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IgG4-related disease: association between chronic rhino-sinusitis and systemic symptoms

  • Rhinology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

Abstract

Purpose

The objective of this study is to analyze the relationship between chronic rhino-sinusitis (CRS) and systemic symptoms in patients with IgG4-related disease (IgG4-RD).

Patients and methods

The patients with IgG4-RD, confirmed by restrict association with clinical and histopathological manifestations between March 2013 and July 2016, were enrolled and followed-up for 1 year at the Tongren Hospital, Capital Medical University. The patients were divided into two groups: the case group included IgG4-RD patients with CRS confirmed by clinical and imaging, while the control group included IgG4-RD patients without CRS confirmed by clinical and imaging. Age, gender, clinical manifestations, the percentage of eosinophils in peripheral blood, sedimentation (ESR), C-reaction protein, serum IgE and IgG4 levels, histopathology, and treatment drugs at the baseline and 1 year of follow-up were compared between the two groups.

Results

A total of 46 cases met the diagnostic criteria for IgG4-RD. A total of 30 patients (65.2%) had IgG4-RD complicated with CRS, and were aged 49.7 ± 13.4 years, with male:female ratio = 2:1. The disease duration in the case group was longer than that in the control group (3.0 versus 0.8, p = 0.009). The ratio of ocular involvement was higher (86.7 versus 60%, p < 0.001), and allergic manifestations including drug allergy, asthma, and allergic skin were more common (56.5 versus 20%, p = 0.004), with a higher percentage of eosinophils in peripheral blood (8.5 versus 3.3%, p = 0.018) and more sensitive to glucocorticoids (6.0 versus 3.5, p = 0.004) than those in the control group.

Conclusions

CRS in patients with IgG4-RD was closely associated with IgG4-related ocular lesions, which was more prone to allergic manifestations accompanied by raised percentage of eosinophils in peripheral blood. The treatment of patients with IgG4-RD complicated with CRS was more effective than those with IgG4-RD without CRS.

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References

  1. Hamano H et al (2001) High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344(10):732–738

    Article  PubMed  CAS  Google Scholar 

  2. Stone JH et al (2012) Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 64(10):3061–3067

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  3. Stone JH, Zen Y and V (2012) Deshpande, IgG4-related disease. N Engl J Med 366(6):539–551

    Article  PubMed  CAS  Google Scholar 

  4. Kamisawa T et al (2015) IgG4-related disease. Lancet 385(9976):1460–1471

    Article  PubMed  CAS  Google Scholar 

  5. Campochiaro C et al (2016) IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients. Scand J Rheumatol 45(2):135–145

    Article  PubMed  CAS  Google Scholar 

  6. Agaimy A, Ihrler S (2014) Immunoglobulin G4 (IgG4)-related disease. A review of head and neck manifestations. Pathologe 35(2):152–159

    Article  PubMed  CAS  Google Scholar 

  7. Wei TW et al (2015) Chronic sclerosing sialadenitis of the submandibular gland: an entity of IgG4-related sclerosing disease. Int J Clin Exp Pathol 8(7):8628–8631

    PubMed  PubMed Central  Google Scholar 

  8. Bhatti RM, Stelow EB (2013) IgG4-related disease of the head and neck. Adv Anat Pathol 20(1):10–16

    Article  PubMed  CAS  Google Scholar 

  9. Takano K et al (2015) Clinical evaluation of sinonasal lesions in patients with immunoglobulin G4-related disease. Ann Otol Rhinol Laryngol 124(12):965–971

    Article  PubMed  Google Scholar 

  10. Moteki H et al (2011) IgG4-related chronic rhinosinusitis: a new clinical entity of nasal disease. Acta Otolaryngol 131(5):518–526

    Article  PubMed  Google Scholar 

  11. Inoue A et al (2016) IgG4-related disease in the sinonasal cavity accompanied by intranasal structure loss. Auris Nasus Larynx 43(1):100–104

    Article  PubMed  Google Scholar 

  12. Ohno K et al (2015) Nasal manifestations of IgG4-related disease: a report of two cases. Auris Nasus Larynx 42(6):483–487

    Article  PubMed  Google Scholar 

  13. Umehara H et al (2012) Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumat of ol 22(1):21–30

    Article  CAS  Google Scholar 

  14. Fokkens WJ et al (2012) European position paper on rhinosinusitis and nasal polyps 2012. Rhinol Suppl 23:1–298

    Google Scholar 

  15. Piao Y et al (2016) Concomitant occurrence of Mikulicz’s disease and immunoglobulin G4-related chronic rhinosinusitis: a clinicopathological study of 12 cases. Histopathology 68(4):502–512

    Article  PubMed  Google Scholar 

  16. Carruthers MN et al (2012) Development of an IgG4-RD Responder Index. Int J Rheumatol 2012:259408

    Article  PubMed  PubMed Central  Google Scholar 

  17. Fernandez-Codina A et al (2015) IgG4-related disease: results from a multicenter Spanish Registry. Medicine (Baltimore) 94(32):e1275

    Article  CAS  Google Scholar 

  18. Martinez-Valle F et al (2017) IgG4-related disease: Evidence from six recent cohorts. Autoimmun Rev 16(2):168–172

    Article  PubMed  CAS  Google Scholar 

  19. Takano K et al (2017) Recent advances in knowledge regarding the head and neck manifestations of IgG4-related disease. Auris Nasus Larynx 44(1):7–17

