Predominantly Mature Interstitial Fibrosis
Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley in Diagnostic Pulmonary Pathology, 2008
The major entities in this category and their distinguishing characteristics are listed in Table 1. The single most common entity in this group is usual interstitial pneumonia (UIP, idiopathic pulmonary fibrosis), a progressive fibrotic disease of unknown etiology. The histologic features of this disorder are characterized by geographic and temporal heterogeneity, incorporating predominantly mature fibrosis, immature fibrosis with conspicuous fibroblast foci, and areas of much spared lung tissue containing normal or near normal alveolar architecture. Variable numbers of chronic inflammatory cells are observed within the interstitium, and hyperplastic type II pneumocytes line the fibrotic alveolar septa. Honeycomb changes, characterized by cystic spaces with fibrotic walls lined by a meta-plastic and generally cuboidal epithelium with mucostasis and inflammatory debris, are frequently observed. Variable numbers of macrophages are present within the alveolar spaces (3).
Scleroderma
Jason Liebowitz, Philip Seo, David Hellmann, Michael Zeide in Clinical Innovation in Rheumatology, 2023
Cardiopulmonary disease, such as interstitial lung disease (ILD), cardiac disease, or pulmonary hypertension, is one of the leading causes of SSc-associated death (21). Interstitial lung disease (ILD) is one of the most common complications of SSc. Depending on the definition used to characterize ILD, the prevalence can vary widely. For example, in early autopsy studies, it was noted that 100% of histopathologic specimens of the lung demonstrated nonspecific interstitial fibrosis (22). Furthermore, it has also been reported that up to 90% of patients will have interstitial abnormalities on high-resolution computed tomography (HRCT) and 40–75% will have decline in the pulmonary function tests (PFTs) (23–25). The most common form of ILD is nonspecific interstitial pneumonia (NSIP) in SSc-associated ILD that can be seen either radiographically or histopathology. Other reported lung involvement include usual interstitial pneumonia (UIP), diffuse alveolar hemorrhage, organizing pneumonia, and/or lymphocytic interstitial pneumonia (26, 27).
Indications for lung transplantation and patient selection
Wickii T. Vigneswaran, Edward R. Garrity, John A. Odell in LUNG Transplantation, 2016
The American Thoracic Society released an updated classification of idiopathic interstitial pneumonias based primarily on histopathology.148 IPF, with its pathologic pattern of usual interstitial pneumonia (UIP), has a uniquely poor prognosis when compared with other histopathologic patterns, and diagnosing it appropriately when considering a patient for a transplant is thus crucial. In contrast to patients with desquamative interstitial pneumonia and respiratory bronchiolitis–associated interstitial lung disease (80% 5-year survival rate) and nonspecific interstitial pneumonia (NSIP) (70% survival rate), patients with UIP in one series were found to have a 20% 5-year survival rate.149 Similarly, a prospective analysis of 315 patients with diffuse parenchymal lung disease demonstrated substantially worse survival for patients with IPF (2-year survival rate = 48.4%, 5-year survival rate = 35.4%) than in those with the other diseases (2-year survival rate = 74.9% to 100%, 5-year survival rate = 69.5% to 91.6%).150 A survival benefit for patients with IPF who receive transplants has been established in several evaluations.106,151,152
The temporal heterogeneity of usual interstitial pneumonia on chest CT
Published in Expert Review of Respiratory Medicine, 2022
Ahmad Abu Qubo, Anjali Saqi, Mary M. Salvatore
Usual interstitial pneumonia (UIP) is the term used by both pathologists and radiologists to describe a pattern of fibrosis on histological specimens and CT imaging. A histopathologic or radiologic diagnosis of UIP is classically associated with IPF, however, other fibrosing interstitial lung diseases (ILDs) can present with this pattern [1,2]. The joint statement by the American thoracic society (ATS), European respiratory society (ERS), Japanese respiratory society (JRS), and Latin America thoracic society (ALAT) in 2018 described 4 categories of confidence for histologic UIP diagnosis: UIP, probable UIP, indeterminate for UIP, and a pattern suggesting alternative diagnosis [3]. These guidelines defined UIP histologically as sub-pleural and/or para-septal dense fibrosis, distributed in a temporally and spatially heterogeneous manner, exhibiting architectural distortion and fibroblast foci with absence of features indicating another diagnosis. The probable UIP pattern is defined as having some of the UIP features with the absence of features indicating another diagnosis or as honeycombing alone [3].
Scleroderma-related interstitial lung disease: principles of management
Published in Expert Review of Respiratory Medicine, 2019
Aparna Das, Anupam Kumar, Andrea Valeria Arrossi, Subha Ghosh, Kristin B. Highland
High resolution computed tomography (HRCT) of the chest typically shows a pattern consistent with NSIP with peripheral lower lobe predominant ground glass opacities (GGO) and reticulation (Figure 1(a)). Subpleural sparing is appreciated in a minority of patients (Figure 1(b)). In the fibrotic variant of NSIP, these changes evolve and may appear as volume loss and traction bronchiectasis (Figure 1(b)). Usual interstitial pneumonia (UIP) is less common and appears as peripheral, basal predominant areas of reticulation, traction bronchiectasis and honeycombing, without significant GGO (Figure 2). The presence of a dilated and/or patulous esophagus is a common radiographic clue to a possible diagnosis of SSc (Figure 3). The discerning clinician should also be cognizant of other radiological patterns that may be seen in patients with SSc. A dilated pulmonary artery or right ventricle may warrant further investigation for the presence of pulmonary hypertension (Figure 4). Rarely, patients with SSc may also develop pulmonary capillary hemangiomatosis (PCH) or pulmonary venoocclusive disease (PVOD), which are indistinguishable from each other, but have characteristic presence of interlobular septal thickening, centrilobular ground glass opacities, mediastinal adenopathy and pleural effusions (Figures 4 and 5) [7,8].
An IPF-like disease course in disorders other than IPF: how can this be anticipated, recognized, and managed?
Published in Expert Review of Clinical Immunology, 2021
Athol U. Wells, Vasileios Kouranos
The problem of IPF-like chronic progression usually applies to patients with disease that is wholly or largely irreversible at presentation. The underlying histopathologic pattern is an essential consideration in this regard. The presence of usual interstitial pneumonia (UIP) has denoted a poor outcome in histologic series [17]. The general consensus is that HP-UIP progresses somewhat similarly to IPF but at, on average, a somewhat slower rate. In point of fact, outcomes in patients with histologically defined HP-UIP were strikingly similar to those in IPF in published series, although the existing data are underpowered [17–20]. It should be stressed that a diagnosis of chronic HP is subject to major clinician/radiologist/pathologist variation, based on a pivotal expert group study, with IPF often the major differential diagnosis [21].
Related Knowledge Centers
- Connective Tissue Disease
- Fibrosis
- Idiopathic Pulmonary Fibrosis
- Interstitial Lung Disease
- Interstitium
- Respiratory Disease
- Shortness of Breath
- Lung
- Shortness of Breath
- Rheumatoid Arthritis
- Hypersensitivity Pneumonitis