Current issue
Archive
Online first
About the Journal
Editorial Office
Editorial Board
Publisher
Editorial Policies
Ethical standards and procedures
Abstracting and indexing
Reviewers
Honorary Reviewers
Subscription
Contact
Most read articles
Instructions for authors
Article processing charge (APC)
Books and Events
Books
Events
CASE-BASED REVIEW
Systemic lupus erythematosus with intestinal pseudo-obstruction and polyserositis complicated by pneumatosis intestinalis and pulmonary embolism: a unique challenge posed by a fatal combination
1
Caritas Hospital and Institute of Health Sciences, Thellakom, Kottayam, India
Submission date: 2025-05-17
Final revision date: 2025-11-06
Acceptance date: 2025-12-31
Publication date: 2026-11-06
Corresponding author
Reumatologia 2026;64(5):434-440
KEYWORDS
TOPICS
ABSTRACT
Systemic lupus erythematosus (SLE) is a multisymptomatic autoimmune rheumatic disease. Some complications are well known and can be life-threatening, such as pulmonary embolism (PE) and, in general, thrombotic events due to endothelial dysfunction, platelet activation, and secondary antiphospholipid syndrome. Gastrointestinal (GI) involvement is less prevalent and is not directly associated with a life-threatening risk. Hovewer, lupus mesenteric vasculitis, intestinal pseudo- obstruction (IPO), and enteropathy indicate that closer attention should be paid to these complications. Therefore, the aim of this review is to identify cases of SLE with IPO, pneumatosis intestinalis (PI), and PE in the current literature. The case of a patient with SLE and IPO became the stimulus for this research and discussion. Finally, after the selection process, 49 studies were reviewed and included in the analysis; as a result, the literature typically describes cases of IPO and PI, but only rare reports present an association of these complications with thrombotic events such as PE. In conclusion, research should focus more deeply on SLE patients with complex GI and thrombotic manifestations.
REFERENCES (49)
1.
Mathur R, Deo K, Raheja A. Systemic lupus erythematosus in India: a clinico-serological correlation. Cureus 14: e25763, DOI: 10.7759/cureus.25763.
2.
Fava A, Petri M. Systemic lupus erythematosus: diagnosis and clinical management. J Autoimmun 2019; 96: 1–13, DOI: 10.1016/ j.jaut.2018.11.001.
3.
Wang JL, Liu G, Liu T, Wei JP. Intestinal pseudo-obstruction in systemic lupus erythematosus: a case report and review of the literature. Medicine (Baltimore) 2014; 93: e248, DOI: 10.1097/MD.0000000000000248.
4.
Ohri AJ, Shah CG, Udani AH. Intestinal pseudo-obstruction – an under-recognized presentation of systemic lupus erythematosus. Indian J Nephrol 2022; 32: 476–479, DOI: 10.4103/ijn.IJN_494_20.
5.
Narváez J, Pérez-Vega C, Castro-Bohorquez FJ, et al. Intestinal pseudo-obstruction in systemic lupus erythematosus. Scand J Rheumatol 2003; 32: 191–195, DOI: 10.1080/ 03009740310002588.
6.
Tian J, Zhang D, Yao X, et al. Global epidemiology of systemic lupus erythematosus: a comprehensive systematic analysis and modelling study. Ann Rheum Dis 2023; 82: 351–356, DOI: 10.1136/ard-2022-223035.
7.
Barber MRW, Drenkard C, Falasinnu T, et al. Global epidemiology of systemic lupus erythematosus. Nat Rev Rheumatol 2021; 17: 515–532, DOI: 10.1038/s41584-021-00668-1.
8.
Duarte-García A, Hocaoglu M, Valenzuela-Almada M, et al. The rising incidence and prevalence of systemic lupus erythematosus: a population-based study over four decades. Ann Rheum Dis 2022; 81: 1260–1266, DOI: 10.1136/annrheumdis-2022-222276.
9.
Fatoye F, Gebrye T, Mbada C. Global and regional prevalence and incidence of systemic lupus erythematosus in low-and-middle income countries: a systematic review and meta- analysis. Rheumatol Int 2022; 42: 2097–2107, DOI: 10.1007/s00296-022-05183-4.
10.
Malaviya AN, Singh RR, Singh YN, et al. Prevalence of systemic lupus erythematosus in India. Lupus 1993; 2: 115–118, DOI: 10.1177/096120339300200209.
11.
Gómez-Puerta JA, Barbhaiya M, Guan H, et al. Racial/ethnic variation in all-cause mortality among U.S. Medicaid recipients with systemic lupus erythematosus: an Hispanic and Asian paradox. Arthritis Rheumatol 2015; 67: 752–760, DOI: 10.1002/ art.38981.
12.
