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Response to steroid therapy of sclerosing cholangitis after duodenopancreatectomy due to autoimmune pancreatitis
  1. D Padilla,
  2. T Cubo,
  3. P Villarejo,
  4. R Pardo,
  5. A Jara,
  6. R de la Plaza,
  7. J Hernández
  1. Department of Surgery, Complejo Hospitalario, Ciudad Real, Spain
  1. Correspondence to:
    Dr D Padilla
    Department of Surgery, Complejo Hospitalario, Ciudad Real, Avd Pio XII, s/n13001, Ciudad Real, Spain; maynonaterra.es

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Autoimmune pancreatitis is a chronic inflammation of the pancreas due to aetiopathogenic mechanisms of autoimmunity. There are no established definitive diagnostic criteria although histological, analytical, and radiological characteristics enable us to identify this entity in the differential diagnosis with chronic alcoholic pancreatitis and cancer of the pancreas.1–3 Nevertheless, this is not always possible, and the patient undergoes surgery with suspected cancer of the pancreas. Lymphoplasmacytic infiltration and the autoimmune response do not only affect the pancreas but can occasionally involve the retropancreatic and extrapancreatic biliary system. The relationship between the appearance of sclerosing cholangitis in patients with pancreatic pseudotumour due to autoimmune pancreatitis has even been considered the result of a systemic fibroinflammatory response.1,4 We present the exceptional case of a patient who, after a cephalic duodenopancreatectomy due to pancreatic pseudotumour, in lymphoplasmacytic pancreatitis, presented with a clinical-radiological picture of post-surgical sclerosing cholangitis, which resolved after therapy with steroids. In common with Kamisawa and colleagues,4 we consider autoimmune pancreatitis a lesion more as part of a condition with multifocal fibrosclerosis and we believe that this sclerosing cholangitis is an additional manifestation of an autoimmune systemic condition, possibly stimulated by surgery.

A 78 year old male patient was admitted to our service for obstructive jaundice of a few days’ history, not accompanied by constitutional syndrome. The patient had undergone surgery 75 days previously, with a preoperative radiological diagnosis of suspected cancer of the head of the pancreas. A radical pylorus preserving cephalic duodenopancreatectomy was performed. The patient was discharged 12 days after operation. The histopathology report of the resected sample revealed the presence of intense fibrosis and inflammatory, lymphoplasmacytic infiltration of the biliary wall with no evidence of malignancy. Similarly, the pancreatic gland presented with intense inflammatory, lymphoplasmacytic, glandular atrophy, and no signs of malignancy. Biochemical work up on admission revealed: BbT 16.2 mg/dl; BbD 12.2 mg/dl; GGT 1264 IU/l; ALP 831 IU/l; CEA 2.81 ng/ml; CA 19.9 >500 IU/ml; anti-IgM (HAV) (−); HBsAg (−); HBcAc (−); anti-HCV (−); IgG 1520 mg/dl; IgA 445 mg/dl; IgG4 28 mg/dl; and IgM: 206 mg/dl. Abdominal echography showed dilation of the intrahepatic biliary tract. Magnetic cholangioresonance revealed moderate dilation of the complete intrahepatic tract with no visualisation of the principal biliary tract or hilar plate, and no anastomotic complications. Transparietohepatic cholangiography demonstrated dilation of the right intraheptic biliary tract and diffuse stenosis affecting the common hepatic duct, hepatic hilum, and segmented biliary branches. External-internal percutaneous drainage of the biliary tract was performed using radiology.

After two days there was no obvious sign of improvement and the biochemical work up was as follows: BbT 19.6 mg/dl; BbD 16.5 mg/dl; GGT 673 IU/l; ALP 648 IU/l; GOT 104 IU/l; and GPT 111 IU/l. Exploratory laparotomy was performed with no pathological findings which justified cholestasis. Intraoperative echography showed only enlargement of the biliary wall with no intraluminal obstructive findings. Immediately after surgery, because of suspicion of a basic inflammatory condition, treatment was begun with methylprednisolone 1 mg/kg/24 h intravenously. Once intake was tolerated, this treatment was maintained for a month orally before being reduced to 10 mg orally/24 h during the second month. The analytical follow up was excellent, with BbT reduced to 1.8 mg/dl, and the remaining biological parameters were normal. Similarly, the episode of bicipital tenosynovitis of the left shoulder evolved satisfactorily. The patient maintained treatment with methylprednisolone, 10 mg orally for a further two months, with clinical-radiological and analytical resolution of the cholestatic process (fig 1).

Figure 1

 (A) Transparietohepatic cholangiograph showing the existence of diffuse stenosis of the principal biliary tract and hepatic hilum. (B) Magnetic cholangioresonance which shows absence of uptake in the principal hepatic ducts, hepatic hilum, and principal biliary tract. (C, D) Transparietohepatic cholangiograph after steroid therapy with radiological improvement of the principal biliary tract, hepatic hilum, and principal hepatic ducts.

What is exceptional about this patient is the triggering of a severe autoimmune inflammatory response in the biliary system based on the presence of lymphoplasmacytic infiltration, coexistence with other autoimmune processes (episode of tenosynovitis in the shoulder of our patient), and good response to steroids that would reveal an autoimmune aetiopathogenesis. Our group would include the possibility of exclusive biliary tract involvement, as was the case with our patient, after the stress of surgery. Taniguchi and colleagues5 reported relapse of autoimmune pancreatitis after cephalic duodenopancreatectomy although they do not refer to alterations in the biliary tract. Toosi and colleagues6 reported the appearance in two of their patients of post-surgical sclerosing cholangitis although only after biopsy of the pancreatic head. The appearance of sclerosing cholangitis after duodenopancreatectomy has not been reported previously. The short period of biliary involvement and the progression maintained in the biliary involvement led us to suspect an inflammatory process similar to that of autoimmune pancreatitis.

Neither therapy nor its duration have been well defined, and this can be seen in the different regimens used both for autoimmune pancreatitis and autoimmune pancreatocholangitis. Erkelens and colleagues7 used prednisone 0.5–1 mg/kg/day, followed by maintenance doses for six months. Some patients also received, albeit exceptionally, azathioprine at 50 mg/day, and this was used temporarily until resolution of the biliary endoprosthesis process. The results were satisfactory, although no therapeutic protocol has been defined. This disparity in criteria is manifested in other studies, such as that of Toosi and colleagues6 who used ursodeoxycholic acid at 750 mg/24 h with almost complete return to a normal clinical and analytical picture. Other authors, such as Kojima and colleagues,8 maintained treatment according to the clinical-radiological changes, using a loading dose of 40 mg/24 h, with maintenance doses of 5 mg/24 h. Taniguchi and colleagues5 used prednisolone at 30 mg/24 h for one month, followed by 5 mg/24 h for nine months with satisfactory evolution. Kamisawa and colleagues,9 on the other hand, used a loading dose of prednisolone of 30–40 mg/24 h and maintenance doses of 5 mg/24 h until clinical check-up. Based on the hypothesis of an excessive fibrosclerotic inflammatory response in our patient, we started therapy with prednisolone 1 mg/kg for four weeks, with progressive reduction to 10 mg/24 h over the following four weeks. The maintenance dose was continued for a further two months, with analytical, radiological, and clinical resolution of the process.

References

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Footnotes

  • Conflict of interest: None declared.

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