[Management of Constrictive Pericarditis : A study of 43 operated cases]. / Gestion de la péricardite constrictive : à travers l'étude de 43 cas opérés.

Chronic constrictive pericarditis is a rare condition characterized by clinical signs of right heart failure, due to the symphysis of the two pericardial leaflets. Our study focused on a retrospective analysis of 43 CCP surgery observations collected over an 11-year period (2003-2013). The mean age of the patients was 32 years; 65% were male; exercise dyspnea (95%) was the most frequent sign. Two main etiologies were observed: tuberculosis 58% and idiopathic causes 42%. All of our patients received a subtotal pericardectomy per median sternotomy, of which 95% had no cardiopulmonary bypass.

[Symptomatic atrial fibrillation revealing chronic constrictive pericarditis in a young patient: A case report with a review of the literature]. / Fibrillation atriale symptomatique révélant une péricardite chronique constrictive chez un patient jeune : à propos d'un cas avec revue de la littérature.

Chronic Constrictive pericarditis (CCP) is a rare clinical entity that can pose diagnostic problems. Constrictive pericarditis is the final stage of a chronic inflammatory process characterized by fibrous thickening and calcification of the pericardium that impairs diastolic filling, reduces cardiac output, and ultimately leads to right heart failure and to atrial dilatation which can caused supravetricular arrythmia. Transthoracic echocardiography, computed tomography, and cardiac magnetic resonance imaging each can reveal severe diastolic dysfunction, increased pericardial thickness and calcifications. The gold standard for diagnosis is cardiac catheterization with analysis of intracavitary pressure curves, which are high and, in end diastole, equal in all chambers. CCP is the commun cause of recurrent heart failure. At present, idiopathic or viral pericarditis is the predominant cause of CP in the Western world, followed by postcardiotomy irritation and mediastinal irradiation. Tuberculosis is still a cause of pericarditis in developing countries and in immunosuppressed patient. We present a patient with symptomatic atrial fibrillation revealing chronic constrictive pericardis. He underwent to drug cardioversion before radical pericardiectomy and to date has made a good recovery without palpitations with a sinus rythm. The diagnosis of CP is often neglected by physicians, who usually attribute the symptoms to another disease process. This case show the difficulty in diagnosing, illustrated as well as the role of multimodality imaging and the excellent outcome of pericardiectomy for total recovery.

[Constrictive pericarditis and disseminated nocardiosis]. / Péricardite constrictive et la nocardiose disséminée.

Human nocardiosis usually involves the respiratory tract or the skin but may disseminate to virtually any organ, it occurs in immunocompromised hosts as well as individuals with no apparent predisposition. Involvement of the pericardium is uncommon, having been reported infrequently in the past, but mandates a special management. This report describes the first case in Europe of a patient with chronic constrictive pericarditis from nocardia brasiliens, successfully treated with pericardiectomy and appropriate antibiotic therapy.

[A rare and atypical form of tularemia in a context of immunodepression]. / Forme rare et atypique de tularémie dans un contexte d'immunodépression.

INTRODUCTION: We present an original severe case of tularemia with cutaneous damage, lymphadenopathy and pericarditis ; pathology of increasing incidence in Europe due to global warming. OBSERVATION: A 33-years-old women consulted emergency unit for altered general condition, anorexia, hyperthermia at 38,3°C, dyspnea and dry cough evolving for few days. Her only history was Crohn's disease with introduction of an anti-TNF alpha for 3 months. The interrogation found regular forest walks ¼. Treatment with Amoxicillin/clavulanic acid 1g 3 times daily and curative anticoagulation was started after the initial diagnosis of infectious pneumonia associated with pulmonary embolism. The patient reconsulted 2 weeks later for clinical deterioration associated with skin lesions. The chest CT scan showed increased mediastinal lymphadenopathy and a circumferential pericardial effusion ; quantified at 5mm on transthoracic ultrasound. Tularemia serology was positive in IgG at 400IU/mL. Despite an adapted antibiotic therapy with Ciprofloxacin, the patient presented a new brutal clinical deterioration. A pericardiocentesis was performed and the analysis revealed a predominantly neutrophilic exudate and a strongly positive PCR Francisella tularensis. Gentamicin 5mg/kg was associated allowing a resolution of the symptoms. CONCLUSION: Tularemia is one of the pathologies whose atypical presentation with pericarditis (favored by a certain immunodepression) worsens the prognosis. Global warming influences the epidemiology of inoculation diseases, including tularemia, making it more frequent.

