Nefrite tubulo-interstiziale ipocomplementemica in IgG4-related disease.

Translated title of the contribution: [Hypocomplementemic tubulointerstitial nephritis in IgG4-related disease].

Davide Rolla, Diego Bellino, Giada Pistoni, Gian Carlo Peloso, Maria Pia Rastaldi, Paola Simonini, Jean Louis Ravetti, Giuseppe Cannella

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

A novel lymphoproliferative disorder producing plasma cell expansion in the affected organ with fibrotic or sclerosing changes, known as ''IgG4-related disease'', was defined in Japan by Umehara's group in 2010. We present the first case reported in Italy. In 2007, a 63-year-old man presented with epigastric pain and elevated serum lipase levels. Computed tomography of the abdomen revealed a Kuttner's tumor of the pancreas. The patient underwent a biliary-enteric anastomosis, and biopsy of the pancreas revealed massive infiltration of lymphocytes and plasma cells. The patient was diagnosed with chronic sclerosing pancreatitis. After one year, he began to show signs of sicca syndrome and at the same time developed progressive renal failure. Immunological tests revealed hypocomplementemia, and the renal biopsy specimen showed diffuse interstitial inflammation. The infiltrate was composed of lymphocytes, while infiltrating plasma cells showed immunoreactivity to IgG-4. Sialography using a radioisotope revealed severe involvement of the salivary glands, and Schirmer's test gave a positive result. This led us to diagnose hypocomplementemic tubulointerstitial nephritis in IgG4-related disease. Corticosteroid treatment resulted in rapid improvement including disappearance of the sicca syndrome and progressive amelioration of renal function. After six months, we discontinued steroid administration and started mycophenolate mofetil to maintain a low degree of immunosuppression. Follow-up after two years showed that this therapy continued to be quite effective in our patient.

Original languageItalian
Pages (from-to)348-352
Number of pages5
JournalGiornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia
Volume29
Issue number3
Publication statusPublished - May 2012

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Interstitial Nephritis
Plasma Cells
Immunoglobulin G
Sjogren's Syndrome
Pancreas
Sialography
Lymphocytes
Mycophenolic Acid
Kidney
Biopsy
Immunologic Tests
Lymphoproliferative Disorders
Chronic Pancreatitis
Salivary Glands
Lipase
Radioisotopes
Abdomen
Immunosuppression
Italy
Renal Insufficiency

ASJC Scopus subject areas

  • Nephrology

Cite this

Nefrite tubulo-interstiziale ipocomplementemica in IgG4-related disease. / Rolla, Davide; Bellino, Diego; Pistoni, Giada; Peloso, Gian Carlo; Rastaldi, Maria Pia; Simonini, Paola; Ravetti, Jean Louis; Cannella, Giuseppe.

In: Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia, Vol. 29, No. 3, 05.2012, p. 348-352.

Research output: Contribution to journalArticle

Rolla, D, Bellino, D, Pistoni, G, Peloso, GC, Rastaldi, MP, Simonini, P, Ravetti, JL & Cannella, G 2012, 'Nefrite tubulo-interstiziale ipocomplementemica in IgG4-related disease.', Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia, vol. 29, no. 3, pp. 348-352.
Rolla, Davide ; Bellino, Diego ; Pistoni, Giada ; Peloso, Gian Carlo ; Rastaldi, Maria Pia ; Simonini, Paola ; Ravetti, Jean Louis ; Cannella, Giuseppe. / Nefrite tubulo-interstiziale ipocomplementemica in IgG4-related disease. In: Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia. 2012 ; Vol. 29, No. 3. pp. 348-352.
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