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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
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A masquerading mass: an unusual presentation of IgG4-related systemic disease with tubulointerstitial nephritis

B Quinn1, J Harty, W Habeichi

  • 1J Harty Daisy Hill Hospital Renal Unit 5 Hospital Road Newry BT35 8DR County Down Northern Ireland. Email john.harty@southerntrust.hscni.net.

The Journal of the Royal College of Physicians of Edinburgh
|July 8, 2014
PubMed

IgG4 tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4 renal disease. When IgG4-TIN is accompanied by other systemic manifestations the disease is known as IgG4-related systemic disease (IgG4-RSD). IgG4-RSD is well recognised in the form of tubulointerstitial nephritis (IgG4-related TIN) and may present with renal failure by mimicking neoplasms (tumefactive lesions) or with both features. We describe a case of IgG4-RSD initially presenting as a submandibular mass and subsequently presenting two years later with secondary infiltration of the kidney causing tubulointerstitial nephritis. This case highlights the importance of recognising IgG4-RSD as a non-malignant disease with presentations having commonly shared features including tumour-like swelling of involved organs and its ability to mimic many systemic diseases. In the majority of patients it can be treated successfully with corticosteroids.

Keywords:
IgG4-related systemic disease (IgG4-RSD)Küttner’s tumourchronic sclerosing sialadenitistubulointerstitial nephritis

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