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New development of cardiac tamponade on underlying effusive-constrictive pericarditis: an uncommon initial presentation of scleroderma.

ABSTRACT: A 40-year-old man with a medical history of hypertension was admitted for weight loss, generalised weakness, joint pains and mottling of fingertips. The initial laboratory data revealed microangiopathic haemolytic anaemia, thrombocytopenia and acute renal failure. Intravenous steroids were started for possible diagnosis of systemic lupus erythematosus based on admission assessment. Intravenous immunoglobulin and plasmapharesis were subsequently added to the treatment plan to cover thrombotic thrombocytopenic purpura while his autoimmune panel was pending. The echocardiogram study on day 2 revealed cardiac tamponade for which he underwent pericardiocentesis and right heart catheterisation. The atrial waveforms postpericardiocentesis demonstrated effusive-constrictive pericarditis. His clinical condition kept on deteriorating with reaccumulation of pericardial effusion and further complicated by hemoperitoneum and colonic obstruction. He had cardiorespiratory arrest on his fourth admission day and was not revived. Anti-Scl-70 antibody came back positive. Autopsy findings confirmed the presence of fibrinous pericarditis and hemoperitoneum.

SUBMITTER: Subramanian SR

PROVIDER: S-EPMC3736295 | biostudies-other | 2013

REPOSITORIES: biostudies-other

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New development of cardiac tamponade on underlying effusive-constrictive pericarditis: an uncommon initial presentation of scleroderma.

Subramanian Stalin R SR Akram Rakhshanda R Velayati Arash A Chadow Hal H

BMJ case reports 20130712

A 40-year-old man with a medical history of hypertension was admitted for weight loss, generalised weakness, joint pains and mottling of fingertips. The initial laboratory data revealed microangiopathic haemolytic anaemia, thrombocytopenia and acute renal failure. Intravenous steroids were started for possible diagnosis of systemic lupus erythematosus based on admission assessment. Intravenous immunoglobulin and plasmapharesis were subsequently added to the treatment plan to cover thrombotic thr ...[more]

PMID: 23853085

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Project description:BackgroundPurulent bacterial pericarditis (PBP) is a highly lethal infection of the pericardial space that arises as a complication of infective illnesses. Purulent bacterial pericarditis remains a diagnostic challenge given its non-specific clinical and investigative features and carries exceedingly high mortality rates due to fulminant sepsis and morbidity including constrictive pericarditis in survivors. We present our management of cardiac tamponade and subsequent constrictive pericarditis due to Actinomyces meyeri PBP.Case summaryA 53-year-old Caucasian male presented with acute New York Heart Association Class IV dyspnoea and chest discomfort, in the context of multiple hospital presentations over the preceding 8 weeks due to presumed recurrent viral pericarditis. On this admission, initial transthoracic echocardiography (TTE) demonstrated a large asymmetric pericardial effusion for which he underwent urgent pericardiocentesis. Serial TTE post-pericardiocentesis, however, demonstrated effusion re-accumulation and effusive-constrictive pericarditis, confirmed on cardiac magnetic resonance imaging. Fluid culture was positive for A. meyeri. He was diagnosed with PBP, but his condition deteriorated despite appropriate intravenous antibiotic therapy, necessitating semi-urgent surgical pericardiectomy. He recovered well and was discharged on Day 10 post-operatively.DiscussionUnlike uncomplicated acute viral or idiopathic pericarditis, PBP portends a very poor prognosis if unrecognized and untreated. Diagnostic challenges persist given its rarity in modern clinical practice; however, PBP should be considered in cases of seemingly recurrent pericarditis. Multi-modal cardiac imaging and careful analysis of pericardial fluid including cultures and lactate dehydrogenase/serum ratios may assist in earlier recognition. In this case, source control and symptom relief were achieved only with combined intravenous antibiotics, surgical evacuation, and pericardiectomy.

