Venkataraj Maamannan, Pierotti Tyler, Sondhi Manush, Balmuri Shravya, Hayat Samina
Internal Medicine, Louisiana State University Health Sciences Center, Shreveport, USA.
Rheumatology, Louisiana State University Health Sciences Center, Shreveport, USA.
Cureus. 2023 Sep 23;15(9):e45805. doi: 10.7759/cureus.45805. eCollection 2023 Sep.
Cardiac sarcoidosis (CS) is a rare auto-immune disorder where immune cells form granulomas in the heart that may lead to potential arrhythmias and heart failure. Due to the low prevalence of CS, the management remains challenging, requiring a multidisciplinary approach. In addition to the management of the resulting arrhythmias and heart failure, corticosteroids and immunosuppressants are used as anti-inflammatories to prevent disease progression. Immunosuppressive regimens for the treatment of CS are not yet well established. Abatacept has been approved for rheumatoid arthritis and psoriatic arthritis and both are mainly Th1-driven autoimmune diseases. Even though there are several different drugs used to treat corticosteroid-dependent sarcoidosis, abatacept may represent a unique option as its side effects differ from other drugs like methotrexate, azathioprine, or mycophenolate, especially bone marrow and liver toxicity. We present the case of a 52-year-old CS patient treated with abatacept after the failure of methotrexate and mycophenolate mofetil. Our patient had a history of stage D heart failure with reduced ejection fraction (HFrEF) with ejection fraction (EF) of 15-20%, nonischemic cardiomyopathy (NICM) s/p left heart catheterization (LHC), CS diagnosed by positron emission tomography (PET), status post implantable cardioverter-defibrillator (ICD) implantation, lung sarcoid, paroxysmal atrial fibrillation, and aflutter, who followed with cardiology, rheumatology, and pulmonology. He had multiple admissions for heart failure exacerbations. The patient was initially diagnosed with pulmonary sarcoidosis after which he completed a small course of steroids. CT chest showed lymphadenopathy; however, endobronchial ultrasound (EBUS) did not show evidence of pulmonary sarcoidosis. During an admission for heart failure about four years later, cardiac PET CT showed CS, and rheumatology was brought on board. The patient initially refused steroids and steroid-sparing agents. At subsequent visits, the patient was amenable to medication and was started on methotrexate 10mg weekly. However, given worsening chronic kidney disorder, methotrexate was discontinued and mycophenolate 200mg daily was started. A couple of weeks after mycophenolate was started, the patient felt like "his throat was closing up" and his stomach was cramping, which was thought to be an allergic response to the mycophenolate so it was discontinued. He then received an abatacept infusion which he tolerated well. Currently, our patient has been referred for heart transplantation.
心脏结节病(CS)是一种罕见的自身免疫性疾病,免疫细胞在心脏中形成肉芽肿,可能导致潜在的心律失常和心力衰竭。由于CS的患病率较低,其管理仍然具有挑战性,需要多学科方法。除了处理由此产生的心律失常和心力衰竭外,还使用皮质类固醇和免疫抑制剂作为抗炎药来预防疾病进展。用于治疗CS的免疫抑制方案尚未完全确立。阿巴西普已被批准用于类风湿性关节炎和银屑病关节炎,这两种疾病主要是由Th1驱动的自身免疫性疾病。尽管有几种不同的药物用于治疗依赖皮质类固醇的结节病,但阿巴西普可能是一种独特的选择,因为它的副作用与甲氨蝶呤、硫唑嘌呤或霉酚酸酯等其他药物不同,尤其是骨髓和肝脏毒性。我们报告了一例52岁的CS患者,在甲氨蝶呤和霉酚酸酯治疗失败后接受阿巴西普治疗的病例。我们的患者有D期心力衰竭病史,射血分数降低(HFrEF),射血分数(EF)为15%-20%,非缺血性心肌病(NICM),经左心导管检查(LHC),通过正电子发射断层扫描(PET)诊断为CS,植入植入式心脏复律除颤器(ICD)后,患有肺部结节病、阵发性心房颤动和心房扑动,随访于心脏病学、风湿病学和肺病学。他因心力衰竭加重多次入院。患者最初被诊断为肺部结节病,之后完成了一个小疗程的类固醇治疗。胸部CT显示淋巴结病;然而,支气管内超声(EBUS)未显示肺部结节病的证据。大约四年后,在因心力衰竭入院期间,心脏PET CT显示为CS,于是请风湿病科会诊。患者最初拒绝使用类固醇和类固醇节约剂。在随后的就诊中,患者愿意接受药物治疗,开始每周服用10mg甲氨蝶呤。然而,由于慢性肾脏疾病恶化,停用了甲氨蝶呤,开始每天服用200mg霉酚酸酯。霉酚酸酯开始服用几周后,患者感觉“喉咙发紧”,胃部绞痛,这被认为是对霉酚酸酯的过敏反应,因此停用了该药。然后他接受了阿巴西普输注,耐受性良好。目前,我们的患者已被转诊进行心脏移植。