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IgG4-related disease: an atypical presentation of steroid-responsive renal mass
  1. Shu Hui Ng1,
  2. Jing Shin Tay1 and
  3. Ee Ling Lai1,2
  1. 1Internal Medicine, Hospital Sultan Ismail, Johor Bharu, Malaysia
  2. 2Medical Department, Rheumatology Unit, Hospital Taiping, Taiping, Malaysia
  1. Correspondence to Dr Ee Ling Lai; lai.eeling{at}yahoo.com

Abstract

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterised by dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Serum IgG4 levels are typically elevated but half of the patients had normal serum IgG4 levels. IgG4-RD represents a spectrum of diseases that involve various organs such as the pancreas, liver, kidneys, and salivary glands often manifesting as diffuse organ enlargement or a mass-like lesion mimicking cancer. An increased incidence of malignancy among patients with IgG4-RD has been reported. Thus, differentiating malignancy from IgG4-RD manifestation is important as the treatment differs. Glucocorticoids are considered first-line therapy and should be started early to prevent fibrosis. Patients usually have an excellent clinical response to steroids, and poor steroid response is indicative of an alternative diagnoses such as malignancy. This case report describes a case of IgG4-RD with renal mass in a young man that resolved with glucocorticoid therapy alone.

  • connective tissue disease
  • urology
  • immunology

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Footnotes

  • Contributors ELL consulted and diagnosed the patient’s disease. SHN arranged for the patient’s tissue samples for staining. ELL, SHN and JST were involved in the care and management of the patient, and contributed equally to the writing up of this case report.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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