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Case Report
Primary Sjogren’s syndrome presenting as acute pancreatitis and interstitial nephritis: A diagnostic challenge
1 Postgraduate Resident, Department of Internal Medicine, Fortis Memorial Research Institute, Gurugram, Haryana, India
2 Senior Director and Unit Head, Department of Internal Medicine, Fortis Memorial Research Institute, Gurugram, Haryana, India
3 Senior Consultant, Department of Rheumatology, Fortis Memorial Research Institute, Gurugram, Haryana, India
4 Director, Renal Pathology, Agilus Diagnostics, New Delhi, India
5 Attending Consultant, Department of Radiology, Fortis Memorial Research Institute, Gurugram, Haryana, India
Address correspondence to:
Amitabh Parti
Senior Director and Unit Head, Department of Internal Medicine, Fortis Memorial Research Institute, Gurugram, Haryana,
India
Message to Corresponding Author
Article ID: 101541Z01CT2026
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How to cite this article
Tatrari C, Parti A, Mendiratta N, Sabhikhi AK, Uberoi A. Primary Sjogren’s syndrome presenting as acute pancreatitis and interstitial nephritis: A diagnostic challenge. Int J Case Rep Images 2026;17(2):7–11.ABSTRACT
Primary Sjogren’s syndrome (pSS) is an autoimmune disorder primarily affecting the exocrine glands, with systemic involvement being increasingly recognized. Presentation with gastrointestinal involvement is a rare presentation of the disease. We report the case of a 72-year-old woman who presented with cholestatic jaundice, fever, and acute pancreatitis. During hospital stay, she also developed pulmonary involvement and an active urinary sediment, which significantly complicated the diagnosis. Serological testing revealed a strongly positive antinuclear antibody (ANA) titer along with anti-Smith antibodies, raising a strong suspicion for systemic lupus erythematosus (SLE). However, renal biopsy demonstrated a plasma cell predominant tubulointerstitial nephritis without immune complex deposition. This pattern effectively ruled out lupus nephritis and established the diagnosis of primary Sjogren’s syndrome. This case highlights the diagnostic challenge posed by overlapping autoimmune features and reinforces the importance of tissue biopsy in differentiating autoimmune mimics. Early recognition of atypical extra-glandular manifestations of pSS is essential for appropriate management and improved outcomes.
Keywords: Acute pancreatitis, Anti-Smith antibody, Primary Sjogren’s syndrome, Tubulointerstitial nephritis
SUPPORTING INFORMATION
Author Contributions
Chetan Tatrari - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Amitabh Parti - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Naval Mendiratta - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Abha K Sabhikhi - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Anmol Uberoi - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2026 Chetan Tatrari et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
