Reversibility of clinical and computed tomographic lesions mimicking pulmonary fibrosis in a young cat

Abstract Background In humans with idiopathic pulmonary fibrosis (IPF), specific thoracic computed tomographic (CT) features in the correct clinical context may be used in lieu of histologic examination. Cats develop an IPF-like condition with similar features to humans. As few cats have invasive lung biopsies, CT has appeal as a surrogate diagnostic, showing features consistent with architectural remodeling supporting “end-stage lung”. Case presentation A 1-year-old female spayed Domestic Shorthair cat presenting with progressive respiratory clinical signs and thoracic CT changes (reticular pattern, parenchymal bands, subpleural interstitial thickening, pleural fissure thickening, subpleural lines and regions of increased attenuation with traction bronchiectasis and architectural distortion) consistent with reports of IPF was given a grave prognosis for long-term survival. The cat was treated with prednisolone, fenbendazole, pradofloxacin and clindamycin. Five months later, while still receiving an anti-inflammatory dose of prednisolone, the cat was re-evaluated with owner-reported absent respiratory clinical signs. Thoracic CT demonstrated resolution of lung patterns consistent with fibrosis. Conclusions Fibrotic lung disease is irreversible. Despite this cat having compatible progressive respiratory signs and associated lung patterns on thoracic CT scan, these abnormalities resolved with non-specific therapy and time, negating the possibility of IPF. While the cause of the distinct CT lesions that ultimately resolved was not determined, infection was suspected. Experimental Toxocara cati infection shows overlapping CT features as this cat and is considered a treatable disease. Improvement of CT lesions months after experimental heartworm-associated respiratory disease in cats has been documented. Reversibility of lesions suggests inflammation rather than fibrosis was the cause of the thoracic CT lesions. This cat serves as a lesson that although thoracic CT has been advocated as a surrogate for histopathology in people with IPF, additional studies in cats are needed to integrate CT findings with signalment, other clinicopathologic features and therapeutic response before providing a diagnosis or prognosis of fibrotic lung disease.

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Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features

Diagnostics ◽

10.3390/diagnostics10070450 ◽

2020 ◽

Vol 10 (7) ◽

pp. 450

Author(s):

Federica Galioto ◽

Stefano Palmucci ◽

Giovanna M. Astuti ◽

Ada Vancheri ◽

Giulio Distefano ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Integrated Approach ◽

Correct Treatment ◽

Radiological Features ◽

Palliative Care Services ◽

Pictorial Essay ◽

Personalized Approach ◽

Fibrotic Lung Disease

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis; it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal prognosis among ILDs. In fact, the progressive fibrosis related to IPF could lead to the development of complications, such as acute exacerbation, lung cancer, infections, pneumothorax and pulmonary hypertension. Pneumologists, radiologists and pathologists play a key role in the identification of IPF disease, and in the characterization of its complications—which unfortunately increase disease mortality and reduce overall survival. The early identification of these complications is very important, and requires an integrated approach among specialists, in order to plane the correct treatment. In some cases, the degree of severity of patients having IPF complications may require a personalized approach, based on palliative care services. Therefore, in this paper, we have focused on clinical and radiological features of the complications that occurred in our IPF patients, providing a comprehensive and accurate pictorial essay for clinicians, radiologists and surgeons involved in their management.

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The Its Not JUST Idiopathic pulmonary fibrosis Study (INJUSTIS): description of the protocol for a multicentre prospective observational cohort study identifying biomarkers of progressive fibrotic lung disease

BMJ Open Respiratory Research ◽

10.1136/bmjresp-2019-000439 ◽

2019 ◽

Vol 6 (1) ◽

pp. e000439 ◽

Cited By ~ 2

Author(s):

Fasihul Khan ◽

Iain Stewart ◽

Lucy Howard ◽

Tricia M McKeever ◽

Steve Jones ◽

...

Keyword(s):

Cohort Study ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Molecular Mechanisms ◽