Anti-SARS-CoV-2 vaccine-induced myocarditis - real but, in general, rare and mild: A consensus statement from the Studies Committee of the Portuguese Society of Cardiology.

Acute myocarditis (especially) and pericarditis have been consistently associated with the administration of vaccines against SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2), generating anxiety in the general population, uncertainty in the scientific community and obstacles to ambitious mass vaccination programs, especially in foreign countries. Like some of its European counterparts, the Portuguese Society of Cardiology (SPC), through its Studies Committee, decided to take a position on some of the most pressing questions related to this issue: (i) How certain are we of this epidemiological association? (ii) What is the probability of its occurrence? (iii) What are the pathophysiological bases of these inflammatory syndromes? (iv) Should their diagnosis, treatment and prognosis follow the same steps as for typical idiopathic or post-viral acute myopericarditis cases? (v) Is the risk of post-vaccine myocarditis great enough to overshadow the occurrence of serious COVID-19 disease in unvaccinated individuals? In addition, the SPC will issue clinical recommendations and offer its outlook on the various paths this emerging disease may take in the future.

Dialysis-related constrictive pericarditis: old enemies may sometimes come back / Pericardite Constritiva relacionada a diálise: velhos inimigos podem, às vezes, voltar

ABSTRACT Cardiovascular disease is the main cause of death in patients with chronic kidney disease (CKD). Several heart conditions have been associated with CKD, including myocardial and pericardial diseases. This paper describes a case of Dialysis-related constrictive pericarditis in a patient diagnosed with sudden hypotension during a hemodialysis session. A 65-year-old man diagnosed with hypertension, diabetes, obesity, and cirrhosis on hemodialysis for two years complained of symptoms during one of his sessions described as malaise, lipothymia, and confusion. The patient had a record of poor compliance with the prescribed diet and missed dialysis sessions. He was sluggish during the physical examination, and presented hypophonetic heart sounds, a blood pressure of 50/30mmHg, and a prolonged capillary refill time. The patient was referred to the intensive care unit and was started on antibiotics and vasoactive drugs. His workup did not show signs of infection, while electrocardiography showed low QRS-wave voltage. His echocardiogram showed signs consistent with a thickened pericardium without pericardial effusion. Cardiac catheterization showed equalization of diastolic pressures in all heart chambers indicative of constrictive pericarditis. The patient underwent a pericardiectomy. Examination of surgical specimens indicated he had marked fibrosis and areas of dystrophic calcification without evidence of infection, consistent with Dialysis-related constrictive pericarditis. Hypotension for unknown causes must be considered in the differential diagnosis of dialysis patients.

