Recurrent pericardial effusion and tamponade in a patient with Erdheim-Chester disease (ECD)

Mishra, Ajay Kumar; Mani, Sunithi; George, Anu Anna; Sudarsanam, Thambu David

doi:10.1136/bcr-2015-212483

Cited by 11 publications

(10 citation statements)

References 18 publications

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“…The latter can be seen in the form of pericarditis, asymptomatic pericardial effusion to life-threatening, rapid, recurrent pericardial tamponade. Recurrent pericardial effusions with tamponade caused by ECD, however, are extremely rare [7]. Mishra et al reported a patient who continued to experience clinical deterioration despite multiple pericardiocentesis and pericardial window [7].…”

Section: Discussionmentioning

confidence: 99%

“…Recurrent pericardial effusions with tamponade caused by ECD, however, are extremely rare [7]. Mishra et al reported a patient who continued to experience clinical deterioration despite multiple pericardiocentesis and pericardial window [7]. Only two cases of ECD with constrictive pericarditis have been reported in the literature [8, 9].…”

Section: Discussionmentioning

confidence: 99%

“…The histiocytes are positive for CD68 and negative for CD1a and S100 [1, 4]. Cytology from pericardiocentesis is usually non-diagnostic [7–9]. Tissue sampling is also necessary for the evaluation of BRAF mutations with immunohistochemical stain or genetic testing.…”

Section: Discussionmentioning

confidence: 99%

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Mysterious quad of constrictive pericarditis, recurrent pleural effusions, bone involvement and interstitial lung disease

Ding

Chahine

Deshwal

et al. 2019

Oxford Medical Case Reports

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Erdheim–Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytic neoplasm. The rarity of the disease and heterogenous clinical presentations often leads to delayed diagnosis. Historically, ECD lacked effective treatment and the prognosis was poor. Following the recent discovery of frequent BRAF-V600E mutation in patients with ECD, vemurafenib, a selective BRAF V600 kinase inhibitor has been approved for BRAF-mutated ECD patients. The prognosis of ECD has dramatically improved with early recognition of the disease and available treatment. ECD affects nearly every organ system. Cardiac involvement with pericardial effusion is common but rarely with constrictive physiology or requiring pericardiectomy. We present a case of a 56-year-old woman with recurrent pericarditis with constrictive physiology along with pleural effusion and interstitial lung disease that was diagnosed with ECD 3 years after initial presentation. The patient’s symptoms were relieved with pericardiectomy and targeted therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Mysterious quad of constrictive pericarditis, recurrent pleural effusions, bone involvement and interstitial lung disease

Ding

Chahine

Deshwal

et al. 2019

Oxford Medical Case Reports

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“…To the best of our knowledge, in all cases of symptomatic pericardial effusion due to ECD described in the medical literature, a first-line pharmacological approach with IFN-α has proven ineffective. In some cases, a surgical procedure such as pleural–pericardial window or pericardiectomy was needed; other patients developed cardiac tamponade despite IFN-α treatment and did not survive ( 25 , 26 ). The pericardial fluid from ECD patients with pericardial involvement is highly inflammatory, with increased levels of pro-inflammatory cytokines ( 14 , 28 ).…”

Section: Discussionmentioning

confidence: 99%

“…The traditional drug of choice in ECD is IFN-α ( 7 ). However, we preferred not to choose IFN-α, as previous experience suggests limited efficacy in cases of severe heart inflammation ( 25 , 26 ). As a valid alternative, we focused on vemurafenib, a small molecule inhibitor of BRAFV600E, which is highly effective in the treatment of ECD ( 27 ).…”

Section: Case Reportmentioning

confidence: 99%

Treating Heart Inflammation With Interleukin-1 Blockade in a Case of Erdheim–Chester Disease

et al. 2018

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Pericarditis is an inflammatory heart disease, which may be idiopathic or secondary to autoimmune or auto-inflammatory diseases and often leads to severe or life-threatening complications. Colchicine and non-steroidal anti-inflammatory drugs represent the mainstay of treatment, whereas use of corticosteroids is associated with recurrence of disease flares. While effective and safe anti-inflammatory therapies remain an unmet clinical need, emerging clinical and experimental evidence points at a promising role of inhibition of the pro-inflammatory cytokine interleukin-1 (IL-1). We thus evaluated treatment with the IL-1 receptor antagonist anakinra in a case of extremely severe pericarditis with cardiac tamponade and heart failure secondary to Erdheim–Chester disease (ECD), a rare clonal disorder of macrophages characterized by rampant inflammation and multiorgan involvement. A 62-year-old man was admitted to the Emergency Department with severe pericardial effusion requiring the creation of a pleuro-pericardial window. A whole-body contrast-enhanced computed tomography pointed at a diagnosis of ECD with involvement of the heart and pericardium and of the retroperitoneal space. Over the following days, an echocardiography revealed a closure of the pleuro-pericardial window and a relapse of the pericardial effusion. Treatment with anakinra, the recombinant form of the naturally occurring IL-1 receptor antagonist, was started at a standard subcutaneous dose of 100 mg/day. After 2 days, we observed a dramatic clinical improvement, an abrupt reduction of the inflammatory markers, and a reabsorption of the pericardial effusion. Anakinra was maintained as monotherapy, and the patient remained asymptomatic in the absence of disease flares for the following year. Recent studies point at inhibition of IL-1 activity as an attractive treatment option for patients with refractory idiopathic recurrent pericarditis. Anakinra treatment may also have a role in patients with pericarditis in the setting of systemic inflammatory disorders, such as ECD.

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A case report of cardiac amyloidosis presenting with chronic pericardial effusion and conduction block

John

Mishra

Iyyadurai

2019

European Heart Journal - Case Reports

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Background Amyloidosis is caused by the deposition of abnormal proteins in the extracellular space of various organs. The clinical features of amyloidosis depend on the type of amyloid protein and the organ system involved. Case summary A 51-year-old woman developed complete heart block which warranted a permanent pacemaker insertion. She was referred for evaluation of chronic pericardial effusion. The patient had stable vital signs and muffled heart sounds on examination of the cardiovascular system. Her chest X-ray film showed a permanent pacemaker in situ, and echocardiogram showed a chronic pericardial effusion without features of tamponade. On further evaluation, she was found to have an M band on serum electrophoresis, elevated free light chain ratio and amyloid deposits in bone marrow biopsy. Technetium pyrophosphate (Tc-PYP) scintigraphy was consistent with cardiac amyloidosis. Discussion Cardiac amyloidosis can have diverse clinical presentations. Chronic pericardial effusion and conduction block can be a rare presentation of cardiac amyloidosis and needs to be considered while evaluating the same. Cardiac magnetic resonance imaging and Tc-PYP imaging can be used in establishing the diagnosis of cardiac amyloidosis, if endomyocardial biopsy is not feasible.

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Recurrent pericardial effusion and tamponade in a patient with Erdheim-Chester disease (ECD)

Cited by 11 publications

References 18 publications

Mysterious quad of constrictive pericarditis, recurrent pleural effusions, bone involvement and interstitial lung disease

Mysterious quad of constrictive pericarditis, recurrent pleural effusions, bone involvement and interstitial lung disease

Treating Heart Inflammation With Interleukin-1 Blockade in a Case of Erdheim–Chester Disease

A case report of cardiac amyloidosis presenting with chronic pericardial effusion and conduction block

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