Lupus Myocarditis Research Articles

Manifestação rara de edema agudo de pulmão associado à miocardite lúpica aguda

Systemic Lupus Erythematosus (SLE) is the most common systemic autoimmune disease, occurring more frequently in women, usually aged between 16 and 55 years 1, 2. Although classically the kidneys are the organs most affected in SLE, cardiopulmonary circulation and the heart may also be affected significantly 3. In this context, the occurrence of acute pulmonary edema associated with lupus myocarditis is rare and specific immunosuppressive therapy remains unclear.

Lupus Myocarditis Presenting as Acute Severe Heart Failure: Two Cases with Different Clinical Courses

Systemic lupus erythematosus (SLE) is an autoimmune disease that affects many organ systems including the heart. Of them, lupus myocarditis is rarely documented as an initial disease, and this complicated state is fatal. We recently encountered 2 cases that manifested severe heart failure (HF) as a first symptom. Both cases revealed severe left ventricular (LV) systolic dysfunction (LV ejection fraction: 20%) and required assist devices for refractory HF. In case 1, the symptoms and LV systolic function dramatically improved after corticosteroid therapy.

Acute lupus myocarditis: Clinical features and outcome

Cardiomyopathy in systemic lupus erythematosus (SLE) may be secondary to myocardial inflammation (i.e. myocarditis) or to systemic complications such as hypertension. Symptomatic left ventricular dysfunction is the most common clinical presentation of cardiomyopathy and is potentially life threatening. Identifying the cause is critical as it dictates therapy. We present three cases of left ventricular failure suggestive of myocarditis in SLE patients followed in the Lupus Clinic of the Montreal General Hospital over a 5-year period. The most frequent presentation is acute onset of a marked reduction of the left ventricular ejection fraction (LVEF). All patients were treated with cardiac support, prednisone, and additional immunosuppressive medications. Improvement of symptoms and LVEF was observed in two of three patients. Myocarditis is a rare, but life-threatening, manifestation of SLE. With immunosuppressive medications and cardiovascular support, the long-term outcome is usually favorable.

Rituximab in childhood lupus myocarditis

Sir, Myocarditis is a life-threatening complication of systemic lupus erythematosus (SLE) [1]. Myocardial involvement demonstrable as reduction in left ventricular ejection fraction can be seen in 8–25% of patients with active SLE [2]. Symptomatic myocarditis, however, with signs of frank congestive heart failure is extremely uncommon especially in childhood lupus [3, 4]. We report a young girl with lupus myocarditis, who had severe congestive cardiac failure while on maximal immunosuppression. R, a 12-year-old girl, presented with complaints of polyarticular arthritis, fever, edema, and painless oral ulcers. She had pallor, pitting pedal edema, hypertension, and arthritis involving knee, ankle, and proximal interphalangeal joints. On investigation, urine examination revealed many red blood cells, and 24-h urine protein was 136 mg/m/h. Blood urea was 56 mg/dl and serum creatinine 1.4 mg/dl. Antinuclear antibody was 4+, diVuse pattern and antidsDNA antibody titer was 760 IU/ml (N < 55). Lupus anticoagulant was positive. Renal biopsy revealed class IV/V lupus nephritis. She was administered Wve daily methylprednisolone pulses (30 mg/kg/dose) followed by oral prednisolone (2 mg/kg/ day). She was also started on intravenous monthly cyclophosphamide pulses (750 mg/m/dose). Within a month, she was readmitted with fever, increasing edema, and respiratory distress. She had persistent disease activity, nonoliguric renal failure (creatinine 3.4 mg/dl), and hypertension. She was treated again with intravenous pulse methylprednisolone, and cyclophosphamide pulses were continued. With above treatment, renal functions normalized and blood pressure got controlled with antihypertensive medications—amlodipine (0.3 mg/kg/day) and carvedilol (0.6 mg/kg/day). Three months later, she presented with loose stools, vomiting for 2 days, and respiratory distress for a day. On examination, she had tachycardia, tachypnoea, and hypotension. She developed basal crepts, hepatomegaly, and gallop rhythm. For hemodynamic stability, she required inotropic support. Chest X-ray showed cardiomegaly (cardio thoracic ratio 64%) and bilateral basal inWltrates. Ventilation/perfusion (V/Q) scan was normal. Echocardiography showed dilated right and left ventricles, decreased myocardial contractility with left ventricular ejection fraction of only 30%. A clinical diagnosis of lupus myocarditis was made. As she had already received two courses of intravenous methylprednisolone and was on monthly cyclophosphamide pulses, a decision was made to start her on rituximab (375 mg/m/dose). She showed prompt clinical recovery, and we were able to taper oV her inotropes. The gallop disappeared. Repeat echocardiogram done after 1 week showed ejection fraction of 50%. She was treated with weekly doses of rituximab (375 mg/m/dose) for 4 weeks. Cyclophosphamide was given every month for 6 months and thereafter every 3 months. After 12 months of follow-up patient, she is asymptomatic and on tapering doses of oral prednisolone along with hydroxychloroquine. Cardiac involvement represents one of the most important clinical manifestations of SLE and contributes signiWcantly to the morbidity and P. Aggarwal · S. Singh (&) · D. Suri · A. Rawat Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India e-mail: surjitsinghpgi@rediffmail.com

