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LETTER TO EDITOR
Ahead of print publication  

It melts like snow: Steroid responsiveness of immunoglobulin G4-related disease


 Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Date of Submission21-Jul-2022
Date of Acceptance16-Aug-2022
Date of Web Publication20-Sep-2022

Correspondence Address:
Urmila Dhakad,
Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_147_22



How to cite this URL:
Mukherjee S, Maurya MK, Mehta P, Dhakad U. It melts like snow: Steroid responsiveness of immunoglobulin G4-related disease. Indian J Rheumatol [Epub ahead of print] [cited 2022 Oct 3]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=356479

Dear Editor,

A 35-year-old male presented with insidious onset gradually progressive bilateral painless submandibular swelling, followed by periorbital swelling [Figure 1] for the past 7 years. He had no constitutional or sicca symptoms. He had no history of addiction or autoimmunity in the family. Systemic examination was unremarkable. On evaluation, he had raised inflammatory markers with a negative autoimmune panel. Magnetic resonance imaging orbit showed bilateral orbital extraconal soft-tissue masses with contrast enhancement. Incisional biopsy from the extraconal mass was suggestive of fibrocollagenous stroma with aggregation of lymphocytes, plasma cells, and histiocytes without any necrosis or granuloma. On further evaluation, submandibular gland biopsy showed chronic sialadenitis with immunoglobulin G4 (IgG4)-positive plasma cell >10/hpf and IgG4: IgG ratio >40% [Figure 2] without any major organ involvement on imaging. The diagnosis of IgG4-related disease was kept. He was started on oral steroid 0.5 mg/kg/day and observed a rapid clinical response within 15 days of steroid therapy. Later, he was started on azathioprine and continued with tapering dose of steroid. He was followed up every month for first 3 months and then every 3 monthly upto 16 months without any signs of recurrence. For a long-standing indolent disease of 7 years, drastic steroid response with resolution of clinical symptoms was quite evident in our case.
Figure 1: (a) Pretreatment bilateral upper eyelid swelling, (b) pretreatment bilateral submandibular swelling, (c and d) during glucocorticoid therapy (day 15) resolution of orbital and submandibular swelling, respectively

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Figure 2: Salivary gland with seromucinous acini and ducts embedded in mildly fibrotic stroma with numerous lymphoid follicles with germinal centers (a and b: H and E). Clusters of plasma cells are highlighted with CD138 immunohistochemical stain (c). IgG4 positive cells, >10/hpf (d)

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IgG4-related disease (IgG4-RD) is a chronic, systemic, fibroinflammatory condition with variable organ manifestation. CD4+ T-cells and B-cells are central to the pathogenesis causing organ damage and fibrosis. Marked expansion of IgG4-secreting plasmablast in the blood is usually seen in active and untreated cases.[1] Key to diagnosis is the classical histopathological hallmarks of lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. IgG4 RD usually has a good response to steroids but a variable relapse rate. Factors associated with lesser treatment response and relapse were well analyzed in a Japanese multicenter study. Multivariate analysis from the study revealed that intermediate steroid initial dose (0.4–0.69 mg/day), slow tapering regimen (<0.4 mg/day), and shorter disease duration were associated with a significantly lower rate of disease relapse.[2] The study of isolated IgG4 ophthalmic disease also echoed similar results regarding steroid response and relapse, with more relapse in those with elevated serum IgG4 levels and a shorter duration of initial steroid treatment (<5 months). Low-dose steroid maintenance should be considered to avoid recurrence in patients with elevated serum IgG4 levels.[3] Later on, rituximab therapy showed promising results in inducing remission[4] and steroid-free long-term maintenance.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Acknowledgment

The author would like to thank Department of Pathology, Sanjay Gandhi Post Graduate institute, Lucknow, U.P.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Pathogenesis of IgG4-Related Disease: A Critical Review | Springer Link. Available from: https://link.springer.com/article/100.1007/s10266-018-0377-y. [Last accessed on 2022 Aug 06].  Back to cited text no. 1
    
2.
Shirakashi M, Yoshifuji H, Kodama Y, Chiba T, Yamamoto M, Takahashi H, et al. Factors in glucocorticoid regimens associated with treatment response and relapses of IgG4-related disease: A multicentre study. Sci Rep 2018;8:10262.  Back to cited text no. 2
    
3.
Hong JW, Kang S, Song MK, Ahn CJ, Sa HS. Clinicoserological factors associated with response to steroid treatment and recurrence in patients with IgG4-related ophthalmic disease. Br J Ophthalmol 2018;102:1591-5.  Back to cited text no. 3
    
4.
Rituximab for IgG4-Related Disease: A Prospective, Open-Label Trial | Annals of the Rheumatic Diseases. Available from: https://ard.bmj.com/content/74/6/1171.long. [Last accessed on 2022 Jul 20].  Back to cited text no. 4
    
5.
Campochiaro C, Della-Torre E, Lanzillotta M, Bozzolo E, Baldissera E, Milani R, et al. Long-term efficacy of maintenance therapy with rituximab for IgG4-related disease. Eur J Intern Med 2020;74:92-8.  Back to cited text no. 5
    


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