Lupus Myocarditis Research Articles

Dilated Cardiomyopathy in the Cardiology Department of the CHU of Casablanca: A One-Year Observational Study

Dilated Cardiomyopathy (DCM) is defined by a left ventricle (LV) with reduced systolic function (<45%) and dilation (LV volume > 90 ml/m²), excluding coronary artery disease or sufficient load conditions to explain it. The etiologies are multiple and increasingly well-known. We conducted a single-centre, retrospective observational study within the cardiology department of the CHU IBN ROCHD in Casablanca, Morocco, over one year. The main objective was to describe the characteristics and particularities of patients followed for DCM. The diagnosis is based only on echocardiography, after exclusion of an ischemic cause, severe valvular disease, and congenital heart disease. During the study, 44 patients were included, with a male predominance of 63.6% versus 36.4%. The average age was 60 ± 16.8 years. The initial presentation of cardiomyopathy showed that 73% of patients were admitted with heart failure (HF). The mean left ventricular ejection fraction (LVEF) was 30.2 ± 7.9%. Among the attributed etiologies, idiopathic DCM was the main cause reported in 59% of cases, followed by systemic lupus erythematosus (14%) and myocarditis (14%). In our sample, treatment was based on HF management, including beta-blockers, renin-angiotensin system blockers, SGLT2 inhibitors, and aldosterone antagonists. Implantation of a defibrillator for primary prevention concerned only one patient (3%) of the 33 patients for whom the theoretical indication was retained.

Lupus Myocarditis with Pericardial Effusion: a Rare Presentation of Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) can manifest with diverse cardiac presentations, with pericarditis being the most common. However, myocarditis, albeit rare, poses significant risks. Here, we present the case of a 20-year-old woman initially diagnosed with tuberculosis but later found to have lupus myocarditis, a rare complication of SLE. She presented with features of left ventricular failure and pericardial effusion, demonstrating the importance of considering alternative diagnoses in such cases. Prompt management with supportive care and oral steroids led to eventual improvement.

Lupus myocarditis presenting as acute congestive heart failure : A case report

Systemic lupus erythematosus (SLE or lupus) is a disease in which the immune system attacks healthy cells and tissues throughout the body. Lupus myocarditis is a life-threatening condition, observed clinically in 3–9 % of patients with SLE. We report the case of a patient followed for multisystem SLE, presenting with de novo heart failure with severe left ventricular dysfunction revealing lupus myocarditis.

Acute myocarditis or apical balloon cardiomyopathy in a patient with systemic lupus erythematosus: The priority of common sense

Myocarditis can mimic takotsubo cardiomyopathy and via versa. We present a 70‐year‐old woman with known systemic lupus erythematous who presented to the emergency department twice during the last 2 years with chest pain, dyspnea and transient LV dysfunction. We also discuss the difficulties in differential diagnosis between lupus myocarditis and takotsubo cardiomyopathy.

A Case of Severe Lupus Myocarditis Preceded by Mesalazine-induced Lupus.

In drug-induced lupus (DIL), symptoms similar to those of systemic lupus erythematosus (SLE) usually resolve after discontinuation of the offending drug. A 41-year-old-woman with a history of ulcerative colitis presented with polyarthritis and myositis and was positive for anti-dsDNA IgG antibody. After discontinuation of mesalazine, the symptoms resolved, and the antibody titer decreased. The patient was diagnosed with DIL. Six months later, lupus myocarditis developed. After treatment with glucocorticoids, cyclophosphamide, intravenous immunoglobulin, and an intra-aortic balloon pump, she showed dramatic improvement. Patients with DIL and an immunological predisposition, such as anti-dsDNA antibodies, may have SLE and should be carefully monitored.

Cardiac Magnetic Resonance Imaging Contributes to the Accurate Diagnosis of Lupus Myocarditis.

