Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics

Chua, Felix; Desai, Sujal R.; Nicholson, Andrew G.; Devaraj, Anand; Renzoni, Elisabetta; Rice, Alexandra; Wells, Adrian

doi:10.1513/annalsats.201902-181cme

Cited by 130 publications

(198 citation statements)

References 60 publications

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“…Regarding the clinical features of pneumothorax in patients with PPFE, previous studies have reported that it is frequently recurrent and intractable [4-8, 13, 16, 18-20]. In this study, approximately half of the patients who developed pneumothorax had a recurrence; however, most were asymptomatic and small in size and resolved spontaneously.…”

Section: Discussionmentioning

confidence: 54%

“…In addition, among patients with idiopathic PPFE in this study, more cases with pneumothorax were treated with the immunosuppressive and/or anti-fibrotic agents than those without pneumothorax, suggesting a relationship between disease progression and the occurrence of pneumothorax. In some cases of pneumothorax associated with idiopathic PPFE, persistent air leak and poor re-expansion of the underlying lung were observed [5-8], which may be related to a poor prognosis [16, 18-20]. In this study, we demonstrated that patients with idiopathic PPFE who had pneumothorax had a significantly poorer prognosis than those who did not have pneumothorax, and pneumothorax was the cause of death in some cases ( n = 7).…”

Section: Discussionmentioning

confidence: 56%

“…Spontaneous secondary pneumothorax frequently occurs in patients with idiopathic PPFE and is considered to be one of their clinical features [1, 4-8]. The incidence of pneumothorax varied with the population studied and was reported within 25–89% [1, 9-21].…”

Section: Discussionmentioning

confidence: 99%

“…Pneumothorax frequently develops during the clinical course of idiopathic PPFE [4-8]. In the original report of IPUF [1], bilateral and/or recurrent pneumothorax developed in 7 (53.8%) of 13 patients, suggesting it was one of the clinical features of IPUF.…”

Section: Introductionmentioning

confidence: 99%

“…The subsequent case series of idiopathic PPFE showed that the incidence of pneumothorax after the diagnosis was 25–89% [9-21], which varied with the population studied. Since a postoperative iatrogenic pneumothorax commonly occurs in these patients and can cause a prolonged air leak [12, 16, 22], surgical lung biopsy (SLB) should be avoided [5-8]. Therefore, recent studies have proposed the clinical diagnostic criteria for PPFE without SLB [16, 23].…”

Section: Introductionmentioning

confidence: 99%

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Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors

et al. 2021

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Background: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare form of idiopathic interstitial pneumonia that is characterized by predominantly upper lobe pleural and subpleural lung parenchymal fibrosis. Pneumothorax is one of the major respiratory complications in PPFE patients; however, its clinical features are poorly understood. Objective: We aimed to investigate the complication of pneumothorax in patients with idiopathic PPFE. Methods: A retrospective multicenter study involving 89 patients who had been diagnosed with idiopathic PPFE was conducted. We investigated the cumulative incidence, clinical features, and risk factors of pneumothorax after the diagnosis of idiopathic PPFE. Results: Pneumothorax developed in 53 patients (59.6%) with 120 events during the observation period (41.8 ± 35.0 months). The cumulative incidence of pneumothorax was 24.8, 44.9, and 53.9% at 1, 2, and 3 years, respectively. Most events of pneumothorax were asymptomatic (n = 85; 70.8%) and small in size (n = 92; 76.7%); 30 patients (56.6%) had recurrent pneumothorax. Chest drainage was required in 23 pneumothorax events (19.2%), and a persistent air leak was observed in 13 (56.5%). Patients with pneumothorax were predominantly male and frequently had pathological diagnoses of PPFE and prior history of pneumothorax and corticosteroid use; they also had significantly poorer survival than those without pneumothorax (log-rank test; p = 0.001). Multivariate analysis revealed that a higher residual volume/total lung capacity ratio was significantly associated with the development of pneumothorax after the diagnosis. Conclusion: Pneumothorax is often asymptomatic and recurrent in patients with idiopathic PPFE, leading to poor outcomes in some cases.

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Section: Discussionmentioning

confidence: 54%

Section: Discussionmentioning

confidence: 56%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors

et al. 2021

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Pleuroparenchymal fibroelastosis as a late complication of childhood cancer therapy: A case series

Marathe,

Santibanez,

Meyers

et al. 2024

Pediatric Blood & Cancer

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Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial pneumonia with distinct clinicopathologic features. It has been associated with exposure to hematopoietic stem cell transplantation (HSCT) and classical alkylating agents. Here, we highlight PPFE as a late complication of childhood cancer therapy by describing the cases of four survivors of childhood cancer with a diagnosis of treatment‐related PPFE. All patients received high‐dose alkylating agents. PPFE should be considered in the differential diagnosis of restrictive lung disease in patients with history of exposure to alkylating agents or HSCT. Development of PPFE‐specific, noninvasive diagnostic tools and disease‐modifying therapies will clinically benefit these patients.

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Major Scleroderma Emergencies

Ong¹,

Denton²

2020

In Clinical Practice

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Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics

Cited by 130 publications

References 60 publications

Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors

Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors

Pleuroparenchymal fibroelastosis as a late complication of childhood cancer therapy: A case series

Major Scleroderma Emergencies

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