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

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.

Prognostic significance of radiological pleuroparenchymal fibroelastosis inMycobacterium aviumcomplex lung disease: a multicentre retrospective cohort study

“…In this study, the presence of radiological PPFE was an independent prognostic factor in patients with MAC lung disease, even after adjustment for these already-reported prognostic factors, the presence of ILD comorbidity and treatment exposure. Similar results were reported in a single-centre retrospective study,16 but the study had statistical limitations owing to its small sample size and results based on a logistic analysis of a binary variable of whether the patients died within 5 years, rather than a time-to-event model (ie, Cox proportional hazards model). Additionally, the study did not report data on cumulative survival or cause of death.…”

“…In this study, none of the patients with radiological PPFE had scleroderma or hypersensitivity pneumonitis, and few of them had CTD. For NTM, a single-centre retrospective study reported that radiological PPFE was found in 59 (26.3%) of 224 patients with MAC lung disease 16. Our multicentre study involving 850 patients showed that the prevalence of radiological PPFE was 11.9%, demonstrating that radiological PPFE is a common HRCT feature in MAC lung disease.…”

“…Patients with PPFE have unique chest high-resolution CT (HRCT) findings characterised by dense subpleural consolidation with traction bronchiectasis and architectural distortion, primarily in the upper lobes of the lungs 13–15. Such HRCT features, which are radiological PPFE, coexisted in patients with an IIP other than idiopathic PPFE and those with various pulmonary diseases, including hypersensitivity pneumonitis 10 16–18. An association between PPFE and recurrent respiratory infections has also been suggested; however, evidence regarding the association between PPPE and MAC lung disease is insufficient 16 19.…”

“…Such HRCT features, which are radiological PPFE, coexisted in patients with an IIP other than idiopathic PPFE and those with various pulmonary diseases, including hypersensitivity pneumonitis 10 16–18. An association between PPFE and recurrent respiratory infections has also been suggested; however, evidence regarding the association between PPPE and MAC lung disease is insufficient 16 19. We conducted this large-scale multicentre retrospective study to investigate the prevalence and associated mortality of radiological PPFE in patients with MAC lung disease.…”

Ground-glass opacity caused by the administration of amikacin liposome inhalation suspension: A case report

“…During the chronic course of refractory nontuberculous mycobacterial pulmonary disease (NTM-PD), the disease is sometimes exacerbated with the appearance of various opacities on chest radiography [1]. The differential diagnosis in such cases is challenging because it includes various conditions such as expansion of the NTM-PD lesion, pneumonia caused by other bacteria or fungi [2], airway bleeding [3], and interstitial pneumonia, including idiopathic [4] or drug-induced interstitial lung disease (DIILD) [5][6][7].…”