Project description:Immunoglobulin G4-related disease (IgG4-RD) has recently been recognized as a systemic fibroinflammatory disease characterized by IgG4-positive lymphoplasmacytic infiltration. Despite the increased awareness of this disease category, cardiovascular involvement has rarely been reported. An 83-year-old man was admitted because of a moderate amount of pericardial effusion concomitant with bilateral pleural effusion. Despite aggressive heart failure therapy, pericardial effusion increased. He underwent pericardial drainage, and heart failure symptoms significantly improved. After only 1 month, right-sided heart failure with re-accumulation of pericardial fluid recurred, although the results of cardiac catheter examination were not indicative of constrictive pericarditis. Despite the aggressive medication, he developed weight gain and pleural effusion 6 months after pericardial drainage. Based on multimodal hemodynamic and morphological evaluations, he was finally diagnosed with effusive constrictive pericarditis and underwent total pericardiectomy. Based on histopathological findings, the final diagnosis was established as IgG4-RD associated constrictive pericarditis. We report an extremely rare case of IgG4-RD associated pericardial involvement, in which rapid transition from cardiac tamponade to effusive constrictive pericarditis was confirmed. Diagnostic work-up for constrictive pericarditis requires a high index of suspicion. Increased recognition of IgG4-RD associated pericardial involvement could potentially mitigate morbidity and might contribute to improved patient outcomes. <Learning objective: Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by chronic IgG4-positive lymphocyte infiltration that causes fibrosclerotic changes in various tissues and organs. Detailed serial multimodal imaging and physical examination contribute to the recognition of latent constrictive physiology and clinical decision-making. Increased recognition of IgG4-RD associated pericardial involvement could lead to early diagnosis and might contribute to improved patient outcomes.>.

Project description:BackgroundConstrictive pericarditis represents a chronic condition and systemic inflammatory diseases are a known, yet uncommon, cause. Pericardial involvement is seldom reported in primary Sjögren's syndrome, usually occurring in association with pericardial effusion or pericarditis. We report a case of constrictive pericarditis with an insidious course and unusual evolution associated with primary Sjögren's syndrome. Due to the challenging nature of the diagnosis, clinical suspicion and multimodality imaging are essential for early identification and prompt initiation of treatment. Long-term outcomes remain uncertain. To the best of our knowledge, no other cases linking this autoimmune disease to constrictive pericarditis have been reported.Case presentationWe present the case of a 48-year-old male patient with moderate alcohol habits and a history of two prior hospitalizations. On the first, the patient was diagnosed with primary Sjögren's syndrome after presenting with pleural effusion and ascites, and empirical corticosteroid regiment was initiated. On the second, two-years later, he was readmitted with complaints of dyspnea and abdominal distension. Thoracic computed tomography revealed a localized pericardial thickening and a thin pericardial effusion, both of which were attributed to his rheumatic disease. A liver biopsy showed hepatic peliosis, which was considered to be a consequence of glucocorticoid therapy. Diuretic therapy was adjusted to symptom-relief, and a tapering corticosteroid regimen was adopted. Four years after the initial diagnosis, the patient was admitted again with recurrent dyspnea, orthopnea and ascites. At this time, constrictive pericarditis was diagnosed and a partial pericardiectomy was performed. Although not completely asymptomatic, the patient reported clinical improvement since the surgery, but still with a need for baseline diuretic therapy.ConclusionAlbeit uncommon, connective tissue disorders, such as primary Sjögren's syndrome, should be considered as a potential cause of constrictive pericarditis, especially in young patients with no other classical risk factors for constriction. In this case, after excluding possible infectious, neoplastic and autoimmune conditions, a primary Sjögren´s syndrome in association with constrictive pericarditis was assumed. This case presents an interesting and challenging clinical scenario, highlighting the importance of clinical awareness and the use of multimodal cardiac imaging for early recognition and treatment.

Dataset Information

New development of cardiac tamponade on underlying effusive-constrictive pericarditis: an uncommon initial presentation of scleroderma.

Publications

New development of cardiac tamponade on underlying effusive-constrictive pericarditis: an uncommon initial presentation of scleroderma.

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