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Management of granulomatosis with polyangiitis complicated by intestinal perforation and pancytopenia: a case report and literature review

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Abstract

Granulomatosis with polyangiitis is a systemic vasculitis. While the classic triad typically comprises otorhinolaryngologic, pulmonary, and renal manifestations, it is essential to recognize that granulomatosis with polyangiitis can affect any organ. Furthermore, reports have documented less common sites of involvement, such as the gastrointestinal tract. In this case-based review, we focus on a case of granulomatosis with polyangiitis presenting with intestinal perforation and the added challenge of concurrent pancytopenia.

A 25-year-old female was diagnosed with granulomatosis with polyangiitis, with her clinical course progressing from joint pain to severe multi-organ involvement, including gastrointestinal complications. Treatment challenges emerged with the development of pancytopenia. While this may not directly result from granulomatosis with polyangiitis, it introduced an additional layer of complexity and delayed the induction of remission with immunosuppressants. Despite initial stabilization, an unexpected jejunal perforation occurred, requiring surgical intervention and subsequent postoperative care. The case underscores the complex nature of granulomatosis with polyangiitis and its potential complications. A literature search yielded discrete relevant cases in the context of our patient’s intricate presentation, which has been summarized.

We highlight the complexities in diagnosing and managing granulomatosis with polyangiitis-related complications, especially in uncommon presentations, and emphasize the importance of a personalized approach to patient care in these circumstances.

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Acknowledgements

The authors thank Dr. Olexandr Kit, MD, from the 1st Surgery Department at Ternopil Regional Hospital, for providing intraoperative photographs of the patient’s intestinal perforation site.

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The authors received no financial support for the research, authorship, and/or publication of this work.

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Authors and Affiliations

  1. 2nd Department of Internal Medicine, Ivan Horbachevsky Ternopil National Medical University, Majdan Voli, 1, Ternopil, 46001, Ukraine

    Svitlana Smiyan, Oksana Kvasnitska, Taras Bidovanets & Roman Komorovsky

  2. Department of Morphology, Clinical Pathology and Forensic Medicine, Shupyk National Healthcare University of Ukraine, Kyiv, Ukraine

    Olena Dyadyk

  3. Department of Rheumatology, Ternopil Regional Hospital, Ternopil, Ukraine

    Olexandra Makhovska & Zhanna Antiuk

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  1. Svitlana Smiyan
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Contributions

SS contributed to conception and design of the work, analyzed and interpreted the patient’s data, performed the review of literature and drafted the manuscript; OD performed the pathology studies, interpreted the results, and contributed to drafting the manuscript; OK, ZA, OM, and TB analyzed the patient’s data, performed the review of literature, and contributed to drafting the manuscript; RK contributed to conception and design of the work, contributed to drafting the manuscript and revised it critically for important intellectual content. All authors read and approved the final version of the manuscript, and agreed to be accountable for all aspects of the work.

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Correspondence to Roman Komorovsky.

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Smiyan, S., Dyadyk, O., Kvasnitska, O. et al. Management of granulomatosis with polyangiitis complicated by intestinal perforation and pancytopenia: a case report and literature review. Rheumatol Int (2024). https://doi.org/10.1007/s00296-024-05586-5

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