Successful treatment of chronic lupus myocarditis with prednisolone and mizoribine

A 36-year-old female patient who was diagnosed with chronic myocarditis as an initial manifestation of systemic lupus erythematosus (SLE) was admitted to our hospital. At her third occurrence of heart failure, we performed an endomyocardial biopsy and proved chronic myocarditis with SLE. Subsequently, she was treated with prednisolone and the immunosuppressive agent mizoribine (MZR), and her cardiac function improved. We describe for the first time treatment with MZR for chronic cardiac involvement of SLE.

Hemophagocytic syndrome as one of the main primary manifestations in acute systemic lupus erythematosus - case report and literature review

Hemophagocytic syndrome is an unusual but fatal disorder characterized by pancytopenia and activation of macrophages. We describe one case of acute systemic lupus erythematosus with an unusual presentation of hemophagocytic syndrome not related to infection. The patient presented with pancytopenia related to increasing hemophagocytic activity of histiocytes in the bone marrow. Concomitant class IV World Health Organization lupus nephritis, serositis, high titer of antinuclear factor and positive test for anti-DNA antibody fitted the diagnostic criteria of systemic lupus erythematosus. She also presented with alveolar hemorrhage and lupus myocarditis. She underwent immunosuppressive therapy with recovery from the hemophagocytic syndrome. Therefore, diagnosis of acute lupus hemophagocytic syndrome was made. The clinical presentation, laboratory diagnosis, and management of the patient are discussed and the literature was reviewed and presented, with emphasis on a possible distinct lupus subset, which includes a more aggressive systemic disease with heart involvement.

Lupus myocarditis: marked improvement in cardiac function after intravenous immunoglobulin therapy

Intravenous immunoglobulin (IVIg) is emerging as the mainstay in the treatment of many autoimmune diseases, including systemic lupus erythematosus. IVIg has been found to be beneficial in myocarditis due to dermatomyositis/polymyositis, Kawasaki disease, and viral myocarditis in children. We report an 18-year-old man of active lupus with worsening cardiac systolic function who did not respond to pulse methylprednisolone and cyclophosphamide, but subsequently showed an improvement in his cardiac function after IVIg administration.

Lupus Myocarditis Presenting as Acute Congestive Heart Failure: A Case Report

A young woman who had a delivery history 3 months previously presented with dyspnea and orthopnea. Initial findings of physical examination, chest radiography, and echocardiogram showed typical congestive heart failure with severe left ventricular (LV) dysfunction. At first, we considered peripartum cardiomyopathy because she had given birth to a baby 3 months previously. However, even though we massively tried conventional drug therapy for 10 days, the patient still remained with refractory heart failure. We performed additional laboratory studies such as complement level and autoantibodies, of which the results supported systemic lupus erythematosus. We could make the diagnosis of acute lupus myocarditis and treated her with corticosteroid. The symptoms were dramatically disappeared and LV function also improved.