Systemic lupus erythematous (SLE) is a multisystem autoimmune disease, with up to 50% of cases having cardiac involvement.1 Myocarditis is one of the cardiac presentations in SLE. Cardiac magnetic resonance imaging (cMRI) is a noninvasive diagnostic technique that provides evidence of acute myocardial inflammation by showing myocardial edema on T2-based imaging and myocardial injury on T1-based imaging.

Lupus myocarditis: review of current diagnostic modalities and their application in clinical practice.

Lupus myocarditis (LM) is a potentially fatal manifestation of SLE, occurring in 5-10% of patients. Clinical manifestations may vary from an unexplained tachycardia to fulminant congestive cardiac failure (CCF). With no single clinical or imaging modality being diagnostic, a rational and practical approach to the patient presenting with possible LM is essential. Markers of myocyte injury (including troponin I and creatine kinase) may be unelevated and do not exclude a diagnosis of LM. Findings on ECG are non-specific but remain essential to exclude other causes of CCF such as an acute coronary syndrome or conduction disorders. Echocardiographic modalities including wall motion abnormalities and speckle tracking echocardiography may demonstrate regional and/or global left ventricular dysfunction and is more sensitive than conventional echocardiography, especially early in the course of LM. Cardiac magnetic resonance imaging (CMRI) is regarded as the non-invasive diagnostic modality of choice in myocarditis. While more sensitive and specific than echocardiography, CMRI has certain limitations in the context of SLE, including technical challenges in acutely unwell and uncooperative patients, contraindications to gadolinium use in the context of renal impairment (including lupus nephritis) and limited literature regarding the application of recommended diagnostic CMRI criteria in SLE. Both echocardiography as well as CMRI may detect subclinical myocardial dysfunction and/or injury of which the clinical significance remains uncertain. Considering these challenges, a combined decision-making approach by rheumatologists and cardiologists interpreting diagnostic test results within the clinical context of the patient is essential to ensure an accurate, early diagnosis of LM.

Cardiovascular Manifestations of Systemic Lupus Erythematosus: An Overview

Systemic Lupus Erythematosus(SLE) is a multisystem autoimmune disease with variable presentations. A proposed mechanism for the etiology of SLE involves the development of autoantibodies that result from a defect in apoptosis. The specific defect involves the “find-me” (adenosine triphosphate [ATP]/uridine triphosphate [UTP]) or “eat-me” (phosphatidylserine) signals activated upon release of red cell nuclei. In the absence of apoptosis, the nuclei break down, causing inflammation and contributing to the development of autoimmunity. Many signs and symptoms of SLE are caused by either circulating immune complexes or direct effects of antibodies on cells. A genetic predisposition for SLE exists, and the concordance rate in monozygotic twins is between 25% and 70%. If a mother has SLE, her daughter’s risk of developing the disease is 1:40, and her son’s risk is 1:250. The course of SLE consists of intermittent remissions punctuated by disease flares, and organ damage often progresses over time. Pericarditis is the most frequent cardiac manifestation; it can be documented by ECG, auscultation of a friction rub, or evidence of pericardial effusion. It usually responds to anti-inflammatory therapy and infrequently leads to tamponade. More serious cardiac manifestations are myocarditis and fibrinous endocarditis of Libman-Sacks. The endocardial involvement can lead to valvular insufficiencies, most commonly of the mitral or aortic valves, or embolic events. It has not been proven that glucocorticoid or other immunosuppressive therapies improve lupus myocarditis or endocarditis. Still, it is usual practice to administer a trial of high-dose steroids and appropriate supportive treatment for heart failure, arrhythmia, or embolic events. As discussed above, patients with SLE are at increased risk for myocardial infarction, usually due to accelerated atherosclerosis, which probably results from immune attack, chronic inflammation, and chronic oxidative damage to arteries.

Clinical characteristics and outcomes of lupus myocarditis: a retrospective case control study in Chinese patients.

