Case Reports
24 September 2024
Vol. 76 No. 4 (2024)
https://doi.org/10.4081/reumatismo.2024.1647

Visceral muscle dysmotility syndrome in systemic lupus erythematosus: which is the role of 18 fluorodeoxyglucose-positron emission tomography-computed tomography? A clinical case and literature review

Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Systemic lupus erythematosus, visceral muscle dysmotility syndrome, generalized megaviscerous of lupus, 18 fluorodeoxyglucose-positron emission tomography-computed tomography
854
Views
268
Downloads

Authors

S. Scriffignano
https://orcid.org/0000-0001-5774-9643
Academic Rheumatology Unit, Department of Medicine and Health Science “Vincenzo Tiberio,” University of Molise, Campobasso, Italy.
F.M. Perrotta
https://orcid.org/0000-0003-3771-5205
Academic Rheumatology Unit, Department of Medicine and Health Science “Vincenzo Tiberio,” University of Molise, Campobasso, Italy.
M. Ricci
https://orcid.org/0000-0002-9277-6337
Nuclear Medicine Unit, Ospedale Cardarelli, Campobasso, Italy.
B. Carabellese
Nuclear Medicine Unit, Ospedale Cardarelli, Campobasso, Italy.
E. Lubrano
enniolubrano@hotmail.com
https://orcid.org/0000-0001-6189-5328
Academic Rheumatology Unit, Department of Medicine and Health Science “Vincenzo Tiberio,” University of Molise, Campobasso, Italy.

Visceral muscle dysmotility syndrome (VMDS) is a rare syndrome described in the systemic lupus erythematosus (SLE) clinical course. It is characterized by diffuse thickened intestinal wall and gastrointestinal-genitourinary-hepatobiliary hollow viscera dilatation and dysmotility. Due to the rarity and the heterogeneity of the clinical characteristics of this syndrome, it is not entirely clear which is the best diagnostic imaging technique for the diagnosis and/or follow-up, even if, in all the described cases, computed tomography (CT) was generally used to study visceral involvement. However, there are no cases describing the visceral metabolic activity by 18 fluorodeoxyglucose-positron emission tomography-CT (18FDG-PET-CT). Here, we reported the first clinical case of VMDS studied by 18FDG-PET-CT, characterizing the metabolic activity of this rare syndrome during SLE flare. We found a high intestinal metabolic burden, hyper-fixation in duodenum, and high hepatic metabolic activity. Moreover, we reviewed the literature on VMDS in SLE, focusing on imaging techniques in different anatomical sites (bowel, urinary tract, bile ducts), patients’ symptoms, and treatment.

Altmetrics

Downloads

Download data is not yet available.

Citations

Crossref
Scopus
Google Scholar
Europe PMC
Kaul A, Gordon C, Crow MK, Touma Z, Urowitz MB, van Vollenhoven R, et al. Systemic lupus erythematosus. Nat Rev Dis Primers 2016; 2: 16039. DOI: https://doi.org/10.1038/nrdp.2016.39
Park FD, Lee JK, Madduri GD, Ghosh P. Generalized megaviscera of lupus: refractory intestinal pseudo-obstruction, ureterohydronephrosis and megacholedochus. World J Gastroenterol 2009; 15: 3555-9. DOI: https://doi.org/10.3748/wjg.15.3555
Chen YQ, Xue Q, Wang NS. Visceral muscle dysmotility syndrome in systemic lupus erythematosus: case report and review of the literature. Rheumatol Int 2012; 32: 1701-3. DOI: https://doi.org/10.1007/s00296-011-1856-4
Xu N, Zhao J, Liu J, Wu D, Zhao L, Wang Q, et al. Clinical analysis of 61 systemic lupus erythematosus patients with intestinal pseudo-obstruction and/or ureterohydronephrosis: a retrospective observational study. Medicine 2015; 94: e419. DOI: https://doi.org/10.1097/MD.0000000000000419
Alexopoulou A, Andrianakos A, Dourakis SP. Intestinal pseudo-obstruction and ureterohydronephrosis as the presenting manifestations of relapse in a lupus patient. Lupus 2004; 13: 954-6. DOI: https://doi.org/10.1191/0961203304u1064cr
Pardos-Gea J, Ordi-Ros J, Selva A, Perez-Lopez J, Balada E, Vilardell M. Chronic intestinal pseudo-obstruction associated with biliary tract dilatation in a patient with systemic lupus erythematosus. Lupus 2005; 14: 328-30. DOI: https://doi.org/10.1191/0961203304lu2047cr
Kansal A, Jain A, Thenozhi S, Agarwal V. Intestinal pseudo-obstruction associated with biliary tract dilatation in a patient with systemic lupus erythematosus. Lupus 2013; 22: 87-91. DOI: https://doi.org/10.1177/0961203312464091
Wen J, Chen W, Gao L, Qiu X, Lin G. Systemic lupus erythematosus simultaneously presenting with visceral muscle dysmotility syndrome and mechanical intestinal obstruction clinically relieved by surgery: a case report and literature review. BMC Gastroenterol 2022; 22: 32. DOI: https://doi.org/10.1186/s12876-022-02105-3
Mir TH, Jabeen B, Wani NA, Khan BA, Javeed R, Omar Parvaiz M. Visceral muscle dysmotility syndrome of lupus. Rheumatology 2023; 62: e45-6. DOI: https://doi.org/10.1093/rheumatology/keac424
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40: 1725. DOI: https://doi.org/10.1002/art.1780400928
Gladman DD, Ibañez D, Urowitz MB. Systemic lupus erythematosus disease activity index 2000. J Rheumatol 2002; 29: 288-91.
Potera J, Palomera Tejeda E, Arora S, Manadan AM. Lupus enteritis: an uncommon presentation of lupus flare. Cureus 2021; 13: e18030. DOI: https://doi.org/10.7759/cureus.18030
Copyright (c) 2024 the Author(s) Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.