Erdheim-Chester Disease Presenting with Pneumothorax

Yamaguchi, Masafumi; Shiota, Tetsuhiro; Kobashi, Yoichiro

doi:10.1159/000329872

Cited by 5 publications

(3 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Other neoplastic processes such as Erdheim-Chester Disease (47), pleuropulmonary blastoma (48, 49), mesenchymal cystic hamartoma (50), adenocarcinoma and squamous cell carcinoma of the lung (51, 52) can rarely cause cystic lung changes and pneumothorax.…”

Section: Introductionmentioning

confidence: 99%

Spontaneous pneumothorax in diffuse cystic lung diseases

Cooley

Lee

Gupta

2017

Current Opinion in Pulmonary Medicine

View full text Add to dashboard Cite

Purpose of review Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis (LAM), Birt-Hogg-Dubé syndrome (BHD), and pulmonary Langerhans cell histiocytosis (PLCH). Recent findings DCLDs are responsible for approximately 10% of apparent primary spontaneous pneumothoraces. CT screening for DCLDs (BHD, LAM, and PLCH) following the first spontaneous pneumothorax has recently been shown to be cost-effective and can help facilitate early diagnosis of the underlying disorders. Patients with DCLD-associated spontaneous pneumothorax have a very high rate of recurrence, and thus pleurodesis should be considered following the first episode of spontaneous pneumothorax in these patients, rather than waiting for a recurrent episode. Prior pleurodesis is not a contraindication to future lung transplant. Summary Although DCLDs are uncommon, spontaneous pneumothorax is often the sentinel event that provides an opportunity for diagnosis. By understanding the burden and implications of pneumothoraces in DCLDs, clinicians can facilitate early diagnosis and appropriate management of the underlying disorders.

show abstract

Section: Introductionmentioning

confidence: 99%

Spontaneous pneumothorax in diffuse cystic lung diseases

Cooley

Lee

Gupta

2017

Current Opinion in Pulmonary Medicine

View full text Add to dashboard Cite

show abstract

“…1 Pathologic examination of cases is important because SP may be the initial presentation in a variety of conditions. [2][3][4] Some of those diseases are well described and have clearly recognizable pathologic features, such as lymphangioleiomyomatosis or malignancy. Others are just recently being recognized, both clinically and pathologically, for example Birt-Hogg-Dubé syndrome.…”

mentioning

confidence: 99%

A Unique, Histopathologic Lesion in a Subset of Patients With Spontaneous Pneumothorax

Belchis

Shekitka

Gocke

2012

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

Context.-Spontaneous pneumothorax can be idiopathic (primary), or it can occur in association with an underlying predisposing condition (secondary). Spontaneous pneumothorax may be a harbinger of an undiagnosed clinical condition, which may be associated with serious systemic abnormalities, making early recognition and diagnosis important. The pulmonary pathology of some of these disorders has not been fully elucidated.Objective.-To review cases of pneumothorax in the hope of identifying pathologic features that might correlate to specific clinical syndromes.Design.-The pathology computer files at 3 hospitals were searched for all cases of spontaneous pneumothorax, primary and secondary, regardless of etiology during a 11-year period. Ninety-two cases were retrieved. Each of the cases was evaluated for reactive eosinophilic pleuritis, elastosis, pleural fibrosis, emphysema, intra-alveolar macrophages, cholesterol clefts, vasculopathy, and intraparenchymal or intrapleural cysts. Clinical information regarding asthma and smoking history, site of the pneumothorax, family history, radiographic findings, predisposing conditions, recurrence, age, and sex were extracted from the medical records.Results.-In 11 patients (12% of all the patients with spontaneous pneumothorax), a distinctive pattern of pleural fibrosis with islands of fibroblastic foci within a myxoid stroma was noted at the pleural-parenchymal interface or leading edge. These lesions correlated with a select subset of patients, consisting predominantly of young men.Conclusions.-Our review identified a distinct pattern of pneumothorax-associated fibroblastic lesions in a subset of cases of spontaneous pneumothorax. Whether this is related to the pathogenesis of the pneumothorax remains to be elucidated.

show abstract

“…The prevalence of lung involvement is variable in the literature [1,4,5,6]. One case series reported 53% of patients having lung involvement [6].…”

Section: Discussionmentioning

confidence: 99%