The aim of this study was to investigate the clinical features and outcomes of systemic lupus erythematosus (SLE) with myocarditis. A retrospective study was conducted in all inpatients diagnosed with SLE and concurrent lupus myocarditis (LM) at Peking Union Medical College Hospital (PUMCH) from July 2013 to July 2019. The case group included patients with LM while patients in the control group were enrolled randomly at a ratio of 1:1 and age- and year-matched SLE patients without myocarditis during the same period. Clinical characteristics and outcomes of LM patients were collected. Among 4719 SLE patients hospitalized to PUMCH over the 6-year period, 79 (1.67%) were diagnosed with LM, with a mean age of 32.38 ± 13.55 years and 89% were female. 52 (66%) cases presented abnormal ventricular wall motion function, and 30 (38%) cases showed a decreased left ventricular ejection fraction (LVEF) (<50%) in echocardiography. 10 (13%) LM patients died during hospitalisation. For risk factors of myocarditis, patients in the LM group had higher percentage of on neurological involvement, higher SLE disease activity index 2000 (SLEDAI-2K) scores, and higher rates of positive anti-nucleosome antibodies compared with the control group. Multivariate logistic regression demonstrated that low levels of C3 and high SLEDAI-2K scores were the independent risk factors for developing LM in SLE patients (OR=0.870, 95%CI 0.762-0.994, p=0.041; OR=1.058, 95%CI 1.008-1.110, p=0.023, respectively). Cardiac involvement especially myocarditis in SLE remains a rare but serious manifestation. Our research provided further insights into clinical features and risk factors of LM. Clinicians should be alerted for LM after cardiac manifestations occurred among those with SLE, which may reduce the risk of death.

Lupus acute cardiomyopathy is highly responsive to intravenous immunoglobulin treatment: Case series and literature review.

Introduction:Intravenous immunoglobulin (IVIg) is currently used with considerable success for the treatment of many autoimmune diseases, including systemic lupus erythematosus (SLE). Among its various indications, IVIg has also been found to be beneficial in myocarditis, whether or not it is associated with an autoimmune disease. Nevertheless, data regarding IVIg treatment for myocarditis/cardiomyopathy in patients with SLE are sparse. The objective of this case series was to describe our experience with IVIg as a treatment for lupus myocarditis and to review the literature for IVIg for this indication.Patient concerns:We report 5 female patients with SLE, who presented with signs of acute heart failure including pulmonary congestion and arrhythmias.Diagnosis:Echocardiography demonstrated new reduced left ventricular ejection fraction of 20% to 30%. Two patients underwent coronary artery angiography, which demonstrated normal coronary arteries, supporting the diagnosis of myocarditis or nonischemic cardiomyopathy.Interventions:High-dose IVIg treatment was initiated in all 5 patients.Outcomes:Following the treatment, clinical and echocardiographic improvement in cardiac function occurred within a few days to 1 month. This dramatic improvement persisted for several years.Conclusion:Based on our case series, we believe that IVIg has an important role in the management of lupus acute cardiomyopathy. This safe, well-tolerated optional treatment should be considered, especially in severe cases.

Therapeutic Strategies for Lupus Myocarditis Complicated by Cardiogenic Shock

Purpose Systemic lupus erythematosus (SLE) is a systemic disease which commonly involves the heart. Lupus myocarditis can present as a catastrophic event requiring aggressive therapy including mechanical circulatory support (MCS). We analyzed reported cases of cardiogenic shock in lupus in order to study utilized strategies and outcomes. Methods Three investigators independently searched PubMed, Ovid/Medline, and Google Scholar using terms “cardiogenic shock”, “cardiac shock” AND “lupus”, “systemic lupus erythematosus”, and “lupus myocarditis” for papers published in English in 1988-2020. We then manually searched the references in relevant articles. We included all cases of adult patients where individual patient data were present. Results We identified 22 cases, 18 (82%) females, mean age 27.7 +/- 3.35 years. All patients except two recovered (one patient died and one received heart transplantation). Left ventricular ejection fraction at the baseline was 20.5 +/- 3.49%, and after treatment it recovered to 46.1 +/- 6.2%. Management included inotropes and immunotherapy in all cases. In terms of immunotherapy, all received corticosteroids, but only 5 patients (25%) did not require any other immunotherapy. In six more patients, combination of steroids and cyclophosphamide appeared to be sufficient. In 10 of 20 patients, either plasmapheresis or intravenous immunoglobulin was also given. Remarkably, half of the patients (10 out of 21 patients who survived) received MCS: intra-aortic balloon bump placement (6 cases), venoarterial extracorporeal membrane oxygenation (5), left ventricular assist device (1), and biventricular support (1). Conclusion Based on reported cases, cardiogenic shock in lupus myocarditis has a high potential for recovery, can be treated successfully, but it requires an aggressive approach with immunotherapy going beyond corticosteroids and cyclophosphamide, and utilization of mechanical circulatory support.

