Coincidence of Systemic Lupus Erythematosus and ANCA-Associated Vasculitis: A Case Report with Perforation of Nasal Septum and Palate

Abstract

Background

An overlap of systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibodies- (ANCA-) associated vasculitis (AAV) is extremely uncommon and no clear definition has been proposed. The SLE/AAV overlap syndrome mainly affects kidney, blood count, nervous system and lung. However, few previous cases reported nasal septal and palatal perforation in this disorder.

Case Presentation

We presented a case of a 16-year-old female with a 6-month history of SLE, developed perforation of the nasal septum and palate. She was diagnosed with SLE due to facial malar rash, oral ulcer, increased erythrocyte sedimentation rate (ESR), low complement levels, and positive anti-Smith antibody. Approximately 6 months later, she had a perforation of the nasal septum and palate with positive anti-proteinase 3 antibody (anti-PR3-ANCA). A nasal endoscopic biopsy revealed an inflammatory polyp with chronic suppurative inflammation and inflammatory granulomatous hyperplasia. In this case, the clinical, biological, radiological, and histological findings substantiated the diagnosis of AAV. Infections, drug abuse, malignancies, IgG4-related disease (IgG4-RD) and trauma were excluded. So we diagnosed her with SLE/AAV overlap syndrome.

Conclusion

When a patient’s symptoms cannot be explained by one disease, we need to consider the overlapping of two diseases, especially in patients with autoimmune diseases.

Keywords:

• perforation of palate
• systemic lupus erythematosus
• ANCA-associated vasculitis
• SLE/AAV overlap syndrome

Data Sharing Statement

The data that support the findings of this study are available from the corresponding author (Jing Wang) upon request.

Ethical Approval and Consent to Participate

Written informed consent was obtained from the patient and the patient’s parents for the publication of all the images and data included in this article. The study was conducted in accordance with the Declaration of Helsinki (as revised in 2013). Ethical review and approval were not required to publish the case details in accordance with the institutional requirements.

Consent to Publish

The patient provided informed consent to publish their case details and any accompanying images.

Acknowledgments

All of us thank the patient and the patient’s parents for consent to the publication of the case.

Author Contributions

Jing Wang serves as the corresponding author for this paper. All authors made a significant contribution to the work reported, whether that is in the conception, study design, execution, acquisition of data, analysis and interpretation, or in all these areas; took part in drafting, revising or critically reviewing the article; gave final approval of the version to be published; have agreed on the journal to which the article has been submitted; and agree to be accountable for all aspects of the work.

Disclosure

The authors report no conflicts of interest in this work.

Additional information

Funding

No funding was received for this study.