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
Submitted: 13 September 2023
Accepted: 1 May 2024
Published: 24 September 2024
Accepted: 1 May 2024
Abstract Views: 400
PDF: 11
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.
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.
Authors
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.
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
How to Cite
Scriffignano, S., Perrotta, F., Ricci, M., Carabellese, B., & Lubrano , E. (2024). 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. Reumatismo, 76(4). https://doi.org/10.4081/reumatismo.2024.1647
Copyright (c) 2024 the Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
Similar Articles
- Y. Emad, T. Gheita, H. Darweesh, P. Klooster, R. Gamal, H. Fathi, N. El-Shaarawy, M. Gamil, M. Hawass, R.M. El-Refai, H. Al-Hanafi, S. Abd-Ellatif, A. Ismail, J. Rasker, Antibodies to extractable nuclear antigens (ENAS) in systemic lupus erythematosus patients: correlations with clinical manifestations and disease activity , Reumatismo: Vol. 70 No. 2 (2018)
- E. Cipriano, F. Ceccarelli, L. Massaro, F. R. Spinelli, C. Alessandri, C. Perricone, G. Valesini, F. Conti, Joint involvement in patients affected by systemic lupus erythematosus: application of the swollen to tender joint count ratio , Reumatismo: Vol. 67 No. 2 (2015)
- M. Fredi, M. Bianchi, L. Andreoli, G. Greco, I. Olivieri, S. Orcesi, E. Fazzi, C. Cereda, A. Tincani, Typing TREX1 gene in patients with systemic lupus erythematosus , Reumatismo: Vol. 67 No. 1 (2015)
- L. Iaccarino, A. Ghirardello, M. Zen, D. Villalta, A. Tincani, L. Punzi, A. Doria, Polarization of TH2 response is decreased during pregnancy in systemic lupus erythematosus , Reumatismo: Vol. 64 No. 5 (2012)
- D. Jain, H.K. Aggarwal, V. Kaverappa, S. Dhayia, P. Jain, S. Yadav, Anti-dsDNA negative and anti-Ro positive lupus nephritis: a report of a rare case , Reumatismo: Vol. 65 No. 6 (2013)
- F. Dall'Ara, I. Cavazzana, M. Frassi, M. Taraborelli, M. Fredi, F. Franceschini, L. Andreoli, M. Rossi, C. Cattaneo, A. Tincani, P. Airò, Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center , Reumatismo: Vol. 70 No. 2 (2018)
- M. A. Marie, R. E. Abu Khalil, H. M. Habib, Urinary CXCL10: a marker of nephritis in lupus patients , Reumatismo: Vol. 65 No. 6 (2013)
- G. Pasero, P. Marson, Short story of antirheumatic therapy.VIII. The immunodepressants , Reumatismo: Vol. 64 No. 1 (2012)
- M. Taraborelli, M.G. Lazzaroni, N. Martinazzi, M. Fredi, I. Cavazzana, F. Franceschini, A. Tincani, The role of clinically significant antiphospholipid antibodies in systemic lupus erythematosus , Reumatismo: Vol. 68 No. 3 (2016)
- E. Chessa, M. Piga, A. Floris, A. Mathieu, A. Cauli, Demyelinating syndrome in SLE: review of different disease subtypes and report of a case series , Reumatismo: Vol. 69 No. 4 (2017)
You may also start an advanced similarity search for this article.