Role of Cardiac Transplant in Systemic Lupus Erythematosus Complicated by Severe Heart Failure

Purpose Systemic lupus erythematosus (SLE) is a systemic disease and is commonly considered a contraindication for cardiac transplantation. Meanwhile, lupus myocarditis can present with severe left ventricular dysfunction which my progress to cardiogenic shock with a fatal outcome. Heart transplantation is rarely considered a treatment option, due to 1) multi-organ involvement, and 2) possibility of recurrence of lupus in the transplanted heart. We summarized published cases of cardiac transplantation in SLE. Methods Two investigators independently searched PubMed, Ovid/Medline, and Google Scholar using terms “heart transplantation”, “cardiac transplantation” AND “lupus”, “systemic lupus erythematosus”, and “lupus myocarditis” for papers published in English in 1988-2020. We then manually searched the references in relevant articles. We included all cases of adult patients where individual patient data were present. Results We identified 11 cases, six males, and five females, mean age 28 +/- 5.47 years. Six of the patients were male and five were female. While all patients had SLE, the most common indication for transplant was pulmonary hypertension (6), followed by lupus myocarditis (2), dilated cardiomyopathy (2), and infective endocarditis (1). In 6 patients there was a dual organ (heart/lung) transplantation, while in five the heart was the only transplanted organ. Two patients died within 2 months post transplant from bowel infarction, and one died in one year from rejection. Remaining eight patients were alive and well after a follow-up ranging nine months to four years, mean 26 +/- 9.9 months. Recurrence of lupus was not reported in any patient, including six cases where it was specifically indicated that there was no recurrence. Conclusion Based on reported cases, heart transplantation in SLE is a viable option, with good survival and no recurrence of lupus in the transplanted heart. In cases of intractable heart failure in patients with SLE, cardiac transplantation should be considered.

Outcome of clinical and subclinical myocardial injury in systemic lupus erythematosus - A prospective cohort study.

To determine the outcome of subclinical lupus myocarditis (LM) over twelve months with regards to: mortality; incidence of clinical LM and change in imaging parameters (echocardiography and cardiac magnetic resonance [CMR]). To evaluate the impact of immunosuppression on CMR evidence of myocardial tissue injury. SLE patients with and without CMR evidence of myocardial injury (as per 2009 Lake Louise criteria [LLC]) were included. Analysis at baseline and follow-up included: clinical evaluation, laboratory and imaging analyses (echocardiography and CMR). Clinical LM was defined as clinical features of LM supported by echocardiographic and/or biochemical evidence of myocardial dysfunction. Subclinical LM was defined as CMR myocardial injury without clinical LM. Forty-nine SLE patients were included with follow-up analyses (after 12 months) available in 36 patients. Twenty-five patients (51%) received intensified immunosuppressive therapy during follow-up for indications related to SLE. Disease activity (SLEDAI-2K) improved (p < 0.001) from 13 (median;IQR:9-20) to 7 (3-11). One patient without initial CMR evidence of myocardial injury developed clinical LM. Mortality (n = 10) and SLE clinical features were similar between patients with and without initial CMR myocardial injury. Echocardiographic left ventricular ejection fraction (LVEF) (p = 0.014), right ventricular function (p = 0.001) and wall motion abnormalities (p = 0.056) improved significantly but not strain analyses nor the left LV internal diameter index. CMR mass index (p = 0.011) and LVEF (p < 0.001) improved with follow-up but not parameters identifying myocardial tissue injury (LLC). A trend towards a reduction in the presence of CMR criteria was counterbalanced by persistence (n = 7) /development of new criteria (n = 11) in patients. Change in CMR mass index correlated with change in T2-weighted signal (myocardial oedema) (r = 386;p = 0.024). Intensified immunosuppressive therapy had no significant effect on CMR parameters. CMR evidence of subclinical LM persisted despite improved SLEDAI-2K, serological markers, cardiac function and CMR mass index. Subclinical LM did not progress to clinical LM and had no significant prognostic implications over 12 months. Immunosuppressive therapy did not have any significant effect on the presence of CMR evidence of myocardial tissue injury. Improvement in CMR mass index correlated with reduction in myocardial oedema and may be used to monitor SLE myocardial injury.

