A 22-year-old female presented with an 18-month history of frequent urination and urinary urgency. Her physical examination showed a cushingoid appearance and no obvious tenderness or palpable mass in the renal and ureteral walking areas. Her past medical history was notable for systemic lupus erythematosus (SLE) and lupus nephritis diagnosed 10 years ago, and SLE-associated intestinal pseudo-obstruction (IPO) diagnosed 3 years ago. Her IPO achieved remission after treatment with intravenous methylprednisolone and cyclophosphamide (cumulative dose 5 g). Her methylprednisolone was tapered to 8 mg/day, when she suffered from frequent urination and urinary urgency. Laboratory tests showed a urinary albumin level of 0.3 g/L and a serum creatinine level of 88 μmol/L (45–84 μmol/L). Assessment for SLE revealed positive anti–Sjögren’ssyndrome-related antigen A (anti-SSA) antibody and a reduced level of serum complement Component 3. No other system involvement or autoantibody positivity was revealed. Computed tomography urography (CTU) was remarkable, with bilateral ureterohydronephrosis (Figure 1). She was diagnosed with ureterohydronephrosis attributed to SLE. Bilateral double-J ureteral stents were placed, and she was treated with prednisone (15 mg/day) and intravenous cyclophosphamide (0.4 g/week) for 6 months. One year later, there was no improvement in the ureterohydronephrosis status or her renal function (Figure 1).

Images of the ureteropelvic system of the patient. CTU (A) with three-dimensional image reconstruction (B) showed bilateral severe ureterohydronephrosis when the patient presented to our hospital; One year later, CTU (C) with three-dimensional image reconstruction (D) revealed the unrelieved ureterohydronephrosis despite placement of double-J ureteral stents.
IPO and ureterohydronephrosis are both uncommon and severe complications of SLE, with a prevalence of 1%–2% in SLE.[1] In addition to vasculitis, mechanisms including primary myopathy, neuropathy, serositis, and direct damage caused by antibodies may be involved.[2, 3] Due to lack of specific symptoms, this rare manifestation is likely to be underrecognized.[1] Clinicians should consider possible underlying SLE when encountering young women with unexplained IPO and/or bilateral ureterohydronephrosis. Timely recognition, a thorough screening, and an early aggressive therapy are crucial to improve prognosis.[4] As in our case, ureteral aperistalsis persisted despite a delayed immunosuppressive treatment, compared to the favorable outcome of IPO benefit from timely intervention.
Funding statement: This work was supported by the Chinese National Key Technology Research and Development Program, Ministry of Science and Technology (2017YFC0907601, 2017YFC0907602), Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences (2019-I2M-2-008), and Beijing Municipal Science and Technology Commission (No. Z201100005520023, Z201100005520027). The funding body had no role in data collection, data analysis, decision to publish, or writing the manuscript.
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Author Contributions
None declared.
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Informed consent
Informed consent has been obtained. The patient has given her consent for her images and other clinical information to be reported in the journal.
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Ethical Statement
None declared.
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Conflict of Interest
Jiuliang Zhao is an Editorial Board Member of the journal. The article was subject to the journal’s standard procedures, with peer review handled independently of the editor and his research groups.
References
[1] 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;43:559–564.10.3899/jrheum.150074Search in Google Scholar PubMed
[2] Khairullah S, Jasmin R, Yahya F, et al. Chronic Intestinal Pseudo-Obstruction: A Rare First Manifestation of Systemic Lupus Erythematosus. Lupus. 2013;22:957–960.10.1177/0961203313492873Search in Google Scholar PubMed
[3] Hill PA, Dwyer KM, Power DA. Chronic Intestinal Pseudo-Obstruction in Systemic Lupus Erythematosus Due to Intestinal Smooth Muscle Myopathy. Lupus. 2000;9:458–463.10.1191/096120300678828505Search in Google Scholar PubMed
[4] Chen YQ, Xue Q, Wang NS. Visceral Muscle Dysmotility Syndrome in Systemic Lupus Erythematosus: Case Report and Review of the Literature. Rheumatol Int. 2012;32:1701–1703.10.1007/s00296-011-1856-4Search in Google Scholar PubMed
© 2022 Wanting Qi, Yangzhong Zhou, Jiuliang Zhao, published by De Gruyter
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
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Articles in the same Issue
- Editorial
- Editorial for “Social Media in Rheumatology”
- Social media and its impact on rheumatology
- Commentary
- How Twitter use in rheumatology has evolved
- Perspective
- Social media and the patient – on education and empowerment
- An overview of social media within APLAR rheumatology societies
- Review
- #RheumTwitter – The rise of social media in rheumatology: Research, collaboration, education, and engagement
- Social media for research discourse, dissemination, and collaboration in rheumatology
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