Autoimmune Pancreatitis

Generoso Uomo
Department of Internal Medicine, “Cardarelli” Hospital. Naples, Italy
Autoimmune pancreatitis is a relatively uncommon,
non-alcohol-related form of chronic pancreatitis which
has received increasing attention in recent years. In
fact, autoimmune pancreatitis represents a “hot topic”
in pancreatology, as witnessed by the many reports and
extensive recently published reviews [1, 2, 3, 4, 5, 6].
The first report of the disease which dates back to 1961
documented patients who suffered from chronic
inflammatory sclerosis of the pancreas associated with
hypergammaglobulinemia [7], even if the possibility of
an autoimmune mechanism in the pathogenesis of
chronic pancreatitis had been already stressed in 1959
[8]. After three decades of single case reports of only
limited series of patients with non-alcoholic chronic
pancreatitis predominantly associated with chronic
inflammatory bowel diseases or sclerosing cholangitis,
in 1991, Kawaguchi et al. [9] gave the first detailed
description of this special type of pancreatitis and
called it “lymphoplasmacytic sclerosing pancreatitis”.
In 1995, Yoshida et al., reporting a relatively large
series from Japan, coined the term autoimmune
pancreatitis [10]. Autoimmune pancreatitis is currently
recognized as a distinct clinical entity worldwide,
identified as a chronic inflammatory condition of the
pancreas in which an autoimmune mechanism is
involved [6, 11]. High serum IgG4 concentrations have
been identified as its distinguishing characteristic and
as a marker of autoimmunity as well as of disease
activity [12]. The presence of concomitant disorders in
other organs together with autoimmune pancreatitis
was mainly intended as the proof that the spectrum
disease of the pancreas was comparable to that of the
bile ducts, liver, colon or salivary glands [13].
Recently, Kamisawa et al. [14] have proposed a novel
clinicopathological entity, named “IgG4-related
sclerosing disease” (IgG4-RSC) which was based on
the histological and immunohistochemical examination
of various organs of patients affected by autoimmune
pancreatitis. This systemic disease may present
different clinical manifestations related to the frequent
involvement not only of the pancreas (autoimmune
pancreatitis), but also of the bile duct (sclerosing
cholangitis, cholecystitis), the salivary gland
(sialoadenitis), the retroperitoneum (fibrosis), the
kidney (tubulointerstitial nephritis), the lung
(interstitial pneumonia), the prostate (prostatitis) and
the lymphnodes (lymphadenopathy). Extensive IgG4-
positive plasma-cells and T-lymphocyte infiltration of
various organs constitutes a common characteristics of
this disease. Most of the affected patients present
autoimmune pancreatitis at the moment of diagnosis,
but pancreatic involvement can be absent [15]; in
general, more than two organs are affected, but it is
possible to find it in only one of them. The disease
occurs more frequently in older men and responds well
to corticosteroid treatment. Beginning with the analysis
of Japanese patients with IgG4-related disorders,
Masaoki et al. [16] have very recently proposed an
extension of the IgG4-RSC syndromic complex into a
new clinical entity characterized by hyper-IgG4
gammaglobulinemia and IgG4-positive plasma-cell
infiltration into the tissue, this being considered an
expression of a lymphoproliferative disease (“IgG4-
positive multiorgan lymphoproliferative syndrome”;
IgG4+MOLPS). In addition to the pathologies
considered within the IgG4-RSC group, this syndrome
also includes Mikulicz’s disease, Küttner’s tumors,
inflammatory pseudotumors (of the lung, liver, and
breast), mediastinal fibrosis and autoimmune
hypophysitis. IgG4+MOLPS should be differentiated
from Sjögren’s syndrome, which presents a similar
organ distribution but different clinical-
epidemiological-histopathological features and a
different autoantibody pattern. Other rare conditions
such as multicentric Castelman’s disease and idiopathic
plasmacytic lymphoadenopathy should also be
considered in differential diagnosis.
Key words
Autoimmune Diseases; Biological Markers;
Diagnosis, Differential; Epidemiology
IgG4+MOLPS: IgG4-positive multiorgan
sclerosing disease
Correspondence Generoso Uomo
Department of Internal Medicine, “Cardarelli” Hospital, via
Cardarelli 9, 80131 Napoli, Italy
Phone: +39-081.747.2101; Fax: +39-081.747.2117
Document URL

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JOP. J Pancreas (Online) 2010 Mar 5; 11(2):191-192.
JOP. Journal of the Pancreas - - Vol. 11, No. 2 - March 2010. [ISSN 1590-8577
These recent papers, based on solid epidemiological,
clinical and histopathological features, raise strongly
doubts as to autoimmune pancreatitis being considered
an autonomous entity and support its classification as a
single entity within a multiorgan syndromic complex.
At the moment, this working hypothesis may be
considered well-grounded only in Eastern populations;
studies from Western countries are necessary to
confirm this fascinating scenario.
Conflict of interest The author has no potential
conflicts of interest
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