The first case of IgG4-related disease in Italy

Davide Rolla, Diego Bellino, Giancarlo Peloso, Maria Pia Rastaldi, Paola Simonini, Jean Louis Ravetti

Research output: Contribution to journalArticle

4 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)144-149
Number of pages6
JournalJournal of Nephropathology
Volume2
Issue number2
DOIs
Publication statusPublished - 2013

Fingerprint

Plasma Cells
Italy
Immunoglobulin G
Pancreas
Sialography
Lymphocytes
Biopsy
Interstitial Nephritis
Immunologic Tests
Sjogren's Syndrome
Chronic Pancreatitis
Eosinophilia
Salivary Glands
Lipase
Abdomen
Renal Insufficiency
Immunoglobulins
Fibrosis
Tomography
Pathology

Keywords

  • Hypocomplementemic tubulointerstitial nephritis
  • IgG-4 related disease
  • Mikulicz’s disease

ASJC Scopus subject areas

  • Nephrology

Cite this

Rolla, D., Bellino, D., Peloso, G., Rastaldi, M. P., Simonini, P., & Ravetti, J. L. (2013). The first case of IgG4-related disease in Italy. Journal of Nephropathology, 2(2), 144-149. https://doi.org/10.12860/JNP.2013.24

The first case of IgG4-related disease in Italy. / Rolla, Davide; Bellino, Diego; Peloso, Giancarlo; Rastaldi, Maria Pia; Simonini, Paola; Ravetti, Jean Louis.

In: Journal of Nephropathology, Vol. 2, No. 2, 2013, p. 144-149.

Research output: Contribution to journalArticle

Rolla, D, Bellino, D, Peloso, G, Rastaldi, MP, Simonini, P & Ravetti, JL 2013, 'The first case of IgG4-related disease in Italy', Journal of Nephropathology, vol. 2, no. 2, pp. 144-149. https://doi.org/10.12860/JNP.2013.24
Rolla, Davide ; Bellino, Diego ; Peloso, Giancarlo ; Rastaldi, Maria Pia ; Simonini, Paola ; Ravetti, Jean Louis. / The first case of IgG4-related disease in Italy. In: Journal of Nephropathology. 2013 ; Vol. 2, No. 2. pp. 144-149.
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