Serum cytokine levels associated with myocardial injury in systemic lupus erythematosus

AbstractObjectivesTo identify cytokines, markers of endothelial activation [soluble vascular cell adhesion molecule-1 (sVCAM-1)] and myocyte strain [soluble ST2 (sST2)] associated with myocardial injury (MInj) in SLE, classified by cardiac magnetic resonance (CMR) criteria.MethodsCMR was performed on patients with SLE, identifying stages of MInj (inflammation and necrosis or fibrosis). Data captured included: clinical assessment, laboratory and serological analyses, cytokine (IL-1β, IL-1Ra, IL-2, IL-6, IL-10, IL-17, IL-18, TNF-alpha), sVCAM-1 and sST2 levels. Cytokines were compared with regard to SLE features and evidence of CMR MInj. Predictors of CMR MInj were determined through regression analyses.ResultsForty-one patients with high disease activity (SLEDAI-2K: 13; IQR: 3–17) were included. SLE features included: LN (n = 12), neurolupus (n = 6) and clinical lupus myocarditis (LM) (n = 6). Nineteen patients had CMR evidence of MInj. Patients with a SLEDAI-2K ≥ 12 had higher sVCAM-1 (P = 0.010) and sST2 (P = 0.032) levels. Neurolupus was associated with higher IL-1Ra (P = 0.038) and LN with lower IL-1Ra (P = 0.025) and sVCAM-1 (P = 0.036) levels. Higher IL-1Ra (P = 0.012), IL-17 (P = 0.045), IL-18 (P = 0.003), and sVCAM-1 (P = 0.062) levels were observed in patients with CMR MInj compared with those without. On multivariable logistic regression, IL-1Ra predicted CMR inflammation and fibrosis/necrosis (P &amp;lt; 0.005) while anti-Ro/SSA [odds ratio (OR): 1.197; P = 0.035] and the SLE damage index (OR: 4.064; P = 0.011) predicted fibrosis/necrosis.ConclusionThis is a novel description of associations between cytokines and SLE MInj. IL-18 and IL-1Ra were significantly higher in patients with MInj. IL-1Ra independently predicted different stages of CMR MInj. Exploration of the role of these cytokines in the pathogenesis of SLE MInj may promote targeted therapies for LM.

The spectrum of lupus myocarditis: from asymptomatic forms to cardiogenic shock.

Lupus myocarditis is a serious, potentially deadly disease. When it presents as an acute or fulminant myocarditis in a patient without an established diagnosis of lupus, lupus as an etiology of the condition is not commonly suspected. Meanwhile, it has a distinct treatment which may be lifesaving. Review of the literature can shed more light as current management is mostly based on clinical experience and case reports rather than randomized control trials. In this review we are discussing this diagnostic entity, focusing on cardiogenic shock as a manifestation of lupus myocarditis, and discussing management including aggressive immunosuppression, mechanical circulatory support, and cardiac transplantation.

