Case Presentation: 59 year old woman with a history of recently diagnosed rheumatoid arthritis (RA) not on therapy presents with substernal non radiating chest pain and right upper quadrant pain worsening with inspiration and dyspnea on exertion (DOE) for one week. Patient also reported dysphagia, dry mouth and Raynaud’s phenomenon. Exam showed normal vital signs and decreased heart sounds. Patient had skin tightening around the mouth, hand and forearms, sclerodactyly, scattered telangiectasias over the chest and nailfold capillary changes. Initial EKG showed low voltage QRS complexes and T-wave abnormalities in V1-V3. Lab values revealed mild leukocytosis, microcytic anemia and mild transaminitis. Initial cardiac enzymes showed CK 1289, CKMB 41.7, CKMB relative index 3.2, high sensitivity troponin 225. Patient was admitted to telemetry for a possible NSTEMI and started ACS protocol. Cardiac catherization showed clean coronaries. Echocardiogram showed a small pericardial effusion and normal ejection fraction. Despite ACS protocol, patient’s chest pain and shortness of breath persisted. Rheumatology was consulted to evaluate for possible autoimmune myocarditis. Labs showed ANA 1:1280, SSA 0.6, SSB <0.2, Centromere Ab <0.2, dsDNA <12, CCP >250, RF >27, CRP 55.8. Cardiac MRI (CMR) showed a 7-11mm pericardial effusion with evidence of paradoxical septal motion of the interventricular septum concerning for constrictive pericarditis without signs of myocarditis.
Given the clinical picture, patient was diagnosed with autoimmune myocarditis and constrictive pericarditis due to systemic sclerosis (SSc). Patient was started on methylprednisolone 50mg IV daily for three days with resultant downtrending cardiac enzymes and resolution of chest pain and DOE within 24 hours. She was subsequently discharged home on daily Prednisone. Three months later patient remained asymptomatic on tapering dose of corticosteroids and repeat CMR showed resolution of pericarditis.
Discussion: Although our patient had a previous diagnosis of RA, her clinical picture is consistent with SSc based on the American College of Rheumatology guidelines. SSc is a systemic disease which can cause a wide range of organ involvement including cardiac disorders such as symptomatic pericarditis (7-20%), cardiac fibrosis, acute myocardial infarction and arrhythmias. However, myocarditis is a rare entity for SSc. Although the patient’s CMR was negative for myocarditis, the diagnosis can be made in setting of chest pain, EKG findings and elevated cardiac markers in the absence of CAD or pre-existing cardiac conditions based on the European Society of Cardiology (ESC) 2013 guidelines. Symptoms of myocarditis are non-specific including chest pain, shortness of breath and fatigue. Often patients present with elevated cardiac enzymes leading to the initial diagnosis of acute coronary syndrome as seen in our patient. In all suspected cases of myocarditis, it is necessary to exclude CAD as stated in the ESC guidelines. In patients being evaluated for myocarditis, EKG, ECHO, CMR, Troponin, and ESR/CRP should be obtained, however, gold standard for diagnosis is an endocardial muscle biopsy. Treatment includes corticosteroids and other immunosuppressive agents.
Conclusions: In patients presenting with chest pain and positive cardiac enzymes with a negative coronary angiogram, other etiologies of myocardial injury should be considered such as autoimmune and viral processes especially in the patients with underlying rheumatologic disorders.