Hoover PJ, Costenbader KH. Insights into the epidemiology and management of lupus nephritis from the U.S. rheumatologist’s perspective. Kidney Int 2016; 90: 487–492, DOI: 10.1016/ j.kint.2016.03.042.
13.
Tian XP, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment. World J Gastroenterol 2010; 16: 2971–2977, DOI: 10.3748/wjg.v16.i24.2971.
14.
Fawzy M, Edrees A, Okasha H, et al. Gastrointestinal manifestations in systemic lupus erythematosus. Lupus 2016; 25: 1456–1462, DOI: 10.1177/0961203316642308.
15.
Ebert EC, Hagspiel KD. Gastrointestinal and hepatic manifestations of systemic lupus erythematosus. J Clin Gastroenterol 2011; 45: 436–441, DOI: 10.1097/MCG.0b013e31820f- 81b8.
16.
Zhang L, Xu D, Yang H, et al. Clinical features, morbidity, and risk factors of intestinal pseudo-obstruction in systemic lupus erythematosus: a retrospective case-control study. J Rheumatol 2016; 43: 559–564, DOI: 10.3899/jrheum.150074.
17.
Adler BL, Timlin H, Birnbaum J. Lupus intestinal pseudo-obstruction and hydronephrosis: case report. Medicine (Baltimore) 2019; 98: e16178, DOI: 10.1097/MD.0000000000016178.
18.
Mok CC, Tang SSK, To CH, Petri M. Incidence and risk factors of thromboembolism in systemic lupus erythematosus: a comparison of three ethnic groups. Arthritis Rheum 2005; 52: 2774–2782, DOI: 10.1002/art.21224.
19.
Al-Homood IA. Thrombosis in systemic lupus erythematosus: a review article. ISRN Rheumatol 2012; 2012: 428269, DOI: 10.5402/2012/428269.
20.
Afeltra A, Vadacca M, Conti L, et al. Thrombosis in systemic lupus erythematosus: congenital and acquired risk factors. Arthritis Rheum 2005; 53: 452–459, DOI: 10.1002/art.21172.
21.
Manger K, Manger B, Repp R, et al. Definition of risk factors for death, end stage renal disease, and thromboembolic events in a monocentric cohort of 338 patients with systemic lupus erythematosus. Ann Rheum Dis 2002; 61: 1065–1070, DOI: 10.1136/ard.61.12.1065.
22.
Love PE, Santoro SA. Antiphospholipid antibodies: anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders: prevalence and clinical significance. Ann Intern Med 1990; 112: 682–698, DOI: 10.7326/0003-4819-112-9-682.
23.
Bazzan M, Vaccarino A, Marletto F. Systemic lupus erythematosus and thrombosis. Thromb J 2015; 13: 16, DOI: 10.1186/s12959-015-0043-3.
24.
de Groot PG, de Laat B. Mechanisms of thrombosis in systemic lupus erythematosus and antiphospholipid syndrome. Best Pract Res Clin Rheumatol 2017; 31: 334–341, DOI: 10.1016/ j.berh.2017.09.008.
25.
Chronic intestinal pseudo-obstruction – symptoms, causes, treatment. NORD. 2012. Available at: https://rarediseases.org/rare-... (Access: 20.03.2025).
26.
Oh DJ, Yang JN, Lim YJ, et al. Intestinal pseudo-obstruction as an initial manifestation of systemic lupus erythematosus. Intest Res 2015; 13: 282–286, DOI: 10.5217/ir.2015.13.3.282.
27.
Lan F, Li T, Zhou L, et al. Intestinal pseudo-obstruction in systemic lupus erythematosus complicated by Castleman disease: a case report. Ann Transl Med 2022; 10: 1148, DOI: 10.21037/atm-22-4461.
28.
Alshehri AM, Alhumaidi HM, Asseri YM, et al. Mesenteric vasculitis as a rare initial presentation of systemic lupus erythematosus: a case report. Saudi J Med Med Sci 2020; 8: 223–226, DOI: 10.4103/sjmms.sjmms_206_19.
29.
Alharbi S. Gastrointestinal manifestations in patients with systemic lupus erythematosus. Open Access Rheumatol Res Rev 2022; 14: 243–253, DOI: 10.2147/OARRR.S384256.
30.
Ayari M, Nakhli A, Teyeb Z, et al. Intestinal pseudo-obstruction: unusual presentation of systemic lupus erythematous. Clin Case Rep 2021; 9: 1759–1762, DOI: 10.1002/ccr3.3907.
31.
Xu N, Zhao J, Liu J, et al. Clinical analysis of 61 systemic lupus erythematosus patients with intestinal pseudo-obstruction and/or ureterohydronephrosis: a retrospective observational study. Medicine (Baltimore) 2015; 94: e419, DOI: 10.1097/MD.0000000000000419.
32.
