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Case Report

IgG4 Related Disease Associated Retroperitoneal Fibrosis: A Rare Cause of Chronic Back Ache

Mahima Nautiyal, Kamal Preet Palta, Bibhash Roy

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Indian Journal of Emergency Medicine 11(1):p 49-54, Jan -April 2025. | DOI: https://doi.org/10.21088/ijem.2395.311X.11125.7

How Cite This Article:

Mahima Nautiyal, Kamal Preet Palta, Bibhash Roy. IgG4 Related Disease Associated Retroperitoneal Fibrosis: A Rare Cause of Chronic Back Ache. Ind J Emerg Med. 2025; 11(1): 49-54.Nautiyal M, Palta KP, Roy B. IgG4 related disease associated retroperitoneal fibrosis: a rare cause of chronic back ache. Ind J Emerg Med. 2025;11(1):49-54.

Timeline

Received : December 28, 2024         Accepted : February 28, 2025          Published : April 20, 2025

Abstract

Title of the article: Immuno-globulin G4 related disease associated retroperitoneal fibrosis: A rare cause of chronic back ache. Introduction: Retroperitoneal fibrosis is an uncommon presentation of IgG4 – Related Disease, often presenting with non-specific symptoms leading to delayed diagnosis and management. Case report: This case report describes a 67-year-old female who presented to the emergency department with complaints of gradually worsening back ache in the last 3 months; associated with urinary hesitancy and fever with chills; not resolving with antibiotics and analgesics. Contrast Enhanced Computed Tomography abdomen was suggestive of left pyelonephritis and retro-peritoneal infl-ammatory fibrosis. IgG4 sub class level of 1.71 (Reference range: 0.1 – 1.2). The patient was started on glucocorticoid therapy resulting in rapid symptoms resolution. Maintenance therapy with Tab. Mycophenolate mofetil 500mg once daily was started to prevent relapse. Conclusion: When lower back pain is present with symptoms like abdominal pain and obstructive urinary tract symptoms retroperitoneal fibrosis should be considered in the differential diagnosis. Recognizing the association is crucial for initiating appropriate therapy to prevent progression.


