Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterised by predominantly upper lobe pleural and subjacent parenchymal fibrosis, the latter being intraalveolar with accompanying elastosis of the alveolar walls. The aim of this study was to review cases fulfilling published imaging and histological criteria, and identify any common clinical features that may suggest an underlying aetiology for a condition that has previously been regarded as idiopathic.Of 12 patients (seven females, median age 57 yrs), the presenting symptoms were shortness of breath (11 out of 12 patients) and dry cough (six out of 12 patients). Seven patients reported recurrent infections during the course of their disease. Five demonstrated nonspecific autoantibody positivity. Two patients had a family history of interstitial lung disease (ILD).High-resolution computed tomography features of lung disease remote from the pleuroparenchymal changes were present in six out of 12 patients (coexistent fibrosis, n55; bronchiectasis, n51). Of seven patients with tissue sampled from the lower lobes, four patients showed less intense PPFE changes (one with additional features of hypersensitivity pneumonitis) and three showed usual interstitial pneumonia.PPFE is a distinct clinicopathological entity, with clinical data suggesting a link to recurrent pulmonary infection. Genetic and autoimmune mechanisms may also contribute to the development of these changes. PPFE may also present with more diffuse involvement than previously reported, and coexist with different patterns of ILD.
Pulmonary complications in CTD are common and can involve the interstitium, airways, pleura and pulmonary vasculature. ILD can occur in all CTD (CTD-ILD), and may vary from limited, non-progressive lung involvement, to fulminant, life-threatening disease. Given the potential for major adverse outcomes in CTD-ILD, accurate diagnosis, assessment and careful consideration of therapeutic intervention are a priority. Limited data are available to guide management decisions in CTD-ILD. Autoimmunemediated pulmonary inflammation is considered a key pathobiological pathway in these disorders, and immunosuppressive therapy is generally regarded the cornerstone of treatment for severe and/or progressive CTD-ILD. However, the natural history of CTD-ILD in individual patients can be difficult to predict, and deciding who to treat, when and with what agent can be challenging. Establishing realistic therapeutic goals from both the patient and clinician perspective requires considerable expertise. The document aims to provide a framework for clinicians to aid in the assessment and management of ILD in the major CTD. A suggested approach to diagnosis and monitoring of CTD-ILD and, where available, evidence-based, diseasespecific approaches to treatment have been provided.
These data suggest that a CT pattern of patchy areas of reticular changes about predominantly upper zone emphysematous spaces may be seen in smokers who do not have clinical evidence of a diffuse ILD. We propose that this lesion be called respiratory bronchiolitis with fibrosis (RBF) to avoid confusion with other forms of ILD. RBF probably accounts for some of the cases of ILD seen in large radiologic surveys of smokers. The pathology literature indicates that RBF either has no functional effects or at worst represents mild, usually nonprogressive disease, and hence separation from other, more serious, forms of ILD is important. Recognition of this lesion on imaging may obviate the need for lung biopsy.
Diffuse dendriform pulmonary ossification is a rare disorder characterized by widespread pulmonary ossification distributed in a characteristic branching pattern. It is usually associated with chronic interstitial inflammation and fibrosis but may occasionally be idiopathic. The underlying pathogenesis remains uncertain. Computed tomography findings characteristically show fine branching heterotopic bone formation within the lungs, often in a lower lobe distribution. A case of idiopathic dendriform ossification is described in a 34-year-old man who presented for investigation of hemoptysis.
Background We report the prevalence and progression of incidentally detected interstitial lung abnormalities (ILA) in the Queensland Lung Cancer Screening Study cohort. Methods About 256 volunteers aged 60–74, with ≥30 pack years smoking history and forced expiratory volume in 1 s (FEV1) ≥50% predicted underwent low‐dose computed tomography (CT) chest screening. Electronic search of baseline (T0) and 2‐year follow‐up (T2) CT reports identified candidate cases using Fleischner Society interstitial terminology. Candidate CT were reviewed in a randomised order by two experienced radiologists and a senior respiratory medicine trainee blinded to the existing reports. Scans were evaluated for the presence and extent of ILA using an in‐house score, and graded for progression. Results ILA were detected in 20/256 baseline cases (7.8%) with no incident cases detected at T2 surveillance imaging. Of these 20 cases, 9 (45%) had reticulation, 18 (90%) had ground glass change, 1 had traction bronchiectasis and 1 had randomly distributed nodularity. Seven cases with ground glass changes also had areas of reticulation, and only two had reticulation alone. All ILA were graded as minor except for traction bronchiectasis, which was moderate. Only one case progressed on T2 imaging. ILA were associated with the presence of auscultatory crackles (50% vs 11.6%, P = 0.001) and a lesser degree of emphysema (mean % volumetric emphysema 6.7% vs 9.8%, P = 0.009). No relationship was observed between baseline and serial lung function parameters. Conclusion ILA are frequent incidental findings in lung cancer screening. In the majority of cases these abnormalities do not appear to change significantly over a 2‐year period of surveillance.
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