Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact

Abstract

<jats:p>Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a major cause of morbidity and mortality, mostly presenting as non-specific interstitial pneumonia. Little is known about the prevalence of pleuroparenchymal fibroelastosis (PPFE), a specific entity affecting the visceral pleura and subpleural parenchyma. We set out to estimate PPFE prevalence in two large cohorts of SSc patients and to assess its impact on survival and functional decline.</jats:p><jats:p>A total of 359 SSc patients, derived from two referral centres in two different countries (UK and Italy), were included. The first available high-resolution computed tomography scan was independently evaluated by two radiologists blind to clinical information, to quantify ILD extent, freestanding bronchial abnormalities, and lobar percentage involvement of PPFE on a four-point categorical scale. Discordant scores were adjudicated by a third scorer. PPFE extent was further classified as limited (≤2/18) or extensive (&gt;2/18). Results were evaluated against functional decline and mortality.</jats:p><jats:p>The overall prevalence of PPFE in the combined SSc population was 18% (11% with extensive PPFE), with no substantial difference between the two cohorts. PPFE was significantly linked to free-standing bronchial abnormalities (61% <jats:italic>versus</jats:italic> 25% in PPFE <jats:italic>versus</jats:italic> no PPFE; p&lt;0.0001) and to worse survival, independently of ILD severity or short-term lung function changes (HR 1.89, 95% CI 1.10–3.25; p=0.005).</jats:p><jats:p>In the current study, we provide an exhaustive description of PPFE prevalence and clinical impact in the largest cohort of SSc subjects published so far. PPFE presence should be carefully considered, due to its significant prognostic implications.</jats:p>

Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment

“…The study findings confirm observations in patients with scleroderma and chronic HP that demonstrated worsened survival with the presence of PPFE [7,8]. However, in neither study was PPFE associated with accelerated FVC decline.…”

“…Visual PPFE was defined as pleurally-based triangular dense opacities (Fig. 2), occurring caudal to the upper 0.5 cm of the lung apices as per previous definitions [7,8]. Visual PPFE was quantified on a…”

Pleuroparenchymal fibroelastosis inMycobacterium aviumcomplex pulmonary disease: clinical characteristics and prognostic impact

“…PPFE accompanied with MAC-PD is a risk factor for lower BMI. Although weight loss and low BMI were reported in idiopathic PPFE and in the disease secondary to systemic sclerosis and idiopathic pulmonary fibrosis [11,13,24], no study has yet reported on the clinical impact of PPFE on BMI in patients with PPFE associated with MAC-PD. Consistent with other studies of secondary PPFE, the present study demonstrated that patients with MAC-PD and PPFE had a lower BMI (Tables 2 and 3).…”

“…PPFE is a specific clinical-pathological entity that Amitani et al and Frankel et al first reported [8,9]. Its pathogenesis remains unclear, but earlier studies reported that PPFE was associated with a variety of diseases, including autoimmune diseases, aspergillosis and NTM infection [7,[10][11][12][13]. Moreover, patients with PPFE and granulomatous diseases, including aspergillosis and MAC infection, had a better prognosis than those with PPFE without granulomas [7].…”

“…In particular, no study has evaluated the prevalence, clinical and radiological characteristics, and prognostic impact of PPFE in patients with MAC-PD. Since the presence of PPFE indicates a poor prognosis in other diseases [11,13], we hypothesized that PPFE might induce severe clinical symptoms and be associated with a poor prognosis in patients with MAC-PD.…”