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Scleroderma
Published in Jason Liebowitz, Philip Seo, David Hellmann, Michael Zeide, 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).
The Interstitial Pneumonias
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Usual interstitial pneumonia (UIP) is a syndrome characterized by progressive exertional dyspnea leading to respiratory failure or corpulmonale causing death within 6 months as reported by Hamman and Rich in five patients seen between 1933 and 1944.118,119 Thickened alveolar walls and inflammatory and connective tissue changes in the distal airspaces were described. A nonfulminant course or a more chronic state lasting several years have been subsequently reported.120,121
Thorax
Published in Dave Maudgil, Anthony Watkinson, The Essential Guide to the New FRCR Part 2A and Radiology Boards, 2017
Dave Maudgil, Anthony Watkinson
Most cases of CFA (or usual interstitial pneumonia, UIP) are idiopathic. Others cases are familial, drug related or associated with connective tissue diseases such as rheumatoid arthritis. NSIP may be due to connective tissue diseases, infection, or toxic inhalation. LCH is severely accelerated by cigarette smoking.
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].
Clinical, radiologic, and physiologic features of idiopathic pulmonary fibrosis (IPF) with and without emphysema
Published in Expert Review of Respiratory Medicine, 2022
Chenfei Li, Yan Wang, Qi Liu, Hai Zhang, Fei Xu, Zhenyun Gao, Xiaohui Wang, Guangyu Tao, Yuqing Chen, Wenwen Rong, Hong Yu, Feng Li
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic interstitial lung disease (ILD) of unexplained causes, occurring mostly in the elderly. The major histopathological feature and/or thoracic imaging is usual interstitial pneumonia (UIP) localized to the lung [1]. The incidence and prevalence of IPF are reported to be 14.6 per 100,000 person-years and 58.7 per 100,000 persons, respectively [2]. Moreover, patients with IPF are prone to developing pulmonary complications including pulmonary hypertension (PH) and lung cancer, resulting in a poor prognosis [3]. Combined pulmonary fibrosis and emphysema (CPFE), first proposed in 2005, is a distinct entity defined by the coexistence of emphysema and/or multiple pulmonary bullae (primarily in the upper lobes) and pulmonary fibrosis (primarily in the lower lobes and subpleural region) [4]. However, there is no consensus on the precise definition of CPFE. High resolution computed tomography (HRCT) imaging of the lungs of CPFE provides a very heterogeneous picture of the pulmonary fibrosis that can be divided into UIP type and non-UIP type. The former includes IPF [5], whereas the latter includes nonspecific interstitial pneumonia (NSIP), alveolar enlargement with fibrosis (AEF), and smoking-related interstitial fibrosis (SRIF) [6].
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].