McConnell's sign is a classic echocardiographic finding most often encountered in the context of acute pulmonary embolism and is defined by severe hypokinesis or akinesis of the right ventricular free wall and hypercontractility of the apex. Despite having a high specificity for pulmonary embolism, other causes of McConnell's sign have been reported. This case is a rare presentation of McConnell's sign in association with myopericarditis. The patient presented with a systemic lupus erythematosus flare as well as acute pleuritic chest pain and was found to have significantly elevated high-sensitivity troponin, pericardial effusion, and McConnell's sign on echocardiography. Pulmonary embolism and underlying pulmonary hypertension were ruled out. The patient improved clinically with treatment of a systemic lupus erythematosus flare, and short-term follow-up echocardiography demonstrated resolution of right ventricular dysfunction. This case challenges the conventional diagnostic specificity attributed to McConnell's sign for pulmonary embolism by describing a similar pattern of regional right ventricular wall motion abnormalities in the setting of an inflammatory myopericardial syndrome. Additionally, it underscores the utility of speckle-tracking strain analysis to help differentiate potential etiologies in right ventricular dysfunction. This case demonstrates the importance of having a broad differential when encountering McConnell's sign and represents an underappreciated cause of McConnell's sign associated with myopericarditis.

NLRP3 inflammasome is a critical cytosolic multiprotein complex central to the innate immune response. Upon activation, NLRP3 oligomerizes and recruits the adapter protein ASC; this scaffold recruits and activates pro-caspase-1. Active caspase-1 catalyzes the proteolytic maturation and secretion of the potent pro-inflammatory cytokines IL-1β and IL-18, and induces a programmed cell death called pyroptosis. Dysregulated or chronic NLRP3 inflammasome activation is a major driver of pathogenesis in a wide spectrum of peripheral inflammatory diseases, including gout, pericarditis, atherosclerosis, nonalcoholic steatohepatitis, and NLRP3 gain-of-function autoinflammatory disorders known as CAPS, as well as neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and multiple sclerosis. Herein we described the discovery of NLRP3 inhibitors based on the [1,2,4]triazolo[1,5-a]pyrimidine scaffold. Represented by compound 25, this scaffold exhibited exceptional potency, favorable physicochemical properties, and desirable pharmacokinetic profiles, including good brain penetration. Compound 25 showed potential as a candidate for the treatment of Parkinson's disease.

Autoimmune pericarditis is commonly idiopathic but may represent the initial manifestation of systemic autoimmune disease. Hepatic autoimmune disorders rarely present with primary cardiac involvement, and recognition of extrahepatic presentations is essential for early diagnosis and prevention of organ damage. Autoimmune hepatitis-primary biliary cholangitis (AIH-PBC) overlap syndrome is an uncommon entity characterized by combined hepatocellular and cholestatic immune-mediated injury. A previously healthy young woman presented with progressive pleuritic chest pain and dyspnea and was found to have a large pericardial effusion causing hemodynamic compromise requiring surgical drainage. Pericardial pathology demonstrated acute and chronic fibrinous pericarditis. Initial evaluation revealed elevated inflammatory markers and mild transaminitis with positive autoimmune serologies including antinuclear antibody and anti-smooth muscle antibody. Following clinical improvement, she developed recurrent pericarditis accompanied by worsening liver enzyme abnormalities. Liver biopsy demonstrated chronic active hepatitis with bile duct injury and bridging fibrosis, consistent with autoimmune hepatitis-primary biliary cholangitis overlap syndrome. Cardiac magnetic resonance imaging confirmed active pericardial inflammation without myocarditis. Immunosuppressive therapy with corticosteroids followed by azathioprine resulted in clinical stabilization. This case highlights autoimmune pericarditis as the presenting manifestation of AIH-PBC overlap syndrome and underscores the importance of evaluating unexplained pericarditis for systemic autoimmune disease. Early recognition of cardio-hepatic autoimmune overlap allows timely immunosuppressive therapy and may prevent progression to advanced hepatic fibrosis.

