# Lymphoplasmacytic Sclerosing Pancreatitis and Retroperitoneal Fibrosis

Authors: Nigel K. F. Koo Ng; Jin J. Bong; Robin C. Williamson
Journal: HPB Surgery
 (2008-02-12)

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## Abstract

Although cases of lymphoplasmacytic sclerosing pancreatitis (LSP) associated with idiopathic retroperitoneal fibrosis have been reported, the association is rare. We describe a 74-year-old man who presented with obstructive jaundice and weight loss. Nineteen months earlier, he had been diagnosed with idiopathic retroperitoneal fibrosis and treated with bilateral ureteric stents.  Initial investigations were suggestive of a diagnosis of LSP, however, a malignant cause could not be ruled out. He underwent an exploratory laparotomy and frozen sections confirmed the diagnosis of LSP. An internal biliary bypass was performed using a Roux loop of jejunum, and the patient made an uneventful recovery. This case illustrates the difficulty in distinguishing LSP from pancreatic carcinoma preoperatively.


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## Body

## 1. Introduction

Lymphoplasmacytic sclerosing
pancreatitis (LSP) or autoimmune pancreatitis is a rare condition characterised
by diffuse fibroinflammatory infiltrates that can involve both the pancreatic
ducts and acinar parenchyma. It is also known as primary inflammatory sclerosis
of the pancreas, sclerosing pancreatitis, autoimmune sclerosing pancreatitis,
duct destructive chronic pancreatitis, and sclerosing pancreatico-cholangitis
[1, 2].Since the condition was first
described in 1961 by Sarles et al., several cases have been reported, and in
recent years, LSP has become increasingly recognised as an important and unique
cause of chronic pancreatitis. Of particular note, it presents with a
pancreatic mass that can be extremely difficult to differentiate from
pancreatic carcinoma (which it may resemble both clinically and radiologically).
It has been reported that “benign but clinically suspicious”
Whipple resections are relatively common in high-volume centers (9.2%) and that
LSP represents 23.4% of Whipple resections performed for benign disease [3]. The occasional association of
LSP with other autoimmune conditions is well documented [4], including
Sjogren's syndrome, primary sclerosing cholangitis, inflammatory bowel disease,
and systemic lupus erythematosus.
Although cases of LSP associated with idiopathic retroperitoneal
fibrosis have also been reported, the association is rare. To our knowledge,
not more than 14 cases have been reported in the literature [4–11].


## 2. Case Report

A 74-year-old Afro-Caribbean man
presented with a 4-week history of obstructive jaundice, weight loss, and
fatigue. He had a background of noninsulin-dependent diabetes and
hypertension.  Nineteen months earlier,
symptoms of polyuria and nocturia had led to a diagnosis of retroperitoneal
fibrosis: renal function was impaired, and both kidneys were poorly perfused on
CT scan, with evidence of retroperitoneal fibrosis obstructing both ureters.  Subsequently, bilateral ureteric stents were
inserted.Since then, the patient had been
well until recently developing jaundice. USS showed extrahepatic and
intrahepatic biliary dilatation but no gallstones. The pancreas was bulky
throughout, although no focal lesion was identified.  ERCP showed an irregular stricture in the
lower CBD suggestive of malignancy (although the biliary brushings later showed
no malignant cells), and an endobiliary stent was inserted. Segments of the
pancreatic duct were also dilated. CT confirmed that the pancreas was markedly
bulky with no focal mass lesion or abnormal calcifications. There was
thickening of soft tissue around the porta hepatis suggestive of
lymphadenopathy. Although the main concern was pancreatic carcinoma, LSP was
also considered to be a possible diagnosis. An autoimmune screen was negative
for ANA, cytoplasmic antibodies (Hep-2), ANCA IIF, mitochondrial antibodies,
smooth muscle antibodies, liver/kidney microsomal antibodies, Anti-GPC,
rheumatoid factor, and striated muscle antibodies. The IgG level was raised at
27.0 g/L (5.3–16.5 g/L),
particularly IgG4 at 15.10 g/L (0.0–1.3 g/L).  Although, there have been reports of an association
with a raised IgG4 level and LSP [12], the diagnosis in this case was still
unclear. To confirm the diagnosis, an exploratory laparotomy was considered to
be most appropriate (and preferable to EUS FNA) as it offered the opportunity
to obtain multiple biopsies from a diffusely enlarged pancreas, whilst also offering
a therapeutic option.At laparotomy, the entire pancreas
was hard, swollen, and actively inflamed, most particularly the body of the
gland. The appearances were not typical for pancreatic cancer or chronic
pancreatitis. A shave biopsy of a nodule on the body of pancreas plus three
core biopsies with a Trucut needle and a fourth from the head of pancreas were
submitted to frozen-section histopathology examination; all showed florid
chronic inflammation but no cancer. A portion of thickened retroperitoneum
tissue in front of the aorta was also removed and this was shown to be benign
fibrotic tissue. Since the pathology was consistent with a diagnosis of LSP, an
internal biliary bypass was performed using a Roux loop of jejunum. The patient
made a good postoperative recovery and was referred to gastroenterology for
further management. Steroids were given postoperatively and he was discharged
nine days later.