RESUMO A doença cardiovascular é a principal causa de morte em pacientes com doença renal crônica (DRC). Várias formas de acometimento cardíaco têm sido associadas. à DRC, incluindo doenças miocárdicas e pericárdicas. Este artigo descreve um caso de pericardite constritiva relacionada a em um paciente diagnosticado com hipotensão súbita durante uma sessão de hemodiálise. Um homem de 65 anos com diagnósticos prévios de hipertensão, diabetes, obesidade e cirrose em hemodiálise por dois anos queixou-se de sintomas durante uma de suas sessões, descritos como mal-estar, lipotímia e confusão mental. Apresentava histórico de baixa adesão à dieta prescrita e faltas frequentes às sessões de diálise. Ele estava fraco durante o exame físico e apresentava bulhas cardíacas hipofonéticas, pressão arterial de 50/30mmHg e tempo de enchimento capilar prolongado. O paciente foi encaminhado para a unidade de terapia intensiva e iniciou o tratamento com antibióticos e drogas vasoativas. Investigação laboratorial não mostrou sinais de infecção, enquanto o eletrocardiograma mostrou baixa voltagem de complexo QRS. Seu ecocardiograma evidenciou sinais consistentes com um pericárdio espessado, sem derrame pericárdico. O cateterismo cardíaco mostrou equalização das pressões diastólicas em todas as câmaras cardíacas, indicativo de pericardite constritiva. O paciente foi submetido a uma pericardiectomia. O exame anatomopatológico mostrou sinais de acentuada fibrose acentuada fibrose e áreas de calcificação distrófica sem evidência de infecção, consistente com pericardite constritiva relacionada a por diálise. A hipotensão por causas desconhecidas deve ser considerada no diagnóstico diferencial de pacientes em diálise.

[Heart involvement in systemic lupus erythematosus and antiphospholipid syndrome]. / Cœur et médecine interne : lupus systémique et syndrome des antiphospholipides.

Cardiac involvement in systemic lupus (SL) and antiphospholipid syndrome (APS) can be due to variables and involve different presentations. Pericarditis is the most common lupus manifestation and occurs in 16% to 25% of patients. While corticosteroids are usually very effective, colchicine may avoid steroids and prevent relapse. Myocarditis during SL is rare and often inaugural. They may manifest as chest pain, acute heart failure, arrhythmias or conduction disturbances, and may progress to dilated cardiomyopathy and/or permanent heart failure. Their prognosis is however generally good, even in the absence of treatment with cyclophosphamide for the less serious forms. Finally, coronary involvement in SL is most often due to atherosclerotic, thrombotic origin (generally in the context of associated APS), and exceptionally explained by coronary vasculitis. During APS, valve disease is frequent and usually asymptomatic. Thrombotic damage can be (1) coronary, typically manifesting as a myocardial infarction in a young subject with healthy coronary arteries, (2) much more rarely intracardiac, or (3) microcirculatory, generally as part of a catastrophic antiphospholipid syndrome (CAPS) leading to a multiorgan failure. Finally, iatrogenic cardiac manifestations can exceptionally be seen during treatment with cyclophosphamide or antimalarials characterized by conduction disorders and/or heart failure.

[Systemic nocardiosis with mediastinal lymph node involvement proven by endoscopic ultrasound]. / Nocardiose systémique avec atteinte ganglionnaire médiastinale prouvée par échoendoscopie.

INTRODUCTION: Systemic nocardiosis is an infectious disease that is rarely associated with mediastinal lymph nodes. CASE REPORT: We report the case of a 72-year-old male patient treated with a high dose of oral corticosteroids for rheumatoid polyarthritis. This patient presented with rapid overall deterioration associated with mediastinal lymph nodes. Endobronchial ultrasound enabled us to establish a diagnosis of systemic nocardiosis. The patient recovered after having received suitable antibiotic treatment for four months. CONCLUSION: This work reports on a rare clinical presentation of systemic nocardiosis associated with mediastinal lymphadenopathies and highlights the key role of endobronchial ultrasound in diagnosing mediastinal lymph nodes, especially in differential diagnosis for lung cancer.

[Characteristics of cardiac involvement in eosinophilic granulomatosis with polyangiitis]. / Caractéristiques de l'atteinte cardiaque au cours de la granulomatose éosinophilique avec polyangéite.

