AJR 2003; 181:993-995
© American Roentgen Ray Society
Autoimmune Pancreatitis Associated with Idiopathic Retroperitoneal Fibrosis
Yoshihiko Fukukura1,
Fumito Fujiyoshi,
Fumihiko Nakamura,
Hiroyuki Hamada and
Masayuki Nakajo
1 All authors: Department of Radiology, Faculty of Medicine, Kagoshima
University, 8-35-1 Sakuragaoka, Kagoshima City 890-8520, Japan.
Received December 2, 2002;
accepted after revision February 4, 2003.
Address correspondence to Y. Fukukura.
Introduction
Autoimmune pancreatitis is a recently described entity characterized by an
absence of acute attacks of pancreatitis, no history of alcohol abuse,
increased serum 
-globulin or IgG levels, presence of autoantibodies,
irregular narrowing of the pancreatic duct, diffuse enlargement of the
pancreas, good clinical response to steroid therapy, and occasional
association with other autoimmune diseases
[1]. To our knowledge, only a
few cases of autoimmune pancreatitis with idiopathic retroperitoneal fibrosis
have been reported in the literature
[2]. We present the radiologic
findings of one such case. Recognizing the existence of this rare disorder may
help in correctly diagnosing similar cases.
Case Report
A 68-year-old man was admitted to our hospital for investigation of general
fatigue. No history of drug ingestion or abdominal surgery was given.
Laboratory test results revealed liver dysfunction (aspartate
aminotransferase, 79 U/L [normal, 14–40 U/L]; alanine aminotransferase,
58 U/L [normal, 10–48 U/L]; -glutamyl transpeptidase, 123 U/L
[normal, 11–83 U/L]), hyperglobulinemia (-globulin level, 34.7%
[normal, 9–20%]), elevated IgG level (3055 mg/dL [normal,
1000–1900 mg/dL]) and antinuclear antibody positive at a titer of
1:320.
Sonography revealed a diffusely enlarged and hypoechoic pancreas.
Unenhanced CT also showed a diffusely enlarged pancreas; contrast-enhanced CT
showed a low-density rim surrounding the pancreas
(Fig. 1A) during arterial and
portal venous phases and delayed enhancement of the pancreatic parenchyma
during a delayed phase. CT also showed bilateral perirenal and perihilar
soft-tissue masses separate from the enlarged pancreas, surrounding both
ureters but not obstructing their lumina
(Fig. 1B). These masses
appeared similar in enhancement to the enlarged pancreas on contrast-enhanced
CT.
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Fig. 1B. —68-year-old man with autoimmune pancreatitis and idiopathic
retroperitoneal fibrosis. Contrast-enhanced CT scan of section inferior to
that imaged in A shows bilateral peripelvic lesions (straight
arrows) and left perirenal lesions (curved arrows).
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MRI revealed an enlarged pancreas, hypointense relative to the hepatic
parenchyma on T1-weighted fast low-angle shot images (TR/TE, 117.3/4.1) and
hyperintense with a hypointense rim on T2-weighted half-Fourier acquisition
single-shot turbo spin-echo (HASTE) images (TR/effective TE, infinite/59)
(Fig. 1C). Bilateral perirenal
and perihilar masses were slightly hyperintense on T1-weighted fast low-angle
shot images (TR/TE, 117.3/4.1) and isointense relative to the renal medulla on
T2-weighted HASTE images (TR/effective TE, infinite/59)
(Fig. 1D). Endoscopic
retrograde cholangiopancreatography revealed smooth narrowing of the entire
pancreatic duct with segmental stenosis of the common bile duct at the head of
the pancreas. Because these clinical and radiologic findings suggested
autoimmune pancreatitis associated with idiopathic retroperitoneal fibrosis,
corticosteroid treatment (prednisolone, 40 mg/day) was initiated.
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Fig. 1C. —68-year-old man with autoimmune pancreatitis and idiopathic
retroperitoneal fibrosis. T2-weighted HASTE MRI (TR/effective TE, infinite/59)
shows slightly hyperintense pancreas with low-intensity rim
(arrows).
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Fig. 1D. —68-year-old man with autoimmune pancreatitis and idiopathic
retroperitoneal fibrosis. T2-weighted MRI (infinite/59) shows bilateral
peripelvic masses (straight arrows) and left perirenal masses
(curved arrows) as isointense relative to renal medullas.
