An unusual case of interstitial lung disease in a patient with cardiopulmonary syndrome as the initial presentation of Erdheim-Chester disease

Franco-Palacios, Domingo; McDonald, April; Aguillard, R. Neal; Berry, Allen D.

doi:10.1136/bcr-2017-220659

Cited by 5 publications

(2 citation statements)

References 14 publications

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“…Interstitial lung disease (ILD) refers to a group of lungs diseases that affect the interstitium, which is the tissue that surrounds and supports the air sacs (alveoli) in the lungs. The interstitial is composed of network of tiny blood vessels, elastic fibres, and collagen fibres that are responsible for exchanging oxygen and carbon dioxide between the lungs and the blood stream [1,2].…”

Section: Introductionmentioning

confidence: 99%

Case study: interstitial lung disease with pulmonary arterial hypertension and COR Pulmonale

Seru

Mohan

et al. 2023

Asian Jour Hosp Phar

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Interstitial lung diseases (ILDs) encompass a diverse group of conditions characterized by inflammation and fibrosis of the lung tissue, which can lead to impaired lungs function and respiratory failure. Pulmonary arterial hypertension (PAH) and cor pulmonale are common complications of ILDs, resulting from increased resistance in pulmonary circulation and right heart strain. ILDs and PAH and cor pulmonale are often difficult to diagnoses as the symptoms can be non-specific and overlap with other respiratory diseases. Diagnostic tool such as pulmonary function tests, imaging studies and right heart catheterization are used to establish a definitive diagnosis and assess to disease severity. This case study summarizes the patient case of ILDs with PAH and cor pulmonale, including the underlying pathophysiology, diagnostic tool and management strategies. Additionally, we discuss the challenges in diagnosing and managing ILDs with PAH and cor pulmonale, as well as potential future direction in research and treatment.

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Section: Introductionmentioning

confidence: 99%

Case study: interstitial lung disease with pulmonary arterial hypertension and COR Pulmonale

Seru

Mohan

et al. 2023

Asian Jour Hosp Phar

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“…It was first described in 1930 by Dr. William Chester and Dr. Jakob Erdheim, and there have been around 600 cases reported in the literature since then. ECD cases have increased dramatically in the last decade due to increased physicians' awareness about the diagnosis and, recently, It has been reclassified as histiocytic dendritic cell neoplasm [ 1 ]. It is a multisystem disease with a poorly understood etiology; most articles in the literature reported proto-oncogene BRAF V600E mutation in patients with ECD between 38% and 68%, and 100% using more sensitive tests in a recent article, which strongly supports possible clonal origin [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Malignant Otitis Externa in a 20-Year-Old Male Patient With Erdheim-Chester Disease: A Case Report

Alarimah¹,

Alanazi²,

Alshammari³

et al. 2021

Cureus

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Malignant otitis externa (MOE) is an uncommon, potentially life-threatening, invasive infection of the external auditory canal and lateral skull base. It is mainly seen in older adults with diabetes or immunocompromised patients and rarely seen in children.Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis disorder. It is a multisystem disease with a poorly understood etiology. It commonly affects the skeletal system, central nervous system (CNS), cardiovascular system, lungs, retroperitoneum, kidneys, and skin. CNS involvement confers poor prognosis and reduced response to treatment. Cardiovascular involvement is another indicator of poor prognosis. This report describes the case of a 20-year-old male with ECD who had bilateral malignant otitis externa/skull base osteomyelitis and hearing loss. The patient was not responding well to treatment. He was on intravenous antibiotics, underwent left mastoidectomy, received targeted immunotherapy, and had four rounds of chemotherapy. The patient died after six months from the diagnosis and targeted treatment, which indicates the aggressiveness of the disease.MOE should be suspected in any patient presenting with intractable otalgia with otorrhea that is not responsive to local treatment for uncomplicated otitis externa or otalgia with temporomandibular joint (TMJ) pain aggravated by chewing. This is the first report of malignant otitis externa in a young patient with ECD to the best of the author's knowledge. This case emphasizes the importance of suspecting MOE in young patients, especially if the risk factors of the disease are present because early diagnosis can prevent or minimize life-threatening complications.

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PIRO-CIC model can predict mortality and futility of care in critically ill cirrhosis patients in the intensive care unit

et al. 2022

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An unusual case of interstitial lung disease in a patient with cardiopulmonary syndrome as the initial presentation of Erdheim-Chester disease

Cited by 5 publications

References 14 publications

Case study: interstitial lung disease with pulmonary arterial hypertension and COR Pulmonale

Case study: interstitial lung disease with pulmonary arterial hypertension and COR Pulmonale

Malignant Otitis Externa in a 20-Year-Old Male Patient With Erdheim-Chester Disease: A Case Report

PIRO-CIC model can predict mortality and futility of care in critically ill cirrhosis patients in the intensive care unit

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