P52 Two pathologies one heart: life-threatening lupus myocarditis in a patient with pre-existing dilated cardiomyopathy

Abstract Background Acute myocarditis is a life-threatening complication of systemic lupus erythematosus (SLE) often indicative of severe multisystem disease. Treatment of lupus myocarditis (LM) is well described in the literature with high-dose corticosteroids and cyclophosphamide providing the backbone of induction therapy. Less commonly reported are outcomes of patients with SLE and pre-existing structural heart disease. Methods We present a case of acute LM occurring in the context of established non-ischaemic dilated cardiomyopathy (DCM) presenting a significant diagnostic challenge. Results A 45-year-old woman from Afghanistan with known SLE presented with acute chest pain and dyspnoea. She had a previous mild SLE disease course limited to her skin and joints and was historically non-compliant with immunosuppressive treatment. She also had non-ischaemic DCM diagnosed six years prior to presentation with non-progressive imaging over this time. Upon acute assessment at the referring centre a widespread livedoid rash and cool peripheries were noted. Initial investigations revealed an elevated Troponin-I of 111ng/L and Brain Natriuretic Peptide (BNP) of 4497ng/L. An electrocardiogram revealed no acute ischaemic changes whilst her chest radiograph revealed features in keeping with pulmonary oedema. Transthoracic echocardiogram (TTE) demonstrated a dilated left ventricle with severe global systolic impairment. Her left ventricular ejection fraction (LVEF) was visually estimated at 10-15%, significantly reduced from her last recorded LVEF of 52% on a cardiovascular magnetic resonance (CMR) scan 18-months previously. Intravenous (iv) methylprednisolone, iv diuresis and B-cell depletion therapy were commenced prior to transfer to our department. Once transferred, a CMR scan confirmed an ejection fraction of only 12%. Septal mid-wall delayed enhancement was seen consistent with a non-ischaemic DCM, however, subepicardial delayed enhancement was visualised inferolaterally on native T1-mapping in keeping with perimyocarditis. Additional results on admission were supportive of a multisystem SLE flare. She was lymphopaenic (1.0x109/L), thrombocytopaenic (66x109/L), hypocomplimentaemic (C3 0.33g/L, C4 &amp;lt;0.03g/L), and had elevated dsDNA antibodies (83unit/mL). Serum creatinine had risen from a baseline of 47µmol/L to 110µmol/L and her urine protein: creatinine ratio (414mg/mmol) raised the suspicion of lupus nephritis (LN). Renal biopsy was not performed due to thrombocytopaenia. An MDT discussion with the cardiology, CMR imaging and renal teams took place with CMR imaging proving pivotal in establishing new LM on a background of established DCM. The decision to treat with intravenous (iv) cyclophosphamide was made and repeat TTE at 14 days revealed a significant improvement in LVEF to 25-30%. Conclusion CMR imaging was crucial in delineating acute and potentially reversible myocarditis on a background of chronic cardiomyopathy. This finding supported the escalation in immunosuppressive treatment to a cyclophosphamide-based regimen and led to the rapid improvement in ejection fraction in this young patient. She received 6 fortnightly doses of iv cyclophosphamide and is now maintained on hydroxychloroquine, mycophenolate mofetil and rituximab. Disclosures H. Wilson-Morkeh: None. T. Youngstein: None. S. Shabbir: None. J. Bakshi: None. T. Pihlajavaara: None. M. Bellamy: None. T. Cairns: None.

