ABC | Volume 111, Nº6, December 2018

Case Report Myocarditis with Cardiogenic Shock as the First Manifestation of Systemic Lupus Erythematosus Jáder Buzati Rebelato, Caroline Ferreira da Silva Mazeto Pupo da Silveira, Tainá Fabri Carneiro Valadão, Fabrício Moreira Reis, Rodrigo Bazan, Silméia Garcia Zanati Bazan Faculdade de Medicina de Botucatu (UNESP), Botucatu, SP – Brazil Mailing Address: Silméia Garcia Zanati Bazan • Faculdade de Medicina de Botucatu - Unesp - Departamento de Clínica Médica - Distrito de Rubião Jr, s/n. Postal Code 18618-687, Botucatu, SP – Brazil E-mail: sgzanati@fmb.unesp.br, sgzanati@cardiol.br Manuscript received February 20, 2018, revised manuscript May 03, 2018, accepted May 09, 2018 Keywords Myocarditis; Shock, Cardiogenic; Lupus Erythematosus, System; Heart Failure; Echocardiography. DOI: 10.5935/abc.20180216 Introduction Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with multisystemic and autoimmune characteristics. It is the most common systemic autoimmune disease, occurring mainly in women between 20 and 40 years old, with a female-to-male ratio of 10:1. Even though the kidneys are classically considered the main organ affected by SLE, cardiomyopathy is one of the complications more frequently associated with morbidity and mortality in SLE patients. 1 Cardiovascular impairment can be highly variable in terms of the affected structures and, in severe cases, may lead to cardiogenic shock. In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) published new criteria for SLE classification, aiming to optimize the diagnosis of cardiovascular impairment. However, cardiovascular disturbances are not part of the SLICC, even though there is such a high prevalence of cardiovascular disturbances in this population. 2 Case report A 30-year-old Caucasian woman with a three-year history of arterial hypertension, who was an irregular user of captopril, sought medical attention due to a one-week history of dyspnea and chest pain. The patient presented with cold and clammy skin, dyspnea, hypotension, and tachycardia and was afebrile. A resting electrocardiogram (ECG) showed ST-segment elevation in all derivations. She was admitted for thrombolysis with streptokinase at the original hospital and was then transferred to the Tertiary Clinical Hospital. The patient was admitted to our emergency department on mechanic ventilation and was hemodynamically unstable and receiving norepinephrine. A chest X-ray revealed cardiomegaly and pulmonary congestion; a transthoracic echocardiogram showed mild to moderate pericardial effusion, with diffuse hypokinesia of the left ventricle and significant systolic impairment with a left ventricular ejection fraction of 30%, as determined by the Teichholz method; the coronary angiography did not show any coronary lesions. Cardiac enzymes such as troponin and CKMB were elevated. There was no recent history of infection. Additionally, blood cultures were negative three times, and serology for HIV was nonreactive. The patient was diagnosed with myopericarditis, and hemodynamic support was provided with dobutamine, norepinephrine, and an intra-aortic balloon pump (IABP). Later, on the tenth day of hospitalization, the patient also showed signs of knee arthritis, altered consciousness and anisocoria. A computed tomography scan of the brain demonstrated multiple areas of cortical and subcortical hypodensity (Figure 1) and a brain arteriography showed a vasculitis pattern in the cerebral arteries. Antinuclear (ANA) and anti-DNA antibody tests were positive. After the diagnosis of lupus myocarditis was made, on the twelfth day of hospitalization, the patient was started on immunosuppressive therapy with methylprednisolone (1 g intravenously once daily for three consecutive days) and later with cyclophosphamide (0.6 g/m 2 intravenously once a month). There was significant clinical improvement, and a repeated transthoracic echocardiogram showed complete resolution of all changes. The patient remained asymptomatic, and on the twenty-eighth day was discharged from the hospital for outpatient clinical follow-up on 25 mg of captopril twice daily, 30 mg of diltiazem twice daily, 20 mg of omeprazole once daily, 70 mg of prednisone once daily and 250 mg of chloroquine once daily. Discussion SLE is a chronic inflammatory multisystemic autoimmune disease with complex characteristics that affects mainly women, of which onset usually occurs between the ages of 16 and 55 years-old; it has a variable frequency in the general population, with an incidence of 1:200 in black women. 1 Recently, the diagnostic criteria for SLE, collectively called the SLICC, have been revised and increased to a total of 17 criteria, from the 11 criteria of the previous 1997 classification. 2 To diagnose SLE according to the new recommendations, four or more criteria must be met, and at least one must be clinical, whereas one must be immunological. 1 In our patient, the diagnosis was confirmed due to the presence of serositis, neurological symptoms, and positive ANA and anti-DNA antibody testing (Table 1). Although cardiovascular impairment is very common in patients with SLE, with a prevalence of up to 40-50% in 864