## 3. Discussion

LSP primarily affects middle-aged
men, with a mean age of 56 years [13]. There is a male predominance of
approximately 2 : 1 [13]. The recently increasing number of articles on LSP is an
indication of the current interest in this subject. Its similarity to
pancreatic carcinoma, both clinically and radiologically, presents a diagnostic
challenge. In distinguishing LSP from pancreatic carcinoma, there are certain suggestive
features (see Table 1) [14–16]. The Japanese Pancreas Society proposed
diagnostic criteria for LSP in 2002 (see Table 2)
[14, 17].Table 1
Diagnostic features suggestive of
lymphoplasmacytic sclerosing pancreatitis.
Features suggestive of LSP(1)Minimal or only mild symptoms, usually without episodes of acute pancreatitis(2)Diffusely irregular narrowing of the main pancreatic duct and occasional stenosis of the
intrapancreatic bile duct on ERCP images(3)Diffuse enlargement of the pancreas(4)Increased levels of serum gamma globulin or IgG(5)Presence of autoantibodies(6)Fibrotic changes with lymphocyte infiltration in the pancreas(7)Occasional association with other autoimmune conditions(8)Rare pancreatic calcification or cysts(9)Clinical response to steroidsTable 2
To make a diagnosis of LSP, criteriaa must be present, together
with criteria b and/or c.
Diagnostic criteria for LSP(a)Pancreatic imaging studies showing diffuse
narrowing of the main pancreatic duct with an irregular wall (more than 1/3 of
the length of the entire pancreas) with enlargement of the pancreas(b)Abnormally elevated levels of serum gamma
globulin and/or IgG, or the presence of autoantibodies(c)Histopathological examination of the pancreas
shows fibrotic changes with lymphocyte and plasma cell infiltrationPatients with LSP also have elevated
serum IgG4 levels which return to normal after administration of oral steroids.
Only a limited number of conditions are associated with elevated IgG4 (such as
atopic dermatitis, some parasitic diseases, pemphigus vulgaris, and pemphigus
foliaceus). Hamano and associates found high serum IgG4 concentrations
specifically in patients with LSP and not in those with “ordinary” chronic
pancreatitis, primary biliary cirrhosis, primary sclerosing cholangitis, or
Sjogren’s syndrome [12].  Not only does
this finding suggest that LSP is immunologically different, but it may also
follow that IgG4 would be a useful marker [12].The association of LSP with other
autoimmune conditions, including retroperitoneal fibrosis, is well recognised. Our
patient developed retroperitoneal fibrosis prior to LSP, but review of the 14
previous cases indicates that the onset of LSP is variable. Further,
hydronephrosis secondary to ureteric obstruction (as in our case) was not
uncommon [4–11]. The aetiology of retroperitoneal fibrosis is unclear, but the
presence of autoantibodies and the response to steroids in some patients may
suggest an autoimmune mechanism. Infiltration with IgG4-positive plasma cells
has been shown in both the pancreas and the retroperitoneal mass of patients
with LSP and retroperitoneal fibrosis [5], indicating a likely common
aetiology.LSP can be treated with medical
therapy (steroids in reducing dosage) or surgically. The prognosis is usually
good with either form of management but an important factor is the difficulty
in diagnosing LSP, which can so often mimic a malignant intra-abdominal mass. Preoperative
or even intraoperative diagnosis can be problematic, particularly in patients
for whom there are no clinical grounds to suspect LSP. It has been suggested
that surgical resection may be an effective treatment for LSP especially when
immunological tests are normal or when malignancy is highly suspected (this may
be the only option to exclude malignancy) [18]. However, surrounding
inflammation can make surgical resection hazardous, so a trial of steroids is
worthwhile if a confident diagnosis can be made without laparotomy [18].



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