SAT-392 CLINICAL ANALYSIS OF LUPUS NEPHRITIS AND LUPUS MYOCARDITIS AND APPLICATION OF CARDIAC MAGNETIC RESONANCE IN DIAGNOSIS

To investigate the clinical manifestations, treatment and prognosis of patients with lupus nephritis (LN) and lupus myocarditis (LM), and the application of cardiac magnetic resonance (CMR) imaging, especially T1-mapping technique in LM diagnosis. Sixty-six patients (61 women and 5 men, with a median onset age of 29 (21,39) years) diagnosed LN and LM from October 2016 to March 2019. The diagnosis for LM was based on clinical manifestations, EKG, serum troponin (CnT) and ultrasonic cardiogram (UCG), or CMR, meeting the Lake-Louise criteria made by the American College of Cardiology in 2009; or T1-mapping > 1370ms. The myocarditis or myocardial injury caused by virus or myocardial ischemia was excluded. The conditions of getting rid of dialysis and recovery of cardiac function were followed up. Fifty-five patients (83.3%) had AKI, Thirty-eight patients (57.6%) met the diagnosis of thrombotic microangiopathy (TMA). Forty-six patients undergoing renal biopsy mainly had pathology of type IV and type IV + V. LM was the first symptom in 22 cases (33%), and 51 (77.3%) presented myocarditis symptoms, among whom 50 patients had dyspnea, 23 had orthopnoea, 18 had palpitation and 7 had chest pain. Serum NT-proBNP was increased in 59 (89.4%), and troponin was increased in 15 (22.7%). Abnormal electrocardiogram was found in 45. UCG showed left ventricular enlargement in 38 (57.6%), ventricular wall thickening in 18 (27.3%), abnormal left ventricular wall motion in 14 (21.2%), and left ventricular ejection fraction (LVEF) < 50% in 14 (21.2%). Sixty-three patients underwent CMR examination, 34 (54%) had LVEF < 50%, 27 (42.8%) had delayed myocardial enhancement, 4 (6.3%) had high signal of myocardial T2 weighted imaging, only 4 patient met the Lake-Louise criteria, and no patients had early enhancement. The T1-mapping values of 49 (77.8%) were increased (1436±74ms). Sixty (90.9%) patients received methylprednisolone venous shock, 42 (63.6%) needed CRRT therapy, and 24 (36.4%) received gamma globulin and dual plasmaphermoplasty. During the follow-up with median of 7.5 months, 21 patients got rid of dialysis, 3 patients became chronic cardiac dysfunction. When SLE involves the kidney and heart simultaneously, the damage of cardiac renal function is serious. Half of them are associated with thrombotic microvascular diseases, and early active treatment on the cardiac and renal function can achieve good improvement. New quantitative imaging technology T1-Mapping can improve the sensitivity of early diagnosis for LM.

Clinical manifestations of parvoviral infection in Uzbekistan

Parvoviral infection (PV B19) is an important but underinvestigated problem. Parvovirus B19 is being studied for the first time in Uzbekistan, and its clinical manifestations are not well known, thus requiring differential diagnosis with other viral infections and noncommunicable diseases. Available literature reported the possible relation of PV B19 in children to a number of pathological conditions, such as allergies, severe anemia, arthralgia, periarteritis nodosa, systemic lupus erythematosus, myocarditis, and hepatitis.

Myocarditis as a lupus challenge: two case reports

BackgroundMyocarditis is an uncommon manifestation of systemic lupus erythematosus in which the clinical presentation can range from subclinical to life-threatening. We report cases of two patients who presented to our hospital with myocarditis as an initial manifestation of systemic lupus erythematosus despite negative results of extensive workup that excluded other diagnoses. The mainstays of treatment are corticosteroids, immunosuppressive agents, and anti-heart failure medications, with use of the latter being case-specific. Mycophenolate mofetil was the cornerstone of the proposed treatment for induction of remission, although it is well known to be used as a maintenance therapy in lupus myocarditis.Case presentationBoth Emirati patients described satisfied the diagnostic criteria for mixed connective tissue disease (systemic lupus predominant) and systemic lupus erythematous. Other differential diagnoses of myocarditis were excluded. The patients were started on pulsed steroid followed by oral steroid, with hydroxychloroquine, mycophenolate mofetil, and anti-heart failure medications used as needed. Dramatic responses were noted in the first few weeks in terms of symptoms.ConclusionEarly recognition and treatment of lupus myocarditis is needed to avoid fatal consequences.