Jin P, Ji X, Zhi H, et al. A review of 42 cases of intestinal pseudo-obstruction in patients with systemic lupus erythematosus based on case reports. Hum Immunol 2015; 76: 695–700, DOI: 10.1016/j.humimm.2015.09.022.
33.
Hajji M, Gorsane I, Badrouchi S, et al. Systemic lupus erythematosus presenting as lupus erythematosus tumidus and lupus nephritis: a case report. J Med Case Rep 2023; 17: 242, DOI: 10.1186/s13256-023-03981-3.
34.
Kim J, Kim N. Intestinal pseudo-obstruction: initial manifestation of systemic lupus erythematosus. J Neurogastroenterol Motil 2011; 17: 423–424, DOI: 10.5056/jnm.2011.17.4.423.
35.
Khairullah S, Jasmin R, Yahya F, et al. Chronic intestinal pseudo-obstruction: a rare first manifestation of systemic lupus erythematosus. Lupus 2013; 22: 957–960, DOI: 10.1177/0961203313492873.
36.
Almousa S, Wannous H, Khedr K, Qasem H. Unusual case presentation of systemic lupus erythematosus in a young woman. Rheumato 2022; 2: 93–97, DOI: 10.3390/rheumato2040013.
37.
PRISMA. PRISMA 2020 flow diagram. PRISMA Statement; Available at: https://www.prisma-statement.o... (Acccess: 15.10.2026).
38.
Weckerle CE, Niewold TB. The unexplained female predominance of systemic lupus erythematosus: clues from genetic and cytokine studies. Clin Rev Allergy Immunol 2011; 40: 42–49, DOI: 10.1007/s12016-009-8192-4.
39.
Zampeli E, Skopouli FN, Moutsopoulos HM. Polyserositis in a patient with active systemic lupus erythematosus: a case of pseudo-pseudo Meigs syndrome. J Rheumatol 2018; 45: 877–878, DOI: 10.3899/jrheum.171296.
40.
Chandwar K, Sahoo RR, Wakhlu A, Wakhlu A. Pneumatosis intestinalis: a rare manifestation of lupus enteritis. BMJ Case Rep 2022; 15: e247779, DOI: 10.1136/bcr-2021-247779.
41.
Stoichitoiu LE, Ionescu GD, Neatu I, Baicus C. Causes of polyserositis: a systematic review. J Pers Med 2023; 13: 834, DOI: 10.3390/jpm13050834.
42.
Fanouriakis A, Kostopoulou M, Andersen J, et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update. Ann Rheum Dis 2024; 83: 15–29, DOI: 10.1136/ard-2023-224762.
43.
Ribaldone DG, Bruno M, Gaia S, et al. Endoscopic ultrasound to diagnose pneumatosis cystoides intestinalis (with video). Endosc Ultrasound 2017; 6: 416–417, DOI: 10.4103/ eus.eus_88_17.
44.
Suárez-Ale H, Fabian-Aquino E, Failoc-Rojas VE, et al. Case report: systemic lupus erythematous associated with thrombotic thrombocytopenic purpura, a diagnostic challenge. F1000Res 2022; 10: 552, DOI: 10.12688/f1000research.51295.2.
45.
Amissah-Arthur MB, Gordon C. Contemporary treatment of systemic lupus erythematosus: an update for clinicians. Ther Adv Chronic Dis 2010; 1: 163–175, DOI: 10.1177/2040622310-380100.
46.
Bertsias G, Askanase A, Doria A, et al. A path to glucocorticoid stewardship: a critical review of clinical recommendations for the treatment of systemic lupus erythematosus. Rheumatology 2024; 63: 1837–1849, DOI: 10.1093/rheumatology/keae041.
47.
Pamuk ON. Thrombocytopenia in patients with systemic lupus erythematosus. Eur J Rheumatol 2023; 10: 159–162, DOI: 10.5152/eurjrheum.2023.23069.
48.
Yvamoto EY, Cheng S, Peixoto de Oliveira GH, et al. Endoscopic ultrasound view of pneumatosis cystoides intestinalis. Diagnostics 2023; 13: 1424, DOI: 10.3390/diagnostics13081424.
49.
Caudill JL, Rose BS. The role of computed tomography in the evaluation of pneumatosis intestinalis. J Clin Gastroenterol 1987; 9: 223–226, DOI: 10.1097/00004836-198704000-00024.
Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Share
RELATED ARTICLE
| eISSN: | 2084-9834 |
| ISSN: | 0034-6233 |
We process personal data collected when visiting the website. The function of obtaining information about users and their behavior is carried out by voluntarily entered information in forms and saving cookies in end devices. Data, including cookies, are used to provide services, improve the user experience and to analyze the traffic in accordance with the Privacy policy. Data are also collected and processed by Google Analytics tool (more).
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
