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DOI:10.2214/AJR.05.5149
AJR 2005; 185:1367-1368
© American Roentgen Ray Society

Lymphoplasmacytic Sclerosing Pancreatitis

Darren Brennan and Ivan Pedrosa

Beth Israel Deaconess Medical Center Boston, MA

We read with interest the article by Kawamoto et al. [1] titled "Lymphoplasmacytic Sclerosing Pancreatitis with Obstructive Jaundice: CT and Pathology Features" [1] in the October 2004 issue of the AJR and compliment the authors on an excellent description of a rare but important condition. We report two biopsyproven cases of lymphoplasmacytic sclerosing pancreatitis in patients who recently attended our institution, in whom we incidentally detected some interesting renal findings.

The first patient was a 67-year-old woman who presented with obstructive jaundice for which a stent had been placed at ERCP in an outside hospital. Her history was significant for rheumatoid arthritis and sarcoidosis. Triphasic contrast-enhanced CT was performed in our institution for staging of a presumptive adenocarcinoma of the head of the pancreas. CT showed a diffusely enlarged pancreas without evidence of pancreatic duct dilatation and without a focal mass. A halo-like area of hypoattenuation surrounding the pancreas was also seen, similar to that described in the study by Kawamoto et al. [1]. The patient had an elevated erythrocyte sedimentation rate, a weakly positive rheumatoid factor, and a positive antinuclear factor. Her total IgG was elevated, but her IgG4 was within the normal limits. Renal function was normal.

On the basis of imaging findings, a diagnosis of autoimmune pancreatitis was suggested and the patient underwent multiple laparoscopic sonographically guided transgastric biopsies, which confirmed the diagnosis of lymphoplasmacytic sclerosing pancreatitis after an initial nondiagnostic CT-guided fine needle aspirate. The patient was treated medically with steroids and remains well.

On the original staging CT images, we noticed evidence of focal peripheral wedge-shaped areas of hypoattenuation in both kidneys on the corticomedullary images (Fig. 1) that became mostly isodense relative to the rest of the cortex on the nephrographic and venous phase images (not shown).



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Fig. 1 67-year-old woman with lymphoplasmacytic sclerosing pancreatitis. Axial CT image during corticomedullary phase shows bilateral foci of hypoattenuation within renal cortex (arrows). Enlarged head of pancreas (arrowhead) is also visualized.

 
The second patient was a 37-year-old man who presented with multiple systemic symptoms as well as two episodes of acute pancreatitis. In the second case, a different pattern of renal findings was observed. CT showed large bilateral hypoenhancing renal masses and enlargement of the proximal two thirds of the pancreas. Renal and pancreatic biopsies were performed. The pancreatic biopsy findings were consistent with lymphoplasmacytic sclerosing pancreatitis. The renal biopsy results showed an intense monocytic infiltrate, but no malignant cells were seen. The patient was placed on steroids, which resulted in dramatic normalization of pancreatic imaging findings, although the renal lesions remained mostly unchanged.

In the images from the article [1], we notice areas of hypoattenuation within the left (Fig. 3A from the article [1]) and right (Fig. 3C from the article [1]) kidneys that are similar to those we encountered in our two cases, and we wonder if the authors could comment whether these finding are similar to those of our patients.

As pointed out by the authors [1], lymphoplasmacytic sclerosing pancreatitis is characterized pathologically to be a peripancreatic lymphoplasmacytic reaction and a distinctive venulitis, and all pathologic series of lymphoplasmacytic sclerosing pancreatitis report the presence of a distinct venulitis. Lymphoplasmacytic sclerosing pancreatitis has been described in association with a number of extrapancreatic conditions [2, 3] and autoimmune phenomena [4].

The findings we describe in the first patient are reported in vasculitis due to other causes including lupus, polyarteritis nodosa, and drug-induced vasculitides [5]. In a recent review of autoimmune pancreatitis, Sahani et al. [6] also described a case of lymphoplasmacytic sclerosing pancreatitis in a patient who had renal "pseudotumors," but those authors did not indicate whether a biopsy had been performed. Our second patient had bilateral renal pseudotumors caused by monocytic infiltrates.

It is unclear if the renal findings in patients with lymphoplasmacytic sclerosing pancreatitis represent true pseudotumors caused by infiltration of the renal parenchyma similar to that in the pancreatic gland or if these findings are the renal manifestation of the diffuse venulitis that characterizes this disease. If the described renal findings are secondary to vasculitis, it would lend support to the hypothesis that lymphoplasmacytic sclerosing pancreatitis is a local manifestation of a systemic disease process. The presence of bilateral wedge-shaped areas of decreased cortical enhancement or hypoenhancing masses in the kidneys could support the diagnosis of lymphoplasmacytic sclerosing pancreatitis in patients with diffuse pancreatic abnormalities in whom this diagnosis is contemplated.


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References
 

  1. Kawamoto S, Siegelman SS, Hruban RH, Fishman EK. Lymphoplasmacytic sclerosing pancreatitis with obstructive jaundice: CT and pathology features. AJR 2004; 183:915 -921[Abstract/Free Full Text]
  2. Toosi MN, Heathcote J. Pancreatic pseudotumor with sclerosing pancreato-cholangitis: is this a systemic disease? Am J Gastroenterol 2004; 99:377 -382[CrossRef][Medline]
  3. Notohara K, Burgart LJ, Yadav D, Chari S, Smyrk TC. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol 2003; 27:1119 -1127[CrossRef][Medline]
  4. Hardacre JM, Iacobuzio-Donahue CA, Sohn TA, et al. Results of pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis. Ann Surg 2003;237 : 853-858[CrossRef][Medline]
  5. Kawashima A, Sandler CM, Ernst RD, Tamm EP, Goldman SM, Fishman EK. CT evaluation of renovascular disease. RadioGraphics2000; 20:1321 -1340[Abstract/Free Full Text]
  6. Sahani DV, Kalva SP, Farrell J, et al. Autoimmune pancreatitis: imaging features. Radiology 2004;233 : 345-352[Abstract/Free Full Text]

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S. Kawamoto, S. S. Siegelman, R. H. Hruban, and E. K. Fishman
Lymphoplasmacytic Sclerosing Pancreatitis (Autoimmune Pancreatitis): Evaluation with Multidetector CT
RadioGraphics, January 1, 2008; 28(1): 157 - 170.
[Abstract] [Full Text] [PDF]


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RadiologyHome page
N. Takahashi, A. Kawashima, J. G. Fletcher, and S. T. Chari
Renal Involvement in Patients with Autoimmune Pancreatitis: CT and MR Imaging Findings
Radiology, March 1, 2007; 242(3): 791 - 801.
[Abstract] [Full Text] [PDF]


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