Porto-sinusoidal vascular liver disease in systemic sclerosis: case study
DOI:
https://doi.org/10.26641/1997-9665.2023.3.30-35Keywords:
porto-sinusoidal vascular disease of the liver, systemic sclerosis.Abstract
Background. When detecting vascular lesions of the liver, especially if they are not accompanied by clear clinical symptoms, pathologists may encounter difficulties in describing and interpreting pathological manifestations due to insufficient study of this pathology and conflicting terminology. Objective. The purpose of the work is to describe a rare variant of porto-sinusoidal vascular liver disease in a patient with systemic sclerosis. Methods. Pathomorphological examination of liver and lung tissue (necropsy) using histological methods. Results. A 44-year-old patient with a confirmed (clinically and pathomorphologically) diagnosis of "systemic sclerosis" died due to respiratory failure. At autopsy, in addition to signs characteristic of systemic sclerosis (diffuse pneumofibrosis of nonspecific interstitial pneumonia type and pleuropulmonary fibroelastosis). changes in the vascular bed of the lungs and liver were detected. The latter can be attributed to several morphological patterns: complete obliteration of medium-sized vessels with replacement by concentric laminar structures, formation of hypervascularized areas of loose extracellular matrix with thin-walled vessels in the place of preexisting vessels, thickening of vessel walls due to hyperplasia and/or sclerosis with lumen stenosis. For such lesions of the hepatic vessels, a relatively new term is suitable - porto-sinusoidal vascular disease of the liver, which does not imply the presence of clinical signs of portal hypertension, which was observed in our case. The genesis of vasculopathy remains unclear, but probably an important role is played by disturbed cell-matrix (endothelial, myofibroblast) interactions characteristic of systemic sclerosis. Conclusion. Despite the rarity of porto-sinusoidal vascular disease of the liver, in each case of detection of vascular changes during the examination of liver tissue, it is necessary to conduct a thorough clinical and anatomical analysis, taking into account the scientific data available today, since this can be important both for assessing the prognosis of the disease and for selection of adequate therapy.
References
- McMichael J. The pathology of hepatoli-enal fibrosis. J. Path. and Bact. 1934;39:481-482.
- Schouten JN, Garcia-Pagan JC, Valla DC, Janssen HL. Idiopathic noncirrhotic portal hyper-tension. Hepatology. 2011;54:1071-1081.
- De Gottardi A, Rautou PE, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J. Porto-sinusoidal vascular disease: proposal and descrip-tion of a novel entity. Lancet Gastroenterol Hepatol. 2019;4:399-411.
- Jin SS, Choi WM. Porto-Sinusoidal Vascu-lar Disease: A Concise Updated Summary of Epi-demiology, Pathophysiology, Imaging, Clinical Fea-tures, and Treatments. Korean J Radiol. 2023;24(1):31-38. DOI: https://doi.org/10.3348/kjr. 2022.0668
- Kmeida M, Liub X, Ballentinec S, Leea H. Idiopathic Non-Cirrhotic Portal Hypertension and PortoSinusoidal Vascular Disease: Review of Cur-rent Data. Gastroenterol Res. 2021;14(2):49-65. DOI: https://doi.org/10.14740/gr1376
- Kleiner DE. Noncirrhotic Portal Hyperten-sion: Pathology and Nomenclature. Clinical Liver Disease. 2015;5(5):123-126.