    Article  PubMed  Google Scholar 

  20. Ebbo M et al (2017) Ophthalmic manifestations in IgG4-related disease: clinical presentation and response to treatment in a French case-series. Medicine (Baltimore) 96(10):e6205

    Article  Google Scholar 

  21. Ishida M et al (2009) Multiple IgG4-related sclerosing lesions in the maxillary sinus, parotid gland and nasal septum. Pathol Int 59(9):670–675

    Article  PubMed  Google Scholar 

  22. Wallace ZS et al (2015) IgG4-related disease: baseline clinical and laboratory features in 125 patients with biopsy-proven disease. Arthritis Rheumatol 67(9):2466–2475

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  23. Chen Y et al (2016) Types of organ involvement in patients with immunoglobulin G4-related disease. Chin Med J (Engl) 129(13):1525–1532

    Article  Google Scholar 

  24. Li J, Ge X, Ma JM (2016) Relationship between dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and paranasal sinusitis. Int J Ophthalmol 9(3):444–447

    PubMed  PubMed Central  Google Scholar 

  25. Gomes GL et al (2010) Association between saddle nose deformity and retro-orbital mass in Wegener’s granulomatosis. Acta Reumatol Port 35(3):340–345

    PubMed  Google Scholar 

  26. Kwan AS, Rose GE (1998) Orbital Wegener’s granuloma resulting from direct extension of nasal disease through a surgical rhinostomy. Br J Ophthalmol 82(2):198

    Article  PubMed  CAS  Google Scholar 

  27. Culver EL et al (2017) Increases in IgE, eosinophils, and mast cells can be used in diagnosis and to predict relapse of IgG4-related disease. Clin Gastroenterol Hepatol 15(9):1444–1452.e6

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  28. Della-Torre E, Lanzillotta M, Doglioni C (2015) Immunology of IgG4-related disease. Clin Exp Immunol 181(2):191–206

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  29. Moosig F et al (2011) Targeting interleukin-5 in refractory and relapsing Churg-Strauss syndrome. Ann Intern Med 155(5):341–343

    Article  PubMed  Google Scholar 

  30. Blom K et al (2012) Eosinophil associated genes in the inflammatory bowel disease 4 region: correlation to inflammatory bowel disease revealed. World J Gastroenterol 18(44):6409–6419

    Article  PubMed  PubMed Central  Google Scholar 

  31. Marzano AV et al (2009) Activation of blood coagulation in bullous pemphigoid: role of eosinophils, and local and systemic implications. Br J Dermatol 160(2):266–272

    Article  PubMed  CAS  Google Scholar 

  32. Min JY, Hulse KE, Tan BK (2016) B-cells and antibody-mediated pathogenesis in chronic rhinosinusitis with nasal polyps. Adv Otorhinolaryngol 79:48–57

    PubMed  Google Scholar 

  33. Kawa S et al (2012) The utility of serum IgG4 concentrations as a biomarker. Int J Rheumatol 2012: 198314

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  34. Lv J,Liu HG (2013) Expression and significance of IgG4 in inflammatory disease of nasal cavity and paranasal sinuses. Zhonghua Bing Li Xue Za Zhi 42(6):386–391

    Google Scholar 

  35. Stone JH et al (2001) A disease-specific activity index for Wegener’s granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum 44(4):912–920

    Article  PubMed  CAS  Google Scholar 

  36. Khosroshahi A et al (2015) International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol 67(7):1688–1699

    Article  PubMed  CAS  Google Scholar 

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Acknowledgements

We thank all the patients who participated in this research.

Funding

This work was supported by the priming scientific research foundation for the senior researcher in Beijing Tongren Hospital, Capital Medical University (2015-YJJ-GGL-016); Municipal Key Laboratory of Nasal Diseases, Capital Medical University (2014BBYJ02). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

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Authors and Affiliations

  1. Department of Rheumatology, Beijing Tongren Hospital, Capital Medical University, Beijing, China

    Yuan Gao, Li Cui, Nan Chen, Yan-ni Wang & Zhen-gang Wang

  2. Department of Otolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China

    Ming Zheng

  3. Department of Gastroenterology, Beijing Tongren Hospital, Capital Medical University, Beijing, China

    Yu-tao Zhan

Authors
  1. Yuan Gao
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  2. Ming Zheng
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  3. Li Cui
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  4. Nan Chen
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  5. Yan-ni Wang
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  6. Yu-tao Zhan
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  7. Zhen-gang Wang
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Corresponding author

Correspondence to Yu-tao Zhan.

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Ethical approval

This study was approved by the Academic and Ethics Committee of Beijing Tongren Hospital in accordance with the principles of the Declaration of Helsinki.

Informed consent

All participants provided written informed consent to participate in this study.

Conflict of interest

The authors declare that they have no conflict of interest.

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Gao, Y., Zheng, M., Cui, L. et al. IgG4-related disease: association between chronic rhino-sinusitis and systemic symptoms. Eur Arch Otorhinolaryngol 275, 2013–2019 (2018). https://doi.org/10.1007/s00405-018-5013-5

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  • DOI: https://doi.org/10.1007/s00405-018-5013-5

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