Lymphoplasmacytic sclerosing pancreatitis without IgG4 tissue infiltration or serum IgG4 elevation

IgG4-related disease without IgG4

Phil A. Hart, Thomas Christopher Smyrk, Suresh T Chari

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Type 1 autoimmune pancreatitis can be diagnosed by and is synonymous with its pathognomonic histopathologic appearance called lymphoplasmacytic sclerosing pancreatitis. Type 1 autoimmune pancreatitis, also called IgG4-related pancreatitis, is the pancreatic manifestation of IgG4-related disease. However, the role of IgG4 in the pathogenesis of IgG4-related disease is unclear. We describe patients with LPSP without serum or tissue IgG4 abnormalities. From the Mayo Clinic database of autoimmune pancreatitis patients, we identified three patients with histologically confirmed type 1 autoimmune pancreatitis (lymphoplasmacytic sclerosing pancreatitis) who had normal serum IgG4 and no increase in IgG4-positive plasma cells in tissue. We reviewed original clinical records and pathologic specimens, and describe the clinical and histologic features of these three patients. All patients (age/gender: 63/F, 70/M and 68/M) had normal serum IgG and IgG4 levels, and multiple sections of pancreatic histology did not show increased IgG4-positive plasma cells. Two patients were diagnosed retrospectively following pancreatic surgery, one relapsed in another organ and one has remained relapse free. Another patient was diagnosed by pancreatic core biopsy and has suffered multiple relapses that have been controlled by rituximab. These cases highlight the fact that although the currently agreed upon name for type 1 autoimmune pancreatitis is IgG4-related pancreatitis, serum and tissue IgG4 abnormalities are best considered characteristic, but not essential for the diagnosis of this enigmatic condition.

Original languageEnglish (US)
Pages (from-to)238-247
Number of pages10
JournalModern Pathology
Volume28
Issue number2
DOIs
StatePublished - Feb 2 2015

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Pancreatitis
Immunoglobulin G
Serum
Plasma Cells
Recurrence
Names
Histology
Databases
Biopsy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Lymphoplasmacytic sclerosing pancreatitis without IgG4 tissue infiltration or serum IgG4 elevation : IgG4-related disease without IgG4. / Hart, Phil A.; Smyrk, Thomas Christopher; Chari, Suresh T.

In: Modern Pathology, Vol. 28, No. 2, 02.02.2015, p. 238-247.

Research output: Contribution to journalArticle

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