AIM OF THE STUDY: Our study aimed to identify the characteristics of cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: We conducted a retrospective analytic study including EGPA cases diagnosed between 2000 and 2019 in an internal medicine department. Diagnosis was made according to the 1990 American College of Rheumatology criteria and the 2012 Chapel Hill Concensus. RESULTS: Eleven EGPA cases were included, 64% of patients were female. Median age at diagnosis was 52 years [42-58]. Heart damage revealed EGPA in 55% of cases with a significant predominance of women (p=0.015). The main cardiac manifestations were myocarditis, ischemic cardiomyopathy due to small vessel vasculitis, cardiac tamponade and intracardiac thrombus. Cardiac magnetic resonance imaging (MRI) mainly showed subendocardial hyposignal in early infusion and late enhancement in the same areas, nodular by locations, associated with impaired left ventricle function and micro-infarctions by distal vasculitis. Cardiac damage was associated to ANCA negativity in 83.3% of cases. The median Birmingham Vasculitis Activity Score version3 (BVAS v3) was 16 [10-17]. Under conventional treatment, no relapses had occurred. The median vasculitis damage index (VDI) was 2 [1-2.3] and the mortality rate was zero after a mean follow-up of 43 months. CONCLUSION: Cardiomyopathy is a frequent revealing mode of EGPA. A late onset asthma and hypereosinophilia should guide the diagnosis. As ANCA research often turns out to be negative, histological evidence is recommended in this context. The contribution of cardiac MRI in the diagnosis of EGPA remains to be defined.

[Characteristics of effusive pericarditis in N'Djamena (Chad)]. / Caracteristiques des pericardites liquidiennes a N'Djamena (Tchad).

INTRODUCTION: Pericarditis is a commun cause of hospitalisation in cardiology and internal medicine wards. OBJECTIVE: We aimed to describe the epidemiological profile of effusive pericarditis at the Department of cardiology of the National Referral Teaching Hospital of N'Djamena, Chad. METHODS: We undertook a descriptive cross-sectional study from January 2017 to December 2019. Patients presenting with effusive pericarditis and who consent to participate were consecutively enrolled during the study period. RESULTS: Overall, 1805 patients were hospitalized at the department of cardiology during the study period with effusive pericarditis accounting for 4.3% of all cases (n = 78). Patients' mean age was 35.84 ± 14 years, [range 16 and 73 years]. The sex ratio was 0.89. Exertional dyspnea, chest pain, poor general condition and fever were main symptoms reported in 90%, 89%, 81% and 51% of the cases respectively. Pericardial rub was found in 51% of the study patients. Eighteen patients (26%) were HIV positive and 97% of the study patients had cardiomegaly. ECG demonstrated low QRS voltage (97%) and diffuse abnormalities of repolarisation (96%). Pericardial effusion was found abundant in 57% of the cases. Etiologies of effusive pericarditis were mainly tuberculosis (47%), idiopathic (21%) and HIV infection (13%). Thirty patients (43%) benefited from pericardiocentesis. The in-hospital mortality rate of the disease was 17%. CONCLUSION: Effusive pericarditis is frequent and associated with poor outcome. Treatment depends on etiology dominated by tuberculosis in Chad.

INTRODUCTION: Les péricardites représentent une cause fréquente d'hospitalisation en cardiologie et en médecine interne. OBJECTIF: Décrire les caractéristiques épidémiologiques et étiologiques des péricardites liquidiennes dans le service de cardiologie du Centre Hospitalier Universitaire la Référence Nationale (CHU-RN). MÉTHODES: Il s'est agi d'une étude transversale descriptive menée de Janvier 2017 à Décembre 2019 sur une série consécutive des patients hospitalisés pour péricardite liquidienne dans le service de cardiologie du Centre Hospitalier Universitaire la Référence Nationale de N'Djamena. RÉSULTATS: Durant la période d'étude, 1805 patients étaient hospitalisés dans le service de cardiologie dont 78 pour une péricardite liquidienne ; soit une prévalence de 4,3%. L'âge moyen était de 35,84 ± 14 ans, [extrêmes 16 et 73 ans]. Le sex-ratio était de 0,89. La dyspnée d'effort, la douleur thoracique, l'altération de l'état général et la fièvre étaient les maitres symptômes dans respectivement 90%, 89%, 81% et 51% des cas. Le frottement péricardique a été retrouvé chez 51% des patients. La sérologie VIH était positive chez 18 patients (26%) et 97% des patients avaient une cardiomégalie. A l'ECG, on retrouvait un micro voltage et des troubles diffus de la repolarisation dans respectivement 97% et 96% des cas. L'épanchement péricardique était majoritairement (57%) de grande abondance à l'échocardiographie. Les étiologies prédominantes étaient la tuberculose dans 47% des cas, une cause idiopathique dans 21% et une infection virale (VIH) dans 13%. Trente patients (43%) avaient bénéficié d'une ponction péricardique. Le taux de mortalité de la péricardite liquidienne était de 17%. CONCLUSION: la péricardite liquidienne est une pathologie fréquente, grave et mortelle au Tchad. Le traitement dépend de l'étiologie qui est dominée par la tuberculose.