- Guido M, Alves VAF, Balabaud C, Bathal PS, Bioulac-Sage P, Colombari R, Crawford JM, Dhillon AP, Ferrell LD, Gill RM, Hytiroglou P, Nakanuma Y, Paradis V, Quaglia A, Rautou PE, Theise ND, Thung S, Tsui WMS, Sempoux C, Snover D, van Leeuwen DJ. Histology of portal vas-cular changes associated with idiopathic non-cirrhotic portal hypertension: nomenclature and definition. Histopathology. 2019;74(2):219-226. DOI: 10.1111/his.13738
- De Gottardi A, Sempoux C, Berzigotti A. Porto-sinusoidal vascular disorder. Journal of Hepa-tology. 2022;77:1124–1135. DOI: https://doi.org/ 10.1016/j.jhep.2022.05.033
- Chaturvedi1 R, Shah T, Joshi A, Mishra T, Karegar M, Shukla A. Histopathological Study of Non-Cirrhotic Portal Fibrosis (NCPF) With Special Emphasis on Advanced Fibrosis. Annals of Patholo-gy and Laboratory Medicine. 2018;5(12):1001-1007. DOI: 10.21276/APALM2358
- Kmeid M, Zuo C, Lagana SM, Choi WT, Lin J, Yang Z, Liu X, Westerhoff M, Fiel MI, Af-folter K, K. Choi EK, Lee H. Interobserver study on histologic features of idiopathic non-cirrhotic portal hypertension. Diagnostic Pathology. 2020;15:129-138.
- Liang L, Shi C, Dupont WD, Salaria SN, Huh WJ, Correa H, Roland JT, Perri RE, Kay M. Key histopathologic features in idiopathic noncir-rhotic portal hypertension: an interobserver agree-ment study and proposal for diagnostic criteria. Washington Modern Pathology. 2021;34:592–602. DOI: https://doi.org/10.1038/s41379-020-00676-8
- Nicoară-Farcău O, Rusu I, Stefănescu H, Tanțău M, Badea RI, Procope P. Diagnostic chal-lenges in non-cirrhotic portal hypertension - porto sinusoidal vascular disease. World J Gastroenterol. 2020;26(22):3000-3011. DOI: 10.3748/wjg.v26.i22.3000
- Bernstein EJ, Huggins JT. Systemic Sclero-sis With Associated Interstitial Lung Disease: Man-agement Considerations and Future Directions. Am J Manag Care. 2021;27(7):138-146. DOI: https:// doi.org/10.37765/ajmc.2021.88656
- Rosendahl AH, Schönborn K, Kaohsiung TK. Pathophysiology of systemic sclerosis (sclero-derma). J Med Sci. 2022;38:187–195. DOI: 10.1002/kjm2.12505
- DeMizio DJ, Bernstein EJ. Detection and classification of systemic sclerosis-related interstitial lung disease: a review. Curr Opin Rheumatol. 2019;31(6):553-560. DOI:10.1097/BOR.0000000000000660
- Rana D, Kaushik N, Sadhu S, Kalra R, Sen R. Idiopathic Lipoid Pneumonia: An incidental find-ing in autopsy specimen. Autopsy and Case Reports 2020;10(1):143. DOI: 10.4322/acr.2020.143
Downloads
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
The authors reserve the right to authorship of their work and transfer to the Journal the right to the first publication of this work under the terms of a license Creative commons Attribution 4.0 International (CC BY 4.0), which allows other people to freely distribute the published work with a mandatory reference to the authors of the original work and the first publication of the work in this journal.By submitting a manuscript to the editorial office of the Journal ‘Morphologia’ authors agree to transfer the rights to protect and use the manuscript (all supplemental materials, particularly protected objects such as photos, drawings, diagrams, tables, etc.), including the reproduction in the press and distribution via the Internet; translation of the manuscript into any language; export and import of journal copies with the Authors’ article in order to make it available for public. Authors convey the rights mentioned above to the editorial office without any temporal or territorial limitation all over the world.
The Authors guarantee that they have the exclusive rights to use the material transferred to editorial office. Editors are not responsible to third parties for contraventions of warranty given by the Authors. The considered rights are transferred to the editorial office since the moment when the current issue is signed for publishing. Reproduction of materials published in the Journal by other individuals and legal entities is possible only with the consent of Editorial office, with the obligatory indication of the full bibliographic reference of the primary publication. The Authors reserve the right to use the published material, its fragments and parts for teaching materials, oral presentations, dissertation thesis prepararion with obligatory bibliographic citation of the original paper. Electron copy of the published article, downloaded from official journal web-site in .pdf format may be put by authors on the official web-site of their institutions, any other official resources with open access.
