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CLINICAL IMAGE
Year : 2020  |  Volume : 152  |  Issue : 7  |  Page : 4

IgG4-related disease: A missed cause of hypereosinophilia


Department of Medicine, All India Institute of Medical Sciences, New Delhi 110 029, India

Date of Submission19-Nov-2019
Date of Web Publication25-May-2021

Correspondence Address:
Animesh Ray
Department of Medicine, All India Institute of Medical Sciences, New Delhi 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmr.IJMR_2160_19

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How to cite this article:
Shankar SH, Ray A. IgG4-related disease: A missed cause of hypereosinophilia. Indian J Med Res 2020;152, Suppl S1:4

How to cite this URL:
Shankar SH, Ray A. IgG4-related disease: A missed cause of hypereosinophilia. Indian J Med Res [serial online] 2020 [cited 2021 Jul 31];152, Suppl S1:4. Available from: https://www.ijmr.org.in/text.asp?2020/152/7/4/316755

Patient's consent obtained to publish clinical information and images.


A 44 yr old male presented to the department of Medicine, All India Institute of Medical Science, New Delhi, in December 2018, for evaluation of his previously diagnosed chronic kidney disease. Investigations revealed anaemia with hypereosinophilia (absolute eosinophil count = 2030 cells/mm3) with bilateral shrunken kidney and eosinophils in urine. After ruling out common causes of eosinophilia, a bone marrow biopsy was done. This showed increased eosinophilic precursors with no abnormal cells [Figure 1]A. Mutations (BCR-ABL-1, FIP1L1/PDGFRα fusion mutation, PDGFRβ, FGFR1) and interleukin (IL)-5 levels were negative. A positron emission tomography-computed tomography showed an fluorodeoxyglucose (FDG)-avid retroperitoneal mass [Figure 1]B and [Figure 1]C. Biopsy revealed obliterative phlebitis, fibrosis, lymphoplasmacytic and eosinophilic infiltrate [Figure 1]D. Immunohistochemistry stained positive for IgG4 plasma cells [Figure 1]E, and serum IgG4 level was elevated with low serum C3 level. A diagnosis of IgG4-related disease with tubulointerstitial nephritis was made. He was started on 0.5 mg/kg of prednisolone with maintenance haemodialysis but was lost to follow up after discharge. This case highlights IgG4-related disease as an uncommon aetiology in a patient presenting with hypereosinophilia.
Figure 1: (A) Bone marrow immunohistochemistry showing eosinophilic precursors with no abnormal cells (black arrowhead). (B) Cross-section of positron emission tomography-computed tomography (PET-CT) showing FDG-avid irregular retroperitoneal mass (green asterisk). (C) Maximum intensity projection of PET-CT showing FDG-avid retroperitoneal mass (red arrow). (D) Histopathology of the retroperitoneal mass showing fibrosis (black asterisk), obliterative phlebitis (red arrowhead), lymphoplasmacytic (black arrowhead) and eosinophilic infiltrate (black arrow). (E) Immunohistochemistry of the biopsy showing IgG4-positive plasma cells staining brown (black arrow).

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Conflicts of Interest: None.




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