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J Nephropathol. 2013;2(2): 144-149.
doi: 10.12860/JNP.2013.24
PMID: 24475442
PMCID: PMC3891151
Scopus ID: 84881177814
  Abstract View: 4281
  PDF Download: 1808

Case Report

The first case of IgG4-related disease in Italy

Davide Rolla 1*, Diego Bellino 1, Giancarlo Peloso 1, Maria Pia Rastaldi 3, Paola Simonini 3, Jean Louis Ravetti 2
*Corresponding Author: *Corresponding author:Davide Rolla, Divisione di Nefrologia –Dialisi - Trapianto, AziendaOspedalieraUniversitaria S. Martino, Genova, Italy. Tel: +39 0105553879, Fax: +390105556652,, Email: davide.rolla@hsanmartino.it

Abstract

Background: Recently, Mikulicz’s disease has been defined as an IgG-4 related disease, a systemic condition, where the hallmark pathology findings are lymphoplasmacytic infiltrates, immunoglobulin (Ig)G4-positive plasma cells, modest tissue eosinophilia, and intense fibrosis.

Case: We present a case of 63-year-old man who showed epigastralgia and elevated serum lipase levels. Computed tomography of the abdomen revealed a bulky mass of the pancreas, so he underwent bilious-digestive anastomosis, and biopsy of the pancreas revealed massive infiltration of lymphocytes and plasma cells. The patient was therefore diagnosed with sclerosing chronic pancreatitis (Kuttner’s tumour). After one year, the patient began to exhibit signs of “sicca syndrome”, and at the same time, he demonstrated progressive renal failure. Immunological tests showed hypocomplementemia, and the renal biopsy specimen demonstrated interstitial inflammation, in which infiltrate was composed of lymphocytes, while infiltrating plasma cells showed immunoreactivity to IgG4. Sialography revealed severe involvement of the salivary glands, and Schirmer’s test resulted positive.

Conclusions:  Here, we report successful treatment of the first case in Italy of a patient with hypocomplementemic tubulointerstitial nephritis in IgG4-related disease.


Implication for health policy/practice/research/medical education:

Recently, Mikulicz’s disease has been defined as an IgG-4 related disease, a systemic condition, where the hallmark pathology findings are lymphoplasmacytic infiltrates, IgG-4-positive plasma cells, modest tissue eosinophilia, and intense fibrosis. 

Please cite this paper as:Rolla D, Bellino D, Peloso GC, Rastaldi MP, Simonini P, Ravetti JL. The first case of IgG4-related disease in Italy.J Nephropathology. 2013; 2(2): 144-149. DOI: 10.5812/nephropathol.10549

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Revision: 10 Dec 2012
ePublished: 01 Apr 2013
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