Pericardial disease represents a diverse clinical spectrum, including acute pericarditis, pericardial effusion, cardiac tamponade, effusive-constrictive pericarditis, and constrictive pericarditis, which vary significantly in presentation, urgency, and management. Recent consensus guidelines from the European Society of Cardiology and the American College of Cardiology have enhanced clinical phenotyping, multimodal imaging techniques, and targeted immunomodulatory therapies; however, their implementation in clinical practice remains variable. We introduce our updated pathway-based framework that structures diagnosis and treatment around five key clinical scenarios: chest pain, hemodynamic collapse, dyspnea, incidental pericardial effusion, and right-sided heart failure. Each scenario guides a systematic process of risk stratification, tiered diagnostic evaluation, and phenotype-specific therapy. In cases of acute pericarditis, management employs a three-tier escalation approach, with interleukin-1 inhibitors (rilonacept, anakinra) as the preferred escalation for inflammatory phenotypes unresponsive to NSAIDs and colchicine. Cardiac magnetic resonance imaging is integral to phenotyping, utilizing late gadolinium enhancement and T2-STIR sequences to inform treatment escalation. Management of pericardial effusion is standardized through a validated drainage scoring system that considers etiology, size, and echocardiographic hemodynamics. Constrictive pericarditis is classified into three subtypes: transient/inflammatory, effusive-constrictive, and chronic/fixed, each with a distinct management pathway, reserving surgical pericardiectomy at specialized centers for fibrotic or medically refractory cases. This pathway consolidates current evidence into a reproducible clinical framework designed to standardize care, enhance diagnostic precision, and facilitate shared decision-making among multidisciplinary pericardial disease teams.

Adult-onset Still's disease (AOSD) is a rare systemic autoinflammatory disorder without universally accepted disease-specific biomarkers, although serum ferritin and glycosylated ferritin may provide supportive diagnostic value. Therefore, diagnosis relies on a combination of clinical manifestations, laboratory abnormalities, and exclusion of alternative diagnoses, commonly using criteria such as Yamaguchi or Fautrel. Although serosal involvement has been reported, presentation with massive hemorrhagic pericardial effusion in the absence of the characteristic rash is extremely rare and may mimic tuberculosis, malignancy, or other rheumatic diseases. We report a 67-year-old Asian man who was referred to our department from a local hospital because of persistent cough, fever, and polyserositis predominantly characterized by massive hemorrhagic pericardial effusion. Laboratory evaluation revealed marked systemic inflammation, elevated ferritin levels, a positive interferon-gamma release assay, elevated adenosine deaminase in pericardial fluid, increased tumor markers, and anti-SAE1 positivity, initially raising suspicion for tuberculous pericarditis, malignancy, or autoimmune myopathy. However, comprehensive microbiological, cytological, imaging, and neuromuscular assessments found no evidence supporting these conditions, and the patient did not develop the typical evanescent rash of AOSD. After initiation of diagnostic glucocorticoid therapy, rapid defervescence and marked resolution of serous effusions were observed, supporting the diagnosis. This case highlights an atypical presentation of AOSD and emphasizes that AOSD should be considered in patients with unexplained serositis, particularly hemorrhagic pericardial effusion accompanied by systemic inflammation.

Uremic pericarditis is currently an uncommon complication of end-stage renal disease (ESRD) in developed countries due to improved access to renal replacement therapy and earlier initiation of hemodialysis. Nevertheless, delayed or interrupted dialysis may still result in life-threatening manifestations, including massive pericardial effusion and cardiac tamponade. We present the case of a 44-year-old man with ESRD requiring hemodialysis, insulin-dependent type 2 diabetes mellitus, morbid obesity, hypertension, and heart failure with preserved ejection fraction who was admitted because of progressive dyspnea, generalized weakness, and systemic deterioration. Following the recent initiation of hemodialysis, renal replacement therapy was interrupted because of recurrent dialysis catheter dysfunction and discontinuation of dialysis care at an outside center. On admission, the patient presented in moderate-to-severe clinical condition with profound fluid overload estimated at approximately 30 liters above dry weight, respiratory distress, an inflammatory response, severe azotemia, and metabolic acidosis. Physical examination was diagnostically challenging because diffuse pulmonary crackles, wheezes, and coarse breath sounds obscured potential pericardial friction rub, while morbid obesity limited assessment of jugular venous distention. Chest radiography demonstrated marked cardiomegaly with a globular "water bottle" configuration suggestive of massive pericardial effusion. Subsequent transthoracic echocardiography revealed a large circumferential pericardial effusion with echocardiographic signs of impending cardiac tamponade. Management included restoration and intensification of renal replacement therapy with repeated hemodialysis sessions and isolated ultrafiltration (IUF), broad-spectrum antimicrobial therapy, and multidisciplinary intensive care treatment. Pericardial fluid cultures remained sterile despite concomitant bloodstream infection associated with a severely contaminated dialysis catheter. Gradual clinical improvement was achieved with the reduction of hypervolemia and stabilization of hemodynamic status. This case highlights a rare but severe presentation of advanced uremic pericarditis occurring after interruption of renal replacement therapy. Despite being considered a largely historical complication in developed healthcare systems, severe uremic pericarditis should remain in the differential diagnosis in patients with ESRD presenting with progressive dyspnea, cardiomegaly, massive fluid overload, and hemodynamic instability.

Malignant pericardial effusion is a life-threatening manifestation of advanced non-small-cell lung cancer (NSCLC). Cardiac tamponade as an initial presentation is uncommon, and coexistent pathologies can complicate diagnosis and management.