Observational Cohort Study ◽

Prospective Observational Cohort Study ◽

Ethical Approval ◽

Observational Cohort ◽

Fibrotic Lung Disease

IntroductionThe Its Not JUST Idiopathic pulmonary fibrosis Study (INJUSTIS) is a multicentre, prospective, observational cohort study. The aims of this study are to identify genetic, serum and other biomarkers that may identify specific molecular mechanisms, reflecting disease endotypes that are shared among patients with progressive pulmonary fibrosis regardless of aetiology. Furthermore, it is anticipated that these biomarkers will help predict fibrotic activity that may identify patterns of disease behaviour with greater accuracy than current clinical phenotyping.Methods and analysis200 participants with the multidisciplinary team confirmed fibrotic lung disease (50 each of rheumatoid-interstitial lung disease (ILD), asbestosis, chronic hypersensitivity pneumonitis and unclassifiable ILD) and 50 idiopathic pulmonary fibrosis participants, recruited as positive controls, will be followed up for 2 years. Participants will have blood samples, lung function tests, quality of life questionnaires and a subgroup will be offered bronchoscopy. Participants will also be given the option of undertaking blinded home handheld spirometry for the first 3 months of the study. The primary end point will be identification of a biomarker that predicts disease progression, defined as 10% relative change in forced vital capacity (FVC) or death at 12 months.Ethics and disseminationThe trial has received ethical approval from the National Research Ethics Committee Nottingham (18/EM/0139). All participants must provide written informed consent. The trial will be overseen by the INJUSTIS steering group that will include a patient representative, and an independent chairperson. The results from this study will be submitted for publication in peer-reviewed journals and disseminated at regional and national conferences.Trial registration numberNCT03670576.

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Clinical, computed tomographic and histopathological findings in two cats with pulmonary fibrosis of unknown aetiology

Journal of Feline Medicine and Surgery Open Reports ◽

10.1177/2055116920968723 ◽

2020 ◽

Vol 6 (2) ◽

pp. 205511692096872

Author(s):

Eleanor C Duffy ◽

Sally Griffin ◽

Erin M O’Connell ◽

Jeremy R Mortier

Keyword(s):

Pulmonary Fibrosis ◽

Wide Spectrum ◽

Case Series ◽

Imaging Study ◽

Surgical Excision ◽

Lung Lobe ◽

Computed Tomographic ◽

Reticular Pattern ◽

Fibrotic Lung Disease ◽

The Right

Case series summary Two cats were presented for further investigation of respiratory signs. One cat had a history of a cough and the other, tachypnoea. In each case, thoracic CT was performed, which revealed a generalised marked reticular pattern in the first cat and focal consolidation of the right caudal lung lobe in the second cat. The first cat was euthanased following completion of the imaging study and a post-mortem examination was performed. The second cat underwent surgical excision of the abnormal lung lobe and survived for 4 years after diagnosis. Histopathology performed on lung tissue removed from each cat was consistent with pulmonary fibrosis. Relevance and novel information This small case series adds to the existing literature and highlights the heterogeneous clinical course and variable appearance of pulmonary fibrosis on CT of affected cats. These cases provide evidence that pulmonary fibrosis in cats incorporates a wide spectrum of fibrotic lung disease and demonstrates the possibility for prolonged survival following diagnosis where disease is localised and amenable to surgical resection.

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Diagnosing fibrotic lung disease: When is high-resolution computed tomography sufficient to make a diagnosis of idiopathic pulmonary fibrosis?

Respirology ◽

10.1111/j.1440-1843.2009.01626.x ◽

2009 ◽

Vol 14 (7) ◽

pp. 934-939 ◽

Cited By ~ 41

Author(s):

Shelley L. SCHMIDT ◽

Baskaran SUNDARAM ◽

Kevin R. FLAHERTY

Keyword(s):

Computed Tomography ◽

High Resolution ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

High Resolution Computed Tomography ◽

Fibrotic Lung Disease

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Hermansky–Pudlak syndrome pulmonary fibrosis: a rare inherited interstitial lung disease

European Respiratory Review ◽

10.1183/16000617.0193-2020 ◽

2021 ◽

Vol 30 (159) ◽

pp. 200193

Author(s):

Tadafumi Yokoyama ◽

Bernadette R. Gochuico

Keyword(s):

Interstitial Lung Disease ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Genetic Diseases ◽

