Multisystem Erdheim-Chester Disease With Extensive Pericardial and Vascular Involvement

Sánchez-Nadales, Alejandro; Wang, Tom Kai Ming; Anampa-Guzmán, Andrea; Xu, Bo

doi:10.1161/circimaging.119.010123

Cited by 4 publications

(3 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This most commonly manifests as infiltration of the pericardium and myocardium with a tendency to involve the right atrial myocardium, often with a pseudotumor appearance ( 5 , 6 ). Extensive vascular involvement of the thoracic aorta and SVC has also been reported ( 7 ). Typical electrocardiographic abnormalities that have been reported include shortening of the PR interval, sinoatrial block, sinus bradycardia, Q-wave abnormalities, and ST-T wave abnormalities ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

Erdheim-Chester Disease

et al. 2020

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Erdheim-Chester Disease

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Vascular involvement is rare in patients with ECD. Among all reported cases with arterial disease, including that affecting the coronary artery, cerebral artery, thoraco-abdominal aorta, subclavian artery, and renal artery, periarteritis is the main manifestation [ 14 – 19 ], In a 53-year-old woman with ECD, both the thoracic aorta and superior vena cava have been found to be involved [ 19 ]. A successful case [ 20 ] of use of the MEK inhibitor Cobimetinib in the treatment of multiple arterial thrombosis in a 71-year-old man with underlying diseases such as hypertension and myeloproliferative neoplasm has been reported.…”

Section: Discussionmentioning

confidence: 99%

Rare presentation of multiple thromboses in Erdheim–Chester disease: a case-based review

Liu

Huang²,

Meng

et al. 2022

Int J Hematol

View full text Add to dashboard Cite

Background ECD is a rare non-Langerhans cell histiocytosis with diverse and heterogeneous clinical manifestations, ranging from single-lesion forms to multi-system involvement, including slowly progressing unifocal forms to rapidly evolving life-threatening disease. Case presentation A female patient presented with a 2-month history of fever. Imaging revealed multiple thromboses, bone destruction, an abnormal pituitary stalk, and clinical manifestations of diabetes insipidus. Excisional biopsy of a tibial lesion was sent for microscopic examination, and subsequent immunohistochemical testing was positive for expression of CD68 and CD163, and negative for expression of the immune markers CD1a, S100, and langerin. This confirmed the diagnosis of ECD. Treatment with methylprednisolone to inhibit the immune inflammatory response along with anti-cytokine therapy with an interleukin-6 antagonist resulted in satisfactory disease control. Conclusion We report a rare case of multiple thromboses, embolism, and multiple organ involvement as the main presentation of ECD, suggesting that ECD should be considered in patients presenting with multiple thromboses associated with multisystem damage. We successfully treated our patient with glucocorticoids and interleukin-6 antagonist. This patient’s response to treatment suggests that hormone therapy and cytokine/chemokine therapy may be a potential novel treatment for patients with ECD without gene mutations.

show abstract

“…A segunda categoria de manifestações foram as manifestações cardiovasculares, presentes em 19 relatos. Entre essas manifestações, foram relatadas: palpitações, derrame pericárdico transitório ou recorrente, tamponamento cardíaco, miocardite, nódulos intracavitários e em miocárdio e pericárdio aos exames de imagem, coarctação de aorta, estenose de veia cava superior, cardiomiopatia isquêmica aguda e aortite (5,6,(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33). Um dos relatos que recebe grande destaque é o de Costa et al em que a primeira manifestação da doença foi um episódio de tamponamento cardíaco.…”

Section: Discussionunclassified

Doença de Erdheim Chester com envolvimento isolado do SNC

Rodrigues¹,

Ribeiro²,

Faria³

et al. 2022

Braz. J. Hea. Rev.

View full text Add to dashboard Cite

Introdução: A Doença de Erdheim-Chester é uma forma de histiocitose de células não-Langerhans que ocorre mais frequentemente após os 40 anos de idade, com leve predomínio no sexo masculino. Apresentação do caso: Paciente, sexo masculino, branco, X anos de idade, admitido no Hospital das Clínicas de Goiânia, com sintomas de cefaléia crônica, de caráter progressivo de inicio há 3 meses, associados a sintomas neurológicos de ataxia e diplopia. Referiu febre baixa diária, não aferida, com perda de 10kg no período descrito, não associado a dieta ou mudança de hábitos de vida. Nega comorbidades ou uso de medicamentos. Ao exame físico, apresenta quadro de Nistagmo e Romberg positivo e alteração da úvula para direita. Paciente não apresentava outros sinais dignos de nota. Discussão: Foram selecionados para a revisão, os artigos de relato de caso ou séries de caso relacionados a manifestações clínicas da doença de Erdheim-Chester. Os principais tipos manifestações clínicas relatadas na literatura são: neurológicas, cardiovasculares, oftálmicas, endócrinas, urinárias, hematológicas, ósseas, cutâneas, respiratórias, gastrointestinais, envolvimento de múltiplos sistemas e outras. Entre esses tipos de manifestações, as mais frequentes foram neurológicas, cardiovasculares e oftálmicas, correspondendo a 48% das manifestações relatadas. Conclusão: É fundamental ter uma equipe multidisciplinar acompanhando o paciente, para que sejam evitadas complicações e se possa proporcionar qualidade de vida ao paciente.

show abstract

Multisystem Erdheim-Chester Disease With Extensive Pericardial and Vascular Involvement

Cited by 4 publications

References 4 publications

Erdheim-Chester Disease

Erdheim-Chester Disease

Rare presentation of multiple thromboses in Erdheim–Chester disease: a case-based review

Doença de Erdheim Chester com envolvimento isolado do SNC

Contact Info

Product

Resources

About