we report a case of sle - associated myopericarditis in a young male without clear evidence of viral infection based on viral markers in blood .
the patient 's cardiac function dramatically improved after treatment with steroids without any additional complications .
a 19-year - old male was admitted to the combined armed forces hospital with a 7-day history of fever , cough , dyspnea , orthopnea , and chest pain .
based on the chest radiograph and computed tomography , he was diagnosed with a pericardial effusion and pneumonia ( fig .
1a ) , and was transferred to our hospital for evaluation of the cause and treatment .
his blood pressure was 110/70 mmhg , pulse rate was 112 beats / min , respiratory rate was 24 breaths / min , and body temperature was 38. jugular veins were engorged . on cardiac auscultation , the cardiac rhythm was regular and rapid , summation gallops were heard at the cardiac apex , and there were pericardial friction rubs along with left lower sternal border in the sitting position .
bilateral pretibial pitting edema was present on admission . on admission , blood tests showed white blood cell count of 5,420/mm , hemoglobin 10.6 g / dl and platelet count of 94,000/mm .
c - reactive protein was 4.59 mg / l ( normal , 0 - 3 mg / l ) .
the blood chemistry revealed blood urea nitrogen to be 24.0 mg / dl , creatinine 1.1 mg / dl , total protein 5.7 g / dl , and albumin 2.6 g / dl .
the levels of cardiac markers were elevated ; troponin - i 0.87 ng / ml ( normal , 0 - 0.05 ng / ml ) and myoglobin 371 ng / dl ( normal , 16.3 - 96.5 ng / dl ) .
an electrocardiogram ( ecg ) revealed sinus tachycardia and diffuse t - wave inversion in which leads ( fig .
echocardiography demonstrated severe left ventricular systolic dysfunction { left ventricular ejection fraction ( lvef ) was 18% } with severe global hypokinesia and preserved wall thickness ( fig .
a large amount of pericardial effusion was observed 13 mm anterior to the right ventricle , 18 mm around the right atrium and 2 mm posterior to the lv .
at first , we suspected viral myopericarditis , and started conservative treatment for congestive heart failure and pericarditis .
but , there was no improvement in lvef as well as in the clinical findings . viral markers for cytomegalovirus , coxsackie virus b type 2 , herpes simplex virus , and epstein - barr virus were all negative . during conservative treatment
, he complained of new - onset ankle joint pain and tender erythematous swellings in both the ankles .
the immunofluorescence tests were positive for anti - nuclear antibody ( 1 : 640 titre ) , anti - dsdna antibodies ( 683.4 iu / ml ) , and anti - extractable nuclear antigen antibodies ( anti - sm , anti - rnp , anti - ro , and anti - la ) , and the complement level was low ( table 1 ) .
we concluded that the patient had sle according to the american rheumatism association / american college of rheumatology classification criteria for sle . on the 13th day of admission
, we started treatment with high - dose glucocorticoids ( methylprednisolone 1,000 mg intravenously daily for three days followed by 1 mg / kg per day in divided doses ) . on follow - up examination , ecg showed normalization of t - wave inversion ( fig . 2c ) and echocardiography obtained just before discharge showed improved systolic function ( fig .
chest x - ray also showed improving consolidation in both the lung fields and cardiomegaly ( fig .
complement 3 was normalized and anti - dsdna antibodies decreased from 683.4 iu / ml to 383.8 iu / ml ( who u / ml , normal 0 - 93 ) .
he was discharged on the 33rd day of admission with oral prednisolone , and he visited the outpatient department 1 month later . in this 1 month , he had no symptoms .
echocardiography revealed normal lvef , without significant valvular disease and pericardial effusion compared to the last examination ( table 2 ) .
subsequently , the steroid medication was tapered and it was planned to maintain him on sle - specific treatment in the rheumatologic department .