Pericardite tuberculosa em paciente imunocompetente: relato de caso / Tuberculous pericarditis in an immunocompetent patient: case report

A pericardite tuberculosa é uma forma rara de apresentação de tuberculose extrapulmonar, sendo mais frequente em regiões endêmicas e em pacientes imunocomprometidos. O quadro clínico é na maioria das vezes insidioso, por vezes com sintomas inespecíficos ou com quadro de tamponamento cardíaco. O diagnóstico é feito pela análise do líquido pericárdico ou biópsia pericárdica e o tratamento é realizado com rifampicina, isoniazida, pirazinamida e etambutol. O presente caso relata uma paciente de 51 anos, imunocompetente, com quadro de febre e dispneia há 7 dias. Em tomografia de tórax foi identificado importante derrame pericárdico, sem repercussão hemodinâmica em ecocardiografia transtorácica. A paciente foi submetida à drenagem do derrame pericárdico, com diagnóstico de tuberculose pericárdica pela análise do líquido. Iniciado o tratamento com RHZE, com boa evolução clínica e seguimento ambulatorial.

Tuberculous pericarditis is a rare form of extrapulmonary tuberculosis, being more frequent in endemic regions and in immunocompromised patients. The clinical picture is most often insidious, sometimes with nonspecific symptoms or with cardiac tamponade. Diagnosis is made by analysis of pericardial fluid or pericardial biopsy, and treatment is performed with rifampicin, isoniazid, pyrazinamide, and ethambutol. The present case reports a 51-year-old patient, immunocompetent, with fever and dyspnea for 7 days. A chest tomography showed significant pericardial effusion, without hemodynamic repercussions on transthoracic echocardiography. The patient underwent drainage of the pericardial effusion, with a diagnosis of pericardial tuberculosis by fluid analysis. Treatment with RHZE was started, with good clinical evolution and outpatient follow-up.

[Polymyalgia rheumatic and chronic myelomonocytic leukemia]. / Pseudo-polyarthrite rhizomélique satellite d'une leucémie myélomonocytaire chronique.

INTRODUCTION: Myelodysplasia (MDS) can occur as systemic manifestations such as connective tissue diseases or vasculitis. Rheumatological manifestations are also described in such context. Herein, we report the observation of a patient with chronic myelomonocytic leukemia (CMML) who developed systemic manifestations: polymyalgia rheumatica and pericarditis. CASE REPORT: A 78-year-old patient was referred for the exploration of two months history of inflammatory shoulder pain associated with biological inflammatory syndrome. He presented with asthenia, anorexia and loss of 5kg in one month. He had a three years follow-up for a CMML without any specific treatment. All of the explorations carried out showed a typical polymyalgia rheumatica. A pericardial effusion requiring emergency drainage was synchronously diagnosed. All the symptoms occurred during a worsening of his hematological disease. The rheumatological manifestation was favorable after a short corticosteroid therapy and pericarditis did not recur after 2 years of follow-up. CONCLUSION: It should be necessary to screen patients for MDS in a context of systemic manifestation, especially in elderly patients with an abnormal blood count (cytopenia, macrocytosis and monocytosis).