Autosomal Recessive Disorder ◽

Oculocutaneous Albinism ◽

Excessive Bleeding ◽

Lung Physiology ◽

Hermansky Pudlak Syndrome ◽

Fibrotic Lung Disease

Pulmonary fibrosis is a progressive interstitial lung disease of unknown aetiology with a poor prognosis. Studying genetic diseases associated with pulmonary fibrosis provides insights into the pathogenesis of the disease. Hermansky–Pudlak syndrome (HPS), a rare autosomal recessive disorder characterised by abnormal biogenesis of lysosome-related organelles, manifests with oculocutaneous albinism and excessive bleeding of variable severity. Pulmonary fibrosis is highly prevalent in three out of 10 genetic types of HPS (HPS-1, HPS-2 and HPS-4). Thus, genotyping of individuals with HPS is clinically relevant. HPS-1 tends to affect Puerto Rican individuals due to a genetic founder effect. HPS pulmonary fibrosis shares some clinical features with idiopathic pulmonary fibrosis (IPF), including dyspnoea, cough, restrictive lung physiology and computed tomography (CT) findings of fibrosis. In contrast to IPF, HPS pulmonary fibrosis generally affects children (HPS-2) or middle-aged adults (HPS-1 or HPS-4) and may be associated with ground-glass opacification in CT scans. Histopathology of HPS pulmonary fibrosis, and not IPF, shows vacuolated hyperplastic type II cells with enlarged lamellar bodies and alveolar macrophages with lipofuscin-like deposits. Antifibrotic drugs approved as treatment for IPF are not approved for HPS pulmonary fibrosis. However, lung transplantation has been performed in patients with severe HPS pulmonary fibrosis. HPS pulmonary fibrosis serves as a model for studying fibrotic lung disease and fibrosis in general.

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Chemokine receptor 2-targeted molecular imaging in pulmonary fibrosis

10.1101/2020.03.04.960179 ◽

2020 ◽

Author(s):

Steven L. Brody ◽

Sean P. Gunsten ◽

Hannah P. Luehmann ◽

Debbie H. Sultan ◽

Michelle Hoelscher ◽

...

Keyword(s):

Pulmonary Fibrosis ◽

Lung Disease ◽

Mouse Models ◽

Chemokine Receptor ◽

Pet Imaging ◽

Lung Fibrosis ◽

Ex Vivo ◽

Inflammatory Monocytes ◽

Fibrotic Process ◽

Fibrotic Lung Disease

AbstractIdiopathic pulmonary fibrosis (IPF) is a progressive, inflammatory lung disease that is monitored clinically by measures of lung function, without effective molecular markers of disease activity or therapeutic efficacy. Lung immune cells active in the pro-fibrotic process include inflammatory monocyte and interstitial macrophages that express the C-C motif chemokine receptor 2 (CCR2). CCR2+ monocyte lung influx is essential for disease phenotypes in models of fibrosis and identified in lungs from subjects with IPF. Here, we show that our peptide-based radiotracer 64Cu-DOTA-ECL1i identifies CCR2+ inflammatory monocytes and interstitial macrophages in multiple preclinical mouse models of lung fibrosis, using positron emission tomography (PET) imaging. Mice with bleomycin-induced fibrosis treated with blocking antibodies to interleukin-1β, a mediator of fibrosis associated with CCR2+ cell inflammation, or with pirfenidone, an approved anti-fibrotic agent, demonstrated decreased CCR2-dependent interstitial macrophage accumulation and reduced 64Cu-DOTA-ECL1i PET uptake, compared to controls. Lung tissues from patients with fibrotic lung disease demonstrated abundant CCR2+ cells surrounding regions of fibrosis, and an ex vivo tissue-binding assay showed correlation between radiotracer localization and CCR2+ cells. In a phase 0/1 clinical study of 64Cu-DOTA-ECL1i PET, healthy volunteers showed little lung uptake, while subjects with pulmonary fibrosis exhibited increased uptake, notably in zones of subpleural fibrosis, reflecting the distribution of CCR2+ cells in the profibrotic niche. These findings support a pathologic role of inflammatory lung monocytes/macrophages in fibrotic lung disease and the translational use of 64Cu-DOTA-ECL1i PET to track CCR2-specific inflammation for image-guided therapy.One Sentence SummaryPET imaging of CCR2+ cells in lung fibrosis identifies a therapeutic response in mouse models and displays a perifibrotic signal in subjects with IPF.

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Idiopathic pulmonary fibrosis: the radiologist’s role in making the diagnosis

British Journal of Radiology ◽

10.1259/bjr.20181003 ◽

2019 ◽

Vol 92 (1099) ◽

pp. 20181003 ◽

Cited By ~ 2

Author(s):

Michael P. Mohning ◽

John Caleb Richards ◽

Tristan J. Huie

Keyword(s):

Differential Diagnosis ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Lung Biopsy ◽

Critical Role ◽

Pattern Identification ◽

Diagnostic Process ◽

Imaging Features ◽

Fibrotic Lung Disease

Radiologists have a critical role in the evaluation and diagnosis of suspected idiopathic pulmonary fibrosis (IPF). Accurate pattern identification on imaging is key in the multidisciplinary diagnostic process and frequently obviates the need for a surgical lung biopsy. In this review, we describe the clinical and imaging features of IPF in the context of recently revised international guidelines; contrast findings in other diseases that may inform differential diagnosis of fibrotic lung disease; and highlight common complications associated with pulmonary fibrosis.

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