CEN Case Reports

, Volume 8, Issue 1, pp 23–30 | Cite as

A case of fatal osteolytic hypercalcemia complicated with IgG4-related ophthalmic disease leading to renal failure

  • Chieko KawakitaEmail author
  • Masaru Kinomura
  • Yoshie Gon
  • Chika Okita
  • Katsuyoshi Katayama
  • Mana Nishikawa
  • Noriaki Shimada
  • Kenji Notohara
  • Masaki Fukushima
  • Kenichiro Asano
Case Report


A 40-year-old male was hospitalized with renal impairment and severe hypercalcemia. His concentration of serum IgG4 was high, but serum whole PTH, 1–25(OH)2 vitamin D3 and PTHrP were not elevated. Computed tomography showed swelling of the bilateral lacrimal glands and systemic lymphadenopathy. The histological findings of lacrimal gland biopsy fulfilled the diagnostic criteria of IgG4-related ophthalmic disease (IgG4ROD). Bone scintigraphy showed increased ectopic uptake in the stomach, heart, lungs, and kidneys. He died on day 16 of admission, although the therapies for hypercalcemia were continued. Autopsy results showed an increase of osteoclasts in the bone marrow and metastatic calcification in multiple organs, and excluded from the differential diagnosis other disorders which present lymph-node swelling and hypercalcemia such as cancer, lymphoma, Castleman’s disease, and sarcoidosis. He was given a diagnosis of IgG4ROD with osteolytic hypercalcemia.


IgG4-related disease Hypercalcemia Metastatic calcification 



We thank Natsuki Matsumoto (Bone Analysis Section, Kureha Special Laboratory Co., Ltd), Taketoshi Shimakura, Noriaki Yamamoto, and Hideaki E. Takahashi (Niigata Bone Science Institute) for performing bone morphometric analysis.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no competing interest.

Human and animal rights

This article does not contain any studies with human participants performed by any of the authors.

Informed consent

Informed consent was obtained from participants included in the article.


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Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  • Chieko Kawakita
    • 1
    • 5
    Email author
  • Masaru Kinomura
    • 1
  • Yoshie Gon
    • 2
  • Chika Okita
    • 3
  • Katsuyoshi Katayama
    • 1
  • Mana Nishikawa
    • 1
  • Noriaki Shimada
    • 1
  • Kenji Notohara
    • 3
  • Masaki Fukushima
    • 4
  • Kenichiro Asano
    • 1
  1. 1.Department of NephrologyKurashiki Central HospitalKurashikiJapan
  2. 2.Department of RheumatologyKurashiki Central HospitalKurashikiJapan
  3. 3.Department of Anatomic PathologyKurashiki Central HospitalKurashikiJapan
  4. 4.Department of Internal MedicineShigei Medical Research HospitalOkayamaJapan
  5. 5.Department of Nephrology, Rheumatology, Endocrinology and MetabolismOkayama University Graduate School of Medicine, Dentistry and Pharmaceutical SciencesOkayamaJapan

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