ABC | Volume 111, Nº6, December 2018

Case Report Rebelato et al Lupus myocarditis with cardiogenic shock Arq Bras Cardiol. 2018; 111(6):864-866 Figure 1 – Computed tomography of the brain showing, in both A and B panels, hypodensity areas compatible with lacunar infarcts caused by vasculitis. Table 1 – Clinical and immunological criteria of the SLICC (Petri et al. 2012) 2 CLINICAL CRITERIA IMMUNOLOGICAL CRITERIA 1. Acute Cutaneous Lupus 1. ANA 2. Chronic Cutaneous Lupus 2. Anti-dsDNA 3. Oral ulcers 3. Anti-Sm 4. Nonscarring alopecia 4. Antiphospholipid Antibody 5. Synovitis involving >2 joints 5. Low Complement 6. Serositis 6. Direct Coombs Test 7. Renal manifestations 8. Neurological Manifestations 9. Hemolytic anemia 10. Leukopenia/Lymphopenia 11. Thrombocytopenia postmortem studies, it is not part of the new diagnostic criteria; it is considered only associated damage due to long‑term disease. 1-5 It may manifest as pericarditis, myocarditis, Libman-Sacks endocarditis, pulmonary arterial hypertension or coronary artery disease; coronary artery disease is the most prevalent one, due to the inflammatory process of the disease itself together with the use of corticosteroids, which are commonly employed in the treatment of lupus. 6 Due to the several impairment sites, the clinical manifestations may be quite variable and may range from asymptomatic or oligosymptomatic to cardiogenic shock, in the most severe cases of myocarditis. In general, patients with lupus myocarditis are usually asymptomatic, with symptoms present in only approximately 5 to 10% of patients. 3 However, severe heart failure may be the first manifestation of the disease. Cardiogenic shock in lupus patients may have several etiologies, such as coronary artery disease, drug-induced cardiotoxicity (e.g., antimalarial drugs), pericarditis with cardiac tamponade, and valvular insufficiency secondary to valvular destruction, among other causes. 6 A definitive diagnosis is made through anatomopathological analysis of an endomyocardial biopsy, which is not necessary in most cases. The endomyocardial biopsy has low sensitivity since the myocardial pattern may be focal in many situations. 5 Thus, clinical suspicion combined with epidemiology, individual history and symptoms continue to be essential for diagnosis. Inflammatory markers associated with the disease may be elevated in cases of myocarditis, along with reduction in serum complement levels. Among all the markers, the presence of anti-DNA antibodies has been associated with lupus myocarditis. 3 An elevation in myocardial necrosis markers can occur; however, it is not related to clinical severity. 7,8 The treatment of cardiogenic shock secondary to SLE begins with the same supportive treatment that is usually employed for patients with severe heart failure, regardless of the etiology. 2,4,5 Thus, patients are usually started on inotropic drugs, vasodilators and vasopressors, and in patients who are refractory to the conventional clinical approach, mechanical support is required. The most common mechanical support, partly due to its availability, is an intra-aortic balloon pump; however, new devices for circulatory assistance may be used based on need. Specific treatments for patients with severe left ventricular dysfunction associated with lupus myocarditis include high‑dose corticosteroids; in some situations, such as in this patient, this involves pulse therapy with methylprednisolone, and other immunosuppressants (cyclophosphamide, azathioprine) or immunoglobuolins. 2,3,5,7 However, the currently used treatments are not supported by scientific findings from controlled studies, due to the difficulty in performing such studies because of the rarity of this kind of presentation. An early and precise diagnosis allows the implementation of an aggressive treatment of lupus myocarditis and leads to better outcomes, including the resolution of left ventricular systolic 865