Galectina-3 em pacientes com pericardite constritiva crônica / Galectin-3 Levels in Patients with Chronic Constrictive Pericarditis

FUNDAMENTO: A galectina-3 (Gal-3) é uma molécula pró-inflamatória e pró-fibrótica, envolvida na patogênese da insuficiência cardíaca. O papel da Gal-3 em pacientes com pericardite constritiva crônica (PCC) não está claro. OBJETIVO: O objetivo deste estudo foi avaliar os níveis de Gal-3 em pacientes com PCC e correlacioná-los com parâmetros clínicos, funcionais e histológicos. MÉTODOS: Nós avaliamos prospectivamente 25 pacientes sintomáticos com PCC agendados à pericardiectomia e 21 controles sadios. Os pacientes foram submetidos à avaliação clínica, medidas de Gal-3 e peptídeo natriurético do tipo B (BNP), ecocardiografia, ressonância magnética cardíaca e teste cardiopulmonar de exercício (TCPE) no período basal. Seis meses após a pericardiectomia, repetiu-se o TCPE. Um erro alfa < 5% foi considerado estatisticamente significativo, com um intervalo de confiança de 95%. RESULTADOS: Foram incluídos 25 pacientes com idade mediana de 45 anos. A etiologia foi principalmente idiopática (n = 19, 76%), e 14 (56%) apresentaram classe funcional New York Heart Association (NYHA) III/IV. Os valores medianos de BNP e Gal-3 foram 143 (89-209) pg/dL e 14,8 (9,7-17,2) ng/mL, respectivamente. Os níveis de Gal-3 não foram estatisticamente maiores nos pacientes com PCC que em controles (p = 0,22). Não foram encontradas correlações significativas da Gal-3 com BNP, medidas ecocardiográficas e de ressonância magnética cardíaca, e achados histológicos. Após a pericardiectomia, encontrou-se uma correlação estatisticamente significativa entre Gal-3 e medidas do TCPE ­ duração do teste (r = ­0,79; p < 0,001) e tempo de exercício (r = ­0,79; p < 0,001). CONCLUSÕES: Pacientes com PCC apresentaram níveis normais de Gal-3, quando comparados aos indivíduos controles. A Gal-3 não se correlacionou com medidas morfológicas e funcionais antes da pericardiectomia. No entanto, associações entre Gal-3 e intolerância ao exercício após pericardiectomia pode sugerir um papel da Gal-3 na predição de prognóstico após a pericardiectomia.

[Diffuse infiltrative lung disease, pericarditis, pleural effusion and ceritinib hypersensitivity]. / Pneumopathie infiltrative diffuse, péricardite et pleurésie d'hypersensibilité compliquant un traitement par ceritinib.

Tyrosine kinase inhibitors are now major actors for the treatment of non-small-cell metastatic lung cancers where ROS 1 gene rearrangement is present. Because of the rapid development of these new therapies, developing information about their monitoring and knowledge about their potential toxicities is essential. We describe the case of a patient who was treated with ceritinib as a third line approach for a metastatic lung adenocarcinoma with ROS1 rearrangement. After two months, the patient developed acute respiratory distress with pericarditis and pleurisy. A hypersensitivity reaction was suggested and supported by favorable clinical and radiological outcomes within three days following ceritinib discontinuation and systemic corticosteroid introduction. Pleural effusion, pericarditis and diffuse pulmonary infiltration associated to ceritinib have not often been described previously. Despite few data of pulmonary toxicity related to ceritinib, the current observation highlights the need for caution and regular monitoring when using these inhibitors.

Precordial pain, leukocytosis and bicytopenia in a teenager with systemic juvenile idiopathic arthritis under immunosuppressive therapy / Dor precordial, leucocitose e bicitopenia em adolescente com artrite idiopática juvenil sistêmica em terapia imunossupressora

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.