A 32‐year‐old woman with arthralgias and severe hypotension

A 32‐year‐old woman with arthralgias and severe hypotension

A Case of Pneumomediastinum in the Course of Treatment in Lupus Myocarditis

Systemic lupus erythematous (SLE) is systemic autoimmune disease of unknown etiology. SLE involve all part of heart but clinically important myocarditis is an unusual feature and is potentially fatal complication of SLE. We describe the woman who had diagnosed as SLE, 5 years ago and in that time, she had sinus tachycardia, mild dyspnea, chest discomfort, and depressed left ventricular function. She was diagnosed to myocarditis clinically and was treated by high-dose corticosteroids of intravenous pulse methylprednisolone. After treatment, she improved and showed improvement of left ventricular function. However, a sudden pneumomediastinum was occurred without trauma, and she died. The cause of pneumomediastinum was unexplained. We report a sudden pneumomediastinum in the course of lupus myocarditis presenting as sinus tachycardia with review of literature.

Clinical characteristics of lupus myocarditis in Korea

Clinically important myocarditis is an unusual feature in systemic lupus erythematosus (SLE). We describe the clinical characteristics, management and outcomes of five SLE patients who developed severe left ventricular dysfunction. Four patients were female with mean age of 36.4 years. Three patients had both lupus myocarditis and lupus nephritis. Four patients had raised anti-dsDNA antibody titer and low complement level and two patients had positive IgG anticardiolipin antibody. Three patients were treated by high-dose corticosteroids, one patient by intravenous pulse methylprednisolone, and one patient by intravenous immunoglobulin and pulse cyclophosphamide with high dose corticosteroids. Left ventricular function improved markedly in four patients and all of them had no recurrence of lupus myocarditis up to follow-up of 33 months. However, one patient, who showed no improvement of left ventricular function, was expired due to sudden cardiac arrest. Lupus myocarditis should be treated by immunosuppressive therapy with high-dose corticosteroids and mostly the prognosis might be good with early treatment.

Systemic Lupus Erythematosus Presenting as Acute Lupus Myocarditis

Systemic lupus erythematous (SLE) is an autoimmune inflammatory disease of unknown etiology which affects various parts of body. SLE can involve all parts of the heart including the pericardium, myocardium, endocardium, heart valves and coronary arteries. Cardiopathy of SLE is the third common cause of death in all patients with SLE. Although cardiopulmonary symptoms are common in SLE, symptomatic acute lupus myocarditis is a very rare and fatal complication of SLE. We report here on a 20-year-old female patient with acute myocarditis as a initial manifestation of SLE and rapidly diagnosed using echocardiogram.

LUPUS MYOCARDITIS TREATED WITH INTRAVENOUS IMMUNOGLOBULIN UNDER HEMODYNAMIC MONITORING

INTRODUCTION: Systemic lupus erythematosus is a systemic autoimmune disease that affects numerous organ systems and can affect any part of heart. Few case reports have demonstrated a beneficial effect of intravenous immunoglobulin (IVIG) therapy in lupus myocarditis. We present a case of severe lupus myocarditis treated with IVIG under hemodynamic monitoring.

Utility of cardiac MRI for diagnosis and post-treatment follow-up of lupus myocarditis

Clinical myocardial involvement in systemic lupus erythematosus is rare. Lupus myocarditis is usually not detected until significant decrease in myocardial function becomes clinically evident. This case report describes MR imaging of lupus myocarditis that was used for diagnosis and follow up in a patient who declined cardiac biopsy. We also review the literature related to myocardial imaging by MRI.

Acute lupus myocarditis: clinical features and outcome of an oriental case series

Symptomatic myocarditis in systemic lupus erythematosus (SLE) is uncommon. We describe the clinical characteristics, management and outcomes of 11 SLE patients without any atherosclerotic risk factors, who presented with acute lupus myocarditis (ALM). All patients were female, 46% Chinese with mean age of 27 +/- 10 years at diagnosis of SLE. ALM was one of the initial manifestations of SLE in eight (73%) patients. The median duration from onset ALM to initiation of treatment was two weeks (range: 0.3-8). All had clinical feature of left ventricle dysfunction. The most common electrocardiographic feature was nonspecific ST/T wave changes (91%). Common echocardiographic findings included segmental wall motion abnormalities (81%) and decreased left ventricular ejection fraction (81%). Median SLE disease activity index at presentation was 16 (range: 4-30). All patients received high dose corticosteroids and 64% received intravenous pulse cyclophosphamide. There were two deaths (18%) from infections. The remaining nine survivors had no recurrence of ALM nor suffer any SLE-related damage (median SLICC damage score of 0), up to a median follow-up of four years (range: 2.5-10.1). Repeat echocardiography after six months or later showed normal LVEF in eight patients (89%). Early immunosuppressive therapy in ALM, with high dose corticosteroids and pulse intravenous cyclophosphamide, results in good cardiac outcome.