sle is an autoimmune inflammatory disease of unknown etiology that affects various parts of the body , including all components of the cardiovascular system.1 ) the prevalence of lupus ranges from approximately 40 - 150 cases per 100,000 in the usa.2 ) in adults , the female - to - male ratio is 7 - 15 : 1.3 ) cardiac involvement in sle may comprise of involvement of the pericardium , myocardium , endocardium , heart valves , and coronary or pulmonary arteries.4 ) pericardial involvement is the most frequent cause of symptomatic cardiac disease5 ) and is the most common echocardiographic finding in sle patients .
the pericardium can be involved by acute and chronic inflammatory changes ; granular deposition of immunoglobulin and c3 , demonstrated by direct immunofluorescence,6 ) supporting the role of immune complexes in the development of pericarditis .
myocarditis is an uncommon , often asymptomatic manifestation of sle with a prevalence of 8 - 25% in different studies.7 ) global hypokinesis may be an echocardiographic indication of myocarditis and is present in approximately 6% of sle patients.8 ) the gold standard for diagnosing myocarditis in sle remains the endomyocardial biopsy.9 ) however , an endomyocardial biopsy is invasive with procedure - related risks and the diagnostic yield is low in 10 - 20% of cases , so it is not powerful enough to confirm the diagnosis.10 ) lupus myocarditis can be diagnosed based on clinical suspicion and echocardiographic evidence of a reduced lvef and segmental or global wall motion abnormality , once the other etiologies , such as viral and ischemic cardiomyopathy , have been excluded.9)11 ) immunofluorescence studies demonstrate fine granular immune complexes and complement deposition in the walls and perivascular tissues of myocardial blood vessels , supporting the hypothesis that lupus myocarditis is an immune complex - mediated disease .
lupus myocarditis , although mild , has to be treated immediately with high - dose corticosteroids . in the most severe forms ,
it is necessary to use intravenous pulse corticosteroid therapy ( methylprednisolone 1 g / day for three consecutive days ) followed by high doses of oral prednisone .
immunosuppressants , namely cyclophosphamide or azathioprine , and intravenous igg may be beneficial in the treatment of myocarditis.5 ) early mechanical circulatory supports help to save the life and prevent multi - organ failures in patients with fulminant myocarditis.12 ) in the present case , the patient was initially diagnosed with myopericarditis on the basis of his symptoms , diffuse t - wave inversion on ecg , elevated cardiac markers and echocardiographic findings .
however , there was no improvement in his symptoms and the common cardiotrophic viral markers were all negative . also , the patient had pericarditis , thrombocytopenia and proteinuria .
because the previous typical clinical signs and the positive immunofluorescence tests were suitable for making the diagnosis of sle , we confirmed the diagnosis of myopericaditis associated with sle in this patient .
after a provisional diagnosis of myopericarditis with sle was made , he was treated with intravenous corticosteroid pulse therapy ( methylprednisolone 1 g / day for three consecutive days ) followed by high doses of oral prednisone .
after 3 days of steroid therapy , the patient 's signs and symptoms were stabilizing and echocardiographic findings were improved .
initially , we had considered myopericarditis to be caused by viral infection ; however , the final diagnosis was myopericarditis during presentation of primary sle based on the clinical manifestations , echocardiography , immunofluorescence tests and good response to steroid therapy . because the treatment of sle induced myopericarditis
should be needed for some specific therapy such as corticosteroid , this case shows that careful investigation is needed for search of the cause of myopericarditis in a young male , and not only viral infection but also autoimmune disease should be investigated for . in this report
, we described the first case of a korean young male sle patient in whom the first manifestation was of myopericarditis and treatment with glucocorticoids resulted in a good clinical outcome .
 myocardial involvement with clinical symptoms is a rare manifestation of systemic lupus erythematosus ( sle ) , despite the relatively high prevalence of myocarditis at autopsies of sle patients . in this review , 
 we report the case of a 19-year - old male sle patient who initially presented with myopericarditis and was successfully treated with high dose of glucocorticoids . 