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Pleuroparenchymal fibroelastosis in Mycobacterium avium complex pulmonary disease: clinical characteristics and

Yuji Yamamoto^1,2, Kazuyuki Tsujino¹, Tomoki Kuge¹

¹Dept of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center, Toyonaka, Japan.

ERJ Open Research

|February 11, 2021

Abstract:

The association between Mycobacterium avium complex pulmonary disease (MAC-PD) and pleuroparenchymal fibroelastosis (PPFE) has been reported previously, and interstitial pneumonia as a comorbidity is associated with a worse prognosis. However, no study has thoroughly reported on PPFE associated with MAC-PD. The present study investigated the prevalence, clinical characteristics, and prognostic impact of PPFE in patients with MAC-PD. A total of 224 patients, newly diagnosed with MAC-PD, were retrospectively reviewed. At the time of diagnosis, chest high-resolution computed tomography (HRCT), sputum examination, and clinical characteristics were collected. The extent of PPFE and MAC-PD was evaluated semi-quantitatively using HRCT scores. Risk factor analysis for clinical or radiological deterioration necessitating multidrug antimicrobial treatment within 3 years, and all-cause mortality within 5 years, from the initial diagnosis was performed based on the PPFE score. PPFE was observed in 59 out of 224 patients (26.3%). A higher PPFE score was a risk factor for dyspnoea, fatigue, and lower body mass index (BMI) (p<0.05). Although PPFE score did not correlate with clinical or radiological deterioration within 3 years (p=0.576), a higher PPFE score (adjusted OR 1.66, 95% CI 1.06-2.60, p=0.028) and lower BMI (adjusted OR 0.61, 95% CI 0.39-0.94, p=0.028) increased the risk of 5-year mortality. PPFE is a relatively common complication and an independent poor prognostic factor of MAC-PD. This study highlights the need for further studies investigating whether the presence of PPFE can be a clinical indicator for initiating treatment of MAC-PD.

Insights

Pleuroparenchymal fibroelastosis (PPFE) is a common complication in Mycobacterium avium complex pulmonary disease (MAC-PD). Higher PPFE scores indicate a worse prognosis and increased risk of mortality in MAC-PD patients.

Area of Science:

Pulmonology
Infectious Diseases
Radiology

Background:

Mycobacterium avium complex pulmonary disease (MAC-PD) can be complicated by interstitial lung diseases, impacting patient prognosis.
Pleuroparenchymal fibroelastosis (PPFE) is a rare form of interstitial pneumonia, and its specific association with MAC-PD requires further investigation.
Previous reports suggest interstitial pneumonia comorbidities worsen MAC-PD outcomes, but detailed analysis of PPFE in MAC-PD is lacking.

Purpose of the Study:

To investigate the prevalence and clinical characteristics of PPFE in patients diagnosed with MAC-PD.
To evaluate the prognostic impact of PPFE on clinical and radiological deterioration and mortality in MAC-PD patients.
To identify risk factors associated with PPFE in the context of MAC-PD.

Main Methods:

Retrospective review of 224 patients newly diagnosed with MAC-PD.
Collection of clinical data, sputum examination, and chest high-resolution computed tomography (HRCT) at diagnosis.
Semi-quantitative evaluation of PPFE and MAC-PD extent using HRCT scores; risk factor analysis for deterioration and mortality based on PPFE score.

Main Results:

PPFE was identified in 59 out of 224 MAC-PD patients (26.3%).
Higher PPFE scores correlated with increased dyspnea, fatigue, and lower body mass index (BMI).
While PPFE score did not predict 3-year deterioration, it was an independent risk factor for 5-year all-cause mortality (adjusted OR 1.66), alongside lower BMI (adjusted OR 0.61).

Conclusions:

PPFE is a frequent complication of MAC-PD and serves as an independent predictor of poor prognosis.
The presence and severity of PPFE significantly impact long-term survival in MAC-PD patients.
Further research is warranted to determine if PPFE presence can guide early treatment initiation for MAC-PD.

Read full analysis

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