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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Chronic fibrosing conditions in abdominal imaging
Verghese George1, Varaha Sairam Tammisetti, Venkateswar Rao Surabhi
1Department of Diagnostic and Interventional Imaging, University of Texas Health Science Center at Houston, MSB 2.130B, 6431 Fannin St, Houston, TX 77030, USA. verghese.george@uth.tmc.edu
Abstract:
Chronic fibrosing conditions of the abdomen are relatively poorly understood and involve varied and often multiple organ systems. At histopathologic analysis, they share the unifying features of proliferative fibrosis and chronic inflammation. Different conditions in this group are often found in association with each other and with other fibrosing conditions outside the abdomen. Some of the confusion about these conditions stems from their complex nomenclature, which includes a gamut of alternate terms and eponyms. Many of them can be categorized within two large subgroups: the fibromatoses and immunoglobulin G4 (IgG4)-related disorders. While many of these entities are of uncertain etiology, some, especially the IgG4-associated conditions, appear to have an immune-mediated pathogenesis. Nephrogenic systemic fibrosis, sclerosing peritonitis, and retroperitoneal fibrosis have iatrogenic associations, while some of the fibromatoses are genetically inherited. Imaging differentiation of these conditions is difficult due to considerable overlap in their radiologic findings. However, certain conditions such as penile fibromatosis and sclerosing peritonitis may have unique imaging features that can help the radiologist make the diagnosis. Others such as deep fibromatoses and inflammatory pseudotumor demonstrate fibroproliferative mass formation and cannot be differentiated from neoplastic conditions at imaging. Thus, histopathologic correlation is often required to confirm their diagnosis.
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