References

  • 1.   Nambiar S., Oliver T.I. IgG4-Related Disease. [Updated 2023 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi. nlm.nih.gov/books/NBK499825/.
  • 2.   Liu C., Zhang P., Zhang W. Immunological mechanism of IgG4-related disease. J Transl Autoimmun. 2020 Mar 19; 3: 100047. doi: 10.1016/j.jtauto.2020.100047. PMID: 32743528; PMCID: PMC7388377.
  • 3.   Kolasinski, S.L., Neogi, T., Hochberg, M. C., Oatis, C., Guyatt, G., Block, J., Callahan, L., Copenhaver, C., Dodge, C., Felson, D., Gellar, K., Harvey, W.F., Hawker, G., Herzig, E., Kwoh, C.K., Nelson, A.E., Samuels, J., Scanzello, C., White, D., Wise, B., Altman, R.D., DiRenzo, D., Fontanarosa, J., Giradi, G., Ishimori, M., Misra, D., Shah, A.A., Shmagel, A.K., Thoma, L.M., Turgunbaev, M., Turner, A.S., & Reston, J. (2020). 2019 American College of Rheumatology/Arthritis Foundation Guideline for the Management of Osteoarthritis of the Hand, Hip, and Knee. Arthritis & Rheumatology, 72(2), 220–233. https://doi.org/10.1002/art.41142.
  • 4.   Duggal L., Singh B.G., Patel J., Gupta M., Grover A.K., Jain N. IgG4-Related Disease: A Clinical Case Series From a Tertiary Care Center in India. J Clin Rheumatol. 2022 Jan 1;28(1):e56-e62. doi: 10.1097/ RHU.0000000000001591. PMID: 33105313.
  • 5.   Bhatt M., Kumar S., Soneja M., Vyas S., Tripathi M., Kumar R., Singh P., Sharma M.C., Wig N. Retrospective analysis of IgG4-related disease cases at a tertiary care centre in India. Reumatologia. 2022; 60(1): 4-11. doi: 10.5114/ reum.2022.113517. Epub 2022 Feb 10. PMID: 35645417; PMCID: PMC9132119.
  • 6.   Hisanori Umehara, Kazuichi Okazaki, Shigeyuki Kawa, Hiroki Takahashi, Hiroshi Goto, Shoko Matsui, Nobukazu Ishizaka, Takashi Akamizu, Yasuharu Sato, Mitsuhiro Kawano, the Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW) Japan., The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD, Modern Rheumatology, Volume 31, Issue 3, 4 May 2021, Pages 529–533, https:// doi.org/10.1080/14397595.2020.1859710.
  • 7.   Lian L., Wang C., Tian J.L. IgG4-related retroperitoneal brosis: a newly characterized disease. Int J Rheum Dis. 2016 Nov;19(11):1049- 1055. doi: 10.1111/1756-185X.12863. Epub 2016 Apr 29. PMID: 27125330.
  • 8.   Mitsuhiro Kawano, Takako Saeki, Hitoshi Nakashima, IgG4-related kidney disease and retroperitoneal fibrosis: An update, Modern Rheumatology, Volume 29, Issue 2, 4 March 2019, Pages 231–239, https://doi.org/10.1080 /14397595.2018.1554321.
  • 9.   Mizushima I., Kasashima S., Fujinaga Y., Notohara K., Saeki T., Zen Y., Inoue D., Yamamoto M., Kasashima F, Matsumoto Y., Amiya E., Sato Y., Yamada K., Domoto Y., Kawa S., Kawano M., Ishizaka N. Clinical and Pathological Characteristics of IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis Diagnosed Based on Experts’ Diagnosis. Ann Vasc Dis. 2019 Dec 25; 12(4): 460-472. doi: 10.3400/avd.oa.19-00085. PMID: 31942203; PMCID: PMC6957903.
  • 10.   Fujimori N., Ito T., Igarashi H., Oono T., Nakamura T., Niina Y., Hijioka M., Lee L., Uchida M., Takayanagi R. Retroperitoneal brosis associated with immunoglobulin G4- related disease. World J Gastroenterol. 2013 Jan 7; 19(1): 35-41. doi: 10.3748/wjg.v19.i1.35. PMID: 23326160; PMCID: PMC3545227.
  • 11.   Pucar D., Hinchcliff M. FDG PET vascular imaging in IgG4-RD: Potential and challenges. J Nucl Cardiol. 2022 Dec; 29(6): 2934-2937. doi: 10.1007/s12350-021-02887-1. Epub 2021 Dec 28. PMID: 34964084.
  • 12.   Jadhav K.K., Kumar V., Punatar CB, Joshi V.S., Sagade S.N. Retroperitoneal fibrosisclinical presentation and outcome analysis from urological perspective. Investig Clin Urol. 2017 Sep; 58(5): 371-377. doi: 10.4111/ icu.2017.58.5.371. Epub 2017 Aug 4. PMID: 28868510; PMCID: PMC5577335.

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There are no additional data available.

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This research received no funding.

Author Contributions

All authors contributed significantly to the work and approve its publication.

Acknowledgements

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Conflicts of Interest

The authors report no conflicts of interest in this work.


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Cite this article

Mahima Nautiyal, Kamal Preet Palta, Bibhash Roy. IgG4 Related Disease Associated Retroperitoneal Fibrosis: A Rare Cause of Chronic Back Ache. Ind J Emerg Med. 2025; 11(1): 49-54.Nautiyal M, Palta KP, Roy B. IgG4 related disease associated retroperitoneal fibrosis: a rare cause of chronic back ache. Ind J Emerg Med. 2025;11(1):49-54.


Licence:

Attribution-Non-commercial 4.0 International (CC BY-NC 4.0)

This license enables reusers to distribute, remix, adapt, and build upon the material in any medium or format for noncommercial purposes only, and only so long as attribution is given to the creator.


Received Accepted Published
December 28, 2024 February 28, 2025 April 20, 2025

DOI: https://doi.org/10.21088/ijem.2395.311X.11125.7

Keywords

Retroperitoneal fibrosisIgG4 related diseaseLower back painObstructive urinary tract symptomsGlucocorticoid therapy Emergency department

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Received December 28, 2024
Accepted February 28, 2025
Published April 20, 2025

licence


Attribution-Non-commercial 4.0 International (CC BY-NC 4.0)

This license enables reusers to distribute, remix, adapt, and build upon the material in any medium or format for noncommercial purposes only, and only so long as attribution is given to the creator.


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