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After 2 years of treatment, the antinuclear antibody titer had decreased to
1:80 and liver function had normalized. Follow-up CT and MRI of the abdomen at
this point showed a marked reduction in the size of both the pancreas and
perirenal and perihilar masses (Figs.
1E and
1F).
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Fig. 1E. —68-year-old man with autoimmune pancreatitis and idiopathic
retroperitoneal fibrosis. Contrast-enhanced CT scan obtained after 2 years of
systemic steroid therapy shows no evidence of pancreatic enlargement.
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Fig. 1F. —68-year-old man with autoimmune pancreatitis and idiopathic
retroperitoneal fibrosis. Contrast-enhanced CT scan of section inferior to
that imaged in E shows marked reduction in size of bilateral peripelvic
and left perirenal masses after steroid treatment.
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Discussion
Chronic pancreatitis of autoimmune origin is a relatively new and
increasingly reported clinicopathologic entity, variously referred to as
autoimmune pancreatitis [1,
3], sclerosing pancreatitis
[2], and nonalcoholic
duct-destructive chronic pancreatitis
[4]. Autoimmune pancreatitis
has the following characteristics: an absence of acute attacks of
pancreatitis, no history of alcohol abuse, increased serum -globulin or
IgG levels, presence of autoantibodies, irregular narrowing of the pancreatic
duct, diffuse enlargement of the pancreas, good clinical response to steroid
therapy, and occasional association with other autoimmune diseases such as
primary sclerosing cholangitis, chronic idiopathic inflammatory bowel disease,
Sjögren's syndrome, and systemic lupus erythematosus
[1,
3,
4]. These features make the
diagnosis of autoimmune pancreatitis relatively straightforward, as it was in
this patient.
In contrast, idiopathic retroperitoneal fibrosis is an uncommon entity of
obscure origin usually confined to the retroperitoneal space and centered
between the renal hila and the pelvic brim
[5]. On rare occasions, this
condition has been reported in patients with immunologic disease or as a
complication of multifocal fibrosclerosis, a rare syndrome characterized by
fibrosis involving multiple organ systems
[6]. Multifocal fibrosclerosis
may manifest as idiopathic retroperitoneal fibrosis, mediastinal fibrosis,
orbital pseudotumor, sclerosing cholangitis, chronic fibrosing thyroiditis,
pelvic fibrosis [6,
7], or pancreatic fibrosis
[7].
In the present case there appeared to be autoimmune pancreatitis and
bilateral perirenal and perihilar lesions consistent with idiopathic
retroperitoneal fibrosis, although the retroperitoneal lesions did not appear
radiographically to be typical for idiopathic retroperitoneal fibrosis.
Contiguous extension of retroperitoneal fibrosis or sclerosing mesenteritis
into the pancreas has been well documented
[8]. However, fibrosis
extending into the pancreas from the bilateral perirenal and perihilar areas
was thought unlikely in our patient because imaging studies did not show the
affected areas to be contiguous.
Idiopathic retroperitoneal fibrosis with non-contiguous pancreatic fibrosis
seems to be rare; to our knowledge, only a few cases have appeared in the
literature. Hamano et al. [2],
who recently reported three patients with clinical findings similar to those
seen in this case, proposed that autoimmune pancreatitis was associated with
retroperitoneal fibrosis and could be complicated by multifocal
fibrosclerosis. Therefore, we concluded that an autoimmune process affected
both the pancreas and the perirenal and perihilar areas in our patient.
Although CT and MRI are effective techniques for the evaluation of
idiopathic retroperitoneal fibrosis, their findings do not have diagnostic
specificity under these conditions. Similar imaging findings could be produced
by many retroperitoneal disease processes, such as malignant lymphoma,
sarcoma, or carcinoma of retroperitoneal organs. Nevertheless, these imaging
findings may be interpreted in the light of the clinical, biochemical, and
immunologic data as suggesting a diagnosis of an autoimmunerelated
retroperitoneal fibrosis.
In conclusion, this article presents an unusual case of autoimmune
pancreatitis associated with idiopathic retroperitoneal fibrosis. This rare
disorder should be promptly recognized so that inappropriate surgical
resection may be avoided and immunosuppressive therapy initiated to optimize
prognosis.
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Chronic pancreatitis caused by an autoimmune abnormality: proposal of the
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Introduction
Case Report
