◽  
pp. 1173-1177 ◽  
Author(s):  
YEN-HUNG LIN ◽  
LIAN-YU LIN ◽  
YING-SHREN CHEN ◽  
HUI-CHUN HUANG ◽  
JEN-KUANG LEE ◽  
...  

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Maria Jesãºs Puchades Montesa ◽  
Nayara Panizo Gonzalez ◽  
Luis D'Marco ◽  
Miguel Gonzalez-Rico ◽  
Patricia Tomas ◽  
...  

Abstract Background and Aims Hyperkalemia (HK) is a potentially life-threatening condition, in patients with chronic kidney disease (CKD) and congestive heart failure (CHF). The majority of patients affected with CKD or CHF, must be treated with inhibitors of renin angiotensin aldosterone system (RAASi) and mineralocorticoid receptor antagonists (MRAs). However, the treatments previously mentioned, increase the risk of HK episodes, which is the main cause of RAASi and MRAs downtitration or discontinuation, representing an undesirable clinical scenario, given that the patients are at high risk of be deprived of their nephroprotective effect and cardio-renal benefits The aim of the study is: to analyze if, in patients with HK, CKD and CHF treated with RAASi and/or MRA, serum potassium (sK) reduction by Sodium zirconium cyclosilicate (SZC) treatment is non-inferior to RAASi and/or MRAs discontinuation or downtitration. Method Results The study will demonstrate results on serum electrolytes, renal function, albuminuria, KDQoL questionnaire and changes in relative overhydration (multifrequency bioimpedance -BCM Fresenius-) Conclusion The KEEP ON study will define the ability of SZC to facilitate the use of RAAS-I and / or MRA in patients with HK and cardiorenal syndrome allowing the maintenance of the medications recommended by international guidelines for the treatment of CHF at different degrees of CKD while maintaining the potential cardio-renal and nephroprotective benefit.


2019 ◽  
Vol 78 (11) ◽  
pp. 1576-1582 ◽  
Author(s):  
Gabriele Valentini ◽  
Dörte Huscher ◽  
Antonella Riccardi ◽  
Serena Fasano ◽  
Rosaria Irace ◽  
...  

ObjectivesTo investigate the influence of vasodilator drugs on the occurrence of features depending on myocardial ischaemia/fibrosis (ventricular arrhythmias, Q waves, cardiac blocks, pacemaker implantation, left ventricular ejection fraction (LVEF) <55%, and/or congestive heart failure and sudden cardiac death) in systemic sclerosis (SSc).Methods601 patients with SSc were enrolled from 1 December 2012 to 30 November 2015 and had a second visit 0.5–4 years apart. 153 received no vasodilators; 448 received vasodilator therapy (ie, calcium channel blockers and/or ACE inhibitors or angiotensin II receptor blockers or combinations of them), 89 of them being also treated with either endothelin receptor antagonists or PDE5 inhibitors or prostanoids. Associations between the occurrence of myocardial disease manifestations and any demographic, disease and therapeutic aspect were investigated by Cox regression analysis. A Cox frailty survival model with centre of enrolment as random effect was performed.ResultsDuring 914 follow-up patient-years, 12 ventricular arrhythmias, 5 Q waves, 40 cardiac blocks, 6 pacemaker implantations and 19 reduced LVEF and/or congestive heart failure (CHF) occurred. In multivariate Cox regression analysis, vasodilator therapy was associated with a lower incidence of ventricular arrhythmias (p=0.03); low-dose acetylsalicylic acid (ASA) with a lower incidence of cardiac blocks and/or Q waves and/or pacemaker implantation (p=0.02); active disease with a higher incidence of LVEF <55% and/or CHF and cardiac blocks and/or Q waves and/or pacemaker implantation (p=0.05).ConclusionsThe present study might suggest a preventative effect on the occurrence of distinct myocardial manifestations by vasodilator therapy and low-dose ASA.


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