Giant cell myocarditis: a rare cardiovascular manifestation in a patient with systemic lupus erythematosus

Giant cell myocarditis (GCM) is a rare form of myocarditis with a median survival of less than one year. It has been reported to occur in patients with various underlying autoimmune diseases; however, no cases of GCM have been described in patients with clear evidence of underlying systemic lupus erythematosus (SLE). The presentation of GCM may mimic that of lupus myocarditis, including an initial response to immunosuppression. Despite initial clinical similarities, lupus myocarditis and GCM are histologically distinct entities with dramatic differences in prognosis. We report herein a patient with a longstanding history of SLE, who presented acutely with myocarditis, responded well to initial immunosuppression and then subsequently died of progressive heart failure that was found to be due to GCM. Endomyocardial biopsy can help define diagnosis and prognosis of lupus patients presenting with myocarditis, and early referral for cardiac transplantation should be considered in patients diagnosed with GCM.

Intravenous cyclophosphamide improves cardiac dysfunction in lupus myocarditis

We describe a Chinese woman who developed severe heart failure 3 years from the onset of systemic lupus erythematosus (SLE). Endomyocardial biopsy confirmed lupus myocarditis, with focal infiltrates of small lymphocytes and some polymorphic neutrophils. The conventional treatment for cardiac failure plus oral prednisolone failed to bring clinical and echocardiographical improvement until the addition of intravenous (i.v.) ‘pulse’ cyclophosphamide. Three weeks after i.v. cyclophosphamide treatment, there was significant improvement of her heart failure symptoms with improvement in the ejection fraction from 19% to 63%.

A Case of Superimposed Viral Myocarditis in a Patient with Systemic Lupus Erythematosus

Lupus myocarditis is usually treated using immunosuppressive agents, such as high-dose corticosteroids, azathioprine and cyclophosphamide. Viral myocarditis and enteroviruses have been identified as the most common causative agents of myocarditis in lupus patients. Although immunosuppressive therapy has an important role in the treatment of lupus myocarditis, it is not recommended in patients with infectious or post-infectious viral myocarditis, and supportive care is very important in these patients. A 25-year old female patient, with systemic lupus erythematosus, was admitted due to severe dyspnea, and diagnosed as having heart failure. She recovered 7 days after supportive care for heart failure, without Immuno-suppression. Her sera neutralized coxsackievirus B3 (CVB3) in neutralization test, with the horse anti-CVB3 antibody (Ab, ATCC V030-501-560) used as a positive control. The titers for the neutralizing Ab in her sera were 4 times higher than that of the standard control ATCC Ab. (Korean Circulation J 2003;33 (12):1147-1150)

Steroid responsive cardiomyopathy

Heart disease responsive to steroids is well described in many disorders, including sarcoidosis, systemic lupus erythematosus, polyarteritis nodosa, myocarditis and Churg-Strauss syndrome. The underlying disorder is oftern obvious and the response is usually slow. We describe a woman who had severe left ventricular failure, cardiac dilatation and pericardial effusion which were rapidly rectified by steroid therapy. Steroid withdrawal led to recurrence of signs, which were reversed by recommencing steroids. The aetiology was not determined.

Myocarditis of Lupus Erythematosus

Letters and Corrections1 March 1979Myocarditis of Lupus ErythematosusMURRAY A. VARAT, M.D.MURRAY A. VARAT, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-90-3-438 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptTo the editor: The association between lupus myositis and myocarditis reported by Borenstein and co-workers in the November issue (1) is very interesting. I wish to comment on the significance of the serum creatine kinase (CK)-cardiac (MB) isoenzyme elevation in their patient and on their suggestion that CK isoenzyme determination may be of value in the diagnosis of lupus myocarditis.A small amount of CK-MB is found in skeletal muscle by tissue analyses (2). In addition, in healthy control subjects, up to 2% of serum CK may be in the MB form (3). Therefore, the total CK of 1730 U/litre...