IgG4-associated disease in differential diagnosis of inflammatory orbitopathy

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Abstract


IgG4-associated disease (IgG4-RD) is a systemic inflammatory disease characterized by tumorlike sclerosing masses in different organs. Differential diagnosis in orbital IgG4-RD includes majority of conditions, such as thyroid eye disease (TED), sarcoidosis, granulomatosis with polyangiitis, idiopatic orbital inflammation, limphoproliferative diseases and others. A case of IgG4-RD with different organs involvement and complicated differential diagnosis is presented. This case demonstrates very uncommon manifestation of IgG4-RD, when orbital involvement was very similar with TED. Systemic process was not recognized during a long period of time and diagnosis of IgG4-RD was established only after biopsy of abnormally increased lacrimal gland. Differential diagnosis included other systemic diseases, first of all sarcoidosis, GPA, and lymphoma. Biopsy results were consistent with the gold standard of diagnosis, e. g. more than 40% of plasma cells were IgG4 positive. This case demonstrates the necessity of orbital biopsy before starting immunosuppression to avoid inappropriate treatment strategy.

Natalia Sviridenko

Endocrinology Research Centre (Moscow)

Author for correspondence.
Email: natsvir@inbox.ru
ORCID iD: 0000-0002-8538-5354
SPIN-code: 5889-6484
https://www.endocrincentr.ru/doctors/sviridenko-natalya-yurevna

Russian Federation, 117036, Moscow, Dm. Uljanova str. 11

BioStatement: MD, DSc, professor, the Deputy Director of the Institute of clinical endocrinology for medical work

  • Stone J.H., Khosroshahi A., Deshpande V. et al. Recommendations for the Nomenclature of IgG4-Related Disease and Its Individual Organ System Manifestations. Arthritis & Rheumatism. 2012; 64 (10): 3061–3067 https://doi.org/10.1002/art.34593
  • Седышев С.Х., Васильев В.И., Ковригина А.М. , Насонов Е.Л. IgG4-связанное системное заболевание. Современный взгляд на «старые» болезни// Научно-практическая ревматология.- 2012.- 50 (5).- C.64-72 [Sedyshev S. Kh., Vasil'ev, V., Kovrigina A. M. , Nasonov, E. L., IgG4-associated systemic disease. Modern view on "old" diseases// Scientific and practical rheumatology. 2012; 50 (5): 64-72 (In Russ).]
  • Hamano H, Arakura N, Muraki T. et al. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol. 2006; 41: 1197-1205 https://doi.org/10.1007/s00535-006-1908-9.
  • Takuma K, Kamisawa T, Anjiki H. et al. Metachronous extrapancreatic lesions in autoimmune pancreatitis. Intern Med. 2010; 49(6): 529-33. Epub 2010 Mar 15. https://doi.org/10.2169/internalmedicine.49.3038
  • Ferry J.A, Klepeis V., Sohani A.R. et al. IgG4-related Orbital Disease and Its Mimics in a Western Population. Am J Surg Pathol. 2015; 39 (12): 1688-1700 doi: 10.1097/pas.0000000000000497.
  • Masaki Y., Kurose N., Umehara H.. IgG4-related disease: a novel lymphoproliferative disorder discovered and established in Japan in the 21st century. J Clin Exp Hematop. 2011; 51: 13–20 https://doi.org/10.3960/jslrt.51.13
  • Sah R.P., Chari S.T. Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol. 2011; 23: 108–113 doi: 10.1097/bor.0b013e3283413469.
  • Strehl I.D., Hartmann A., Agaimy A. Numerous IgG4-positive plasma cells are ubiquitous in diverse localized non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol. 2011; 64: 237–243 doi: 10.1136/jcp.2010.085613.
  • Berry-Brincat A, Rose GE. Idiopathic orbital inflammation: a new dimension with the discovery of immunoglobulin G4-related disease. Curr Opin Ophthalmol. 2012; 23(5): 415-419 doi: 10.1097/ICU.0b013e32835563ec.
  • Tiegs-Heiden C.A., Eckel L.J., Hunt C.H. et al. Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients. AJNR Am J Neuroradiol. 2014; 35(7): 1393-1397 doi: 10.3174/ajnr.A3865.
  • Siakallis LC, Uddin JM, Miszkiel KA. Imaging Investigation of Thyroid Eye Disease. Ophthalmic Plast Reconstr Surg. 2018; 34(4S Suppl 1): S41-S51 doi: 10.1097/IOP.0000000000001139.
  • Hagiya C., Tsuboi H., Yokosawa M. et al. Clinicopathological features of IgG4-related disease complicated with orbital involvement. Mod Rheumatol. 2014; 24(3): 471-476 doi: 10.3109/14397595.2013.844307.
  • Detiger S.E., Karim A..F, Verdijk R.M. et al. The treatment outcomes in IgG4-related orbital disease: a systematic review of the literature. Acta Ophthalmol. 2019 Feb 7. doi: 10.1111/aos.14048.

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