Noninfective Endocarditis in Rheumatoid Arthritis

Abstract

Noninfective endocarditis with inflammatory or thrombotic vegetations is typically described in patients with systemic lupus erythematosis (SLE) and primary antiphospholipid syndrome. This case describes a patient with rheumatoid arthritis, noninfective endocarditis and recurrent embolic events, and is remarkable for its rarity and significant regression of valvular heart disease with anticoagulation and disease-modifying anti-rheumatic drugs (DMARDs). A 52-year-old woman with severe, active rheumatoid arthritis presented with acute aphasia and right-sided hemiparesis. Her medications included Methotrexate, Hydroxychloroquine, Prednisone, and recently, Infliximab. On presentation, she was afebrile and had severe and diffuse joint inflammation, right hemiparesis with ipsilateral facial droop, and expressive aphasia. No cardiovascular abnormalities were noted. She received recombinant tissue plasminogen activator (rTPA) and her neurologic symptoms improved. A brain magnetic resonance imaging (MRI) study showed an acute left frontoparietal infarction and an old right parietal lobe infarction (Figure). Laboratory workup revealed negative blood cultures for bacteria, fungi, and atypical microorganisms; elevated C-reactive protein (CRP) of 4.3 mg/dL (normal 0-0.8), but normal erythrocyte sedimentation rate (ESR) of 28 mm/h (normal 0-30 mm/h); low Protein C of 14% (normal 70% to 151%); and mildly elevated anticardiolipin IgA at 22 IU/mL (normal 1-19 IU/mL). Electrocardiography demonstrated normal sinus rhythm, and carotid Dopplers showed no disease. A transesophageal echocardiogram (TEE) demonstrated thickening with soft tissue echoreflectance of the mitral and aortic valves. Additionally, 2 hyper-mobile, multilobed mitral valve masses were visualized, each measuring 10×15 mm. These masses were elongated with irregular borders and of soft-tissue echoreflectance. Only mild mitral regurgitation was demonstrated. Noninfective endocarditis with thrombotic or inflammatory vegetations due to rheumatoid arthritis was deemed most likely. Therefore, Warfarin was added to Methotrexate, Hydroxychloroquine, and Prednisone. Etanercept was initiated in lieu of Infliximab for unclear reasons. Two months later, she presented with acute aphasia despite a therapeutic international normalized ratio of 3.0 and stable rheumatoid arthritis. An MRI study showed interval development of an acute cerebral infarct around the left previous infarct margins. The cardiovascular examination was still unremarkable. Laboratory abnormalities included a CRP of 4.3 mg/dL, ESR of 66 mm/h, and anticardiolipin IgA of 24 IU/mL. A repeat TEE showed improved thickening of the mitral and aortic leaflets and now a single, smaller mass on the mitral valve. Cardiothoracic Surgery declined the patient for valve replacement. She was continued on Methotrexate, Prednisone, Hydroxychloroquine, Warfarin, and Etanercept with improvement of her clinical syndrome leading to discharge in stable condition. A follow-up TEE 6 months later showed improvement in mitral and aortic valve thickening and the single mitral valve mass was significantly smaller. This is a unique case of rheumatoid arthritis-associated endocarditis with noninfective vegetations complicated by recurrent embolic events. This case is exceptional due to the significant improvement in the patient’s clinical course and valvular heart disease with DMARDs and anticoagulation, which has not previously been demonstrated.1Gonzalez-Juanatey C. Garcia-Porrua C. Testa A. Gonzalez-Gay M.A. Potential role of mitral valve strands on stroke recurrence in rheumatoid arthritis.Arthritis Rheum. 2003; 49: 866-868Crossref PubMed Google Scholar, 2Kang H. Baron M. Embolic complications of a mitral valve rheumatoid nodule.J Rheumatol. 2004; 31: 1001-1003PubMed Google Scholar This case illustrates that patients with rheumatoid arthritis can develop noninfective inflammatory or thrombotic valve vegetations and that embolism from valvular heart disease should be considered as a cause of stroke or transient ischemic attacks in these patients. Additionally, DMARDs and anticoagulation may be a reasonable therapeutic alternative for noninfective endocarditis in patients posing a high surgical risk.