21. Sepsis?

IntroductionSepsis is a major cause of mortality and there have been many patient safety initiatives to improve the outcome such as ‘Think Sepsis’ and ‘Sepsis 6’. It is essential to consider mimics of sepsis in those presenting with fever, particularly when there is no improvement despite antimicrobials. Macrophage activation syndrome (MAS) is a complication of uncontrolled inflammation from underlying connective tissue disease resulting in a sepsis-like condition. Delay to the detection and treatment of this condition can also result in significant morbidity and mortality.Case descriptionWe report a 28-year-old female presenting with shortness of breath, chest pain, bilateral leg swelling and fevers. Relevant past medical history included a recent diagnosis of Graves’ disease for which she had been commenced on carbimazole. On presentation CRP 53 mg/L and d-dimer 14100, chest X-ray; cardiomegally and bilateral pleural effusions, CT thoracic aorta angiogram; large pericardial effusion with maximum depth 5.9cm and compression of the cardiac chambers. Echocardiogram; preserved ejection fraction of 55-60%. The patient was commenced on intravenous antibiotics, furosemide and pericardial drain removed 1.5L. The patient was referred to the rheumatology team on day 10. She reported episodic arthralgia, recurrent mouth ulcers, anaemia; with no evidence of haemolysis and pericardial effusion (serositis). Serology; ANA 1:1280 fine speckled pattern, dsDNA > 200, ENA positive (RNP and Smith antibodies) and low complement; C3 (0.35) and C4 (0.005), ferritin 1400, CRP 40 and ESR 62; consistent with a diagnosis of systemic lupus erythematosus (SLE). The patient was pulsed with IV methylprednisolone 500mg on Day 13 and started on prednisolone 40mg and azathioprine.Day 16; the patient developed a new pyrexia and profound hypotension resulting in admission to intensive care unit for inotropic support and escalation of antibiotics. Bedside echo revealed a small volume pericardial fluid; no tamponade, but significant new biventricular failure. Repeat ferritin was 8489, Triglycerides 5.5, LDH 331. The patient had persistent fevers and blood dyscrasias consistent with macrophage activation syndrome (MAS) with lupus myocarditis. The patient was pulsed with a further two doses of IV methylprednisolone 750mg and commenced on ciclosporin 3mg/kg.Day 18; the patient became increasingly tachycardic with increasing oxygen demands and a worsening metabolic acidosis despite renal replacement therapy. Based on the rapidity of deterioration, and lack of response to intervention the patient was transferred for extracorporeal membrane oxygenation (ECMO).DiscussionThis case was of interest due to the diagnostic difficulties the team faced as an underlying autoimmune condition was not initially considered. Distinguishing the need for antibiotics versus immunosuppression can be daunting for general medical teams. This case highlights the importance of involving rheumatology early in the course of a patient presenting with fever not responding to conventional therapies. Lupus myocarditis was confirmed by cardiac biopsy after the patient went into cardiogenic shock with a LVEF 10% due to lupus myocarditis requiring ECMO support showing the severity of disease activity. The use of ECMO remains rare in our practice and there are only five centres within the UK offering this service.The patient has made an excellent response to treatment and is well maintained on mycophenolate mofetil and hydroxychloroquine; most recent echocardiogram May 2018 shows resolved effusion with normal biventricular size, ejection fraction 50 to 55%.Key learning pointsMAS secondary to underlying rheumatic and connective tissue disease can present with fever and features that mimic sepsis. An awareness of MAS is needed as early diagnosis and prompt initial treatment are both key factors for a favourable outcome. This case resulted in a change to clinical practice. A Trust-wide guideline on the management of MAS has now been produced to guide teams on the management of MAS in the patient with sepsis who is not improving. This case also shows the importance of collaborative team working (physicians, ICU, tertiary centre).Conflicts of interestThe authors have declared no conflicts of interest.