Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand.

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. Autoimmune-mediated 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, disease-specific approaches to treatment have been provided.

Interstitial lung abnormalities in the Queensland Lung Cancer Screening Study: prevalence and progression over 2 years of surveillance.

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.

Delayed cardiac herniation after left pneumonectomy.

Cardiac herniation is a rare potentially life-threatening complication that can occur after pneumonectomy, involving displacement of the heart through a pericardial defect, which can lead to hemodynamic instability, impaired cardiac function, and in severe cases, death. We describe a case of delayed cardiac herniation 1-month post left pneumonectomy for pulmonary leiomyosarcoma.

A rare case of true thymic hyperplasia in an adult.

True thymic hyperplasia (TTH) is a rare cause of a fat-containing mediastinal mass, most commonly found in infants and young children. We report a case of TTH in a 22-year-old adult, successfully managed via minimally invasive video-assisted thoracoscopic surgery.

Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes.

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterised by predominantly upper lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar 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, n=5; bronchiectasis, n=1). 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.

The IRIS paradox: Imaging findings in a case of PJP-IRIS.

Immune reconstitution inflammatory syndrome (IRIS) in patients with human immunodeficiency virus (HIV) and Pneumocystis jirovecii pneumonia infection reflects an exaggerated inflammatory response of the host immune system to an antigen, which is temporally related to recovery of the immune system. Clinical manifestations include fever, cough, dyspnoea and hypoxia following the commencement of antiretroviral therapy. Diagnosis is made on clinical and radiological criteria with exclusion of other infective and non-infective causes. Unrecognized, IRIS may be associated with significant morbidity and mortality. Treatment with corticosteroids often results in prompt recovery. There is limited literature on radiological findings of Pneumocystis jirovecii pneumonia-associated IRIS. Here we describe cross-sectional imaging findings of PJP-IRIS in a patient following commencement of antiretroviral therapy.

Respiratory bronchiolitis with fibrosis. High-resolution computed tomography findings and correlation with pathology.

BACKGROUND: Recent pathology studies report that the lungs of cigarette smokers may demonstrate small localized subpleural foci of interstitial fibrosis associated with emphysema. Pathologically, this lesion has been termed smoking-related interstitial fibrosis, respiratory bronchiolitis-interstitial lung disease (ILD) with fibrosis, or airspace enlargement with fibrosis, but it is commonly misinterpreted on imaging and biopsy as a diffuse fibrosing interstitial pneumonia. The high-resolution computed tomography (HRCT) appearance of this process has not been defined. OBJECTIVES: We describe HRCT imaging of seven patients with this lesion present histologically. MEASUREMENTS AND MAIN RESULTS: HRCT features consisted of patchy areas of mild reticulation around predominantly subpleural upper/mid zone emphysematous changes in all cases, with variable patchy ground-glass change in six cases. Pathologically, there were distinctly circumscribed foci of dense, often hyalinized, interstitial fibrosis mixed with emphysema; variable numbers of smoker's macrophages were present in the airspaces. The fibrosis was confined to the subpleural regions. Three patients with follow-up information were clinically stable. CONCLUSIONS: 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.

Incidental vertebral fractures on computed tomography.

Vertebral fractures are the most common osteoporotic fracture and predict subsequent fracture and mortality. We undertook an audit (Auckland City Hospital, Auckland, New Zealand) to determine whether targeted assessment for incidental vertebral fractures on computed tomography (CT) examinations of the chest or abdomen in older people would detect previously unidentified vertebral fractures. In 175 consecutive patients aged >65 years, sagittal images of the spine were obtained by reformatting data from CT examinations of the chest or abdomen. Vertebral fractures were assessed using a semi-quantitative technique. The prevalence of vertebral fractures was 13%, with 41 vertebral fractures identified in 22 patients; 12/22 (55%) had vertebral fracture mentioned in the formal CT report, and 2/12 patients with contemporaneous plain films had vertebral fracture mentioned in the X-ray report. The vertebral fracture was newly identified in 17 (77%) patients, but vertebral fracture and osteoporosis were each listed in the relevant discharge summary or clinic letter for only 14% of patients, and only 31% of patients with fracture subsequently received osteoporosis treatment. In summary, assessing sagittal spine images reformatted from CT examinations of the chest or abdomen detects previously unidentified vertebral fractures, offering an undervalued opportunity to assess fracture risk and intervene with treatments that prevent fractures and reduce mortality.

Idiopathic dendriform pulmonary ossification.

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.