Clin J Gastroenterol (2010) 3:243–247 DOI 10.1007/s12328-010-0166-9
CASE REPORT
A case of autoimmune pancreatitis with metachronous appearance of idiopathic thrombocytopenic purpura Hiroaki Sawai • Hiroyuki Matsubayashi Masaki Tanaka • Yuichiro Yamaguchi • Hiroyuki Ono
•
Received: 25 February 2010 / Accepted: 7 July 2010 / Published online: 10 August 2010 Ó Springer 2010
Abstract Autoimmune pancreatitis (AIP) is often associated with systemic disorders, but a case accompanied with idiopathic thrombocytopenic purpura (ITP) is very rare. A 67-year-old man was referred to our institution with complaints of abdominal pain and jaundice. Multiple images showed diffuse enlargement of the pancreas, narrowing of the main pancreatic duct, stenosis of the lower common bile duct and thickness of the anterior wall of the abdominal aorta. Serum levels of IgG and IgG4 were elevated and a diagnosis of AIP was made based on the Japanese criteria. The pancreatic lesion and serum markers responded well to steroid therapy. A year after withdrawal of steroids, the peripheral blood platelet levels gradually decreased to 5.8 9 104/ll. With elevated serum PAIgG levels and exclusion of other causes, we diagnosed ITP. Immediately after restarting steroid therapy, the platelet level in the peripheral blood recovered. Keywords Autoimmune pancreatitis Idiopathic thrombocytopenic purpura IgG4 Steroid Retroperitoneal fibrosis
Introduction Primary inflammatory sclerosis of the pancreas, which may occur with an autoimmune mechanism, was first reported by Sarles et al. [1]. Yoshida et al. [2] proposed the concept of autoimmune pancreatitis (AIP) in 1995. The Japan Pancreas Society then proposed diagnostic criteria for autoimmune pancreatitis in 2002 and revised it in 2006. The diagnostic criteria include: diffuse or segmental narrowing of the main pancreatic duct with enlargement of the pancreas; high levels of serum gammaglobulin (c-globulin), IgG, or IgG4, or the presence of autoantibodies; and lymphoplasmacytic sclerosing pancreatitis (LPSP). A unique feature of AIP is an association with systemic disorders and autoimmune diseases such as sialaodenitis, sclerosing cholangitis and diabetes mellitus [3]. Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder characterized by production of an autoreactive antibody to platelet antigens, resulting in both accelerated destruction of platelets and reduced platelet production. It is considered that the onset of ITP is associated with autoimmune mechanism. So far, only a small number of reports have demonstrated cases of AIP accompanied with ITP.
Case report This case report is ethically approved by institutional review board of Shizuoka Cancer Center. H. Sawai (&) H. Matsubayashi M. Tanaka Y. Yamaguchi H. Ono Division of Endoscopy, Shizuoka Cancer Center, 1007, Shimonagakubo, Nagaizumi, Suntogun, Shizuoka 411-8777, Japan e-mail: [email protected]
A 67-year-old Japanese man was referred to our institution for further examination of a pancreatobiliary disorder, found under endoscopic biliary drainage (EBD) placement in the previous hospital. His physical findings were not remarkable. Laboratory tests showed elevated levels of hepatobiliary enzymes, i.e., AST 99 IU/l (normal range 10–40 IU/l), ALT 322 IU/l (normal range 5–40 IU/l), ALP
123
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Clin J Gastroenterol (2010) 3:243–247
leukocyte, coagulation factor and hepatobiliary enzymes were within normal range, serum PAIgG was elevated to 58.2 ng/107 cells. By excluding other causes, such as drugs, radiation and viral infection, we diagnosed ITP. Helicobacter pylori (H. pylori) was not detected by culture of the gastric mucosa obtained by endoscopic biopsy. Taking his warfarization for atrial fibrillation into the consideration, we restarted steroid therapy (25 mg/day of prednisolone) to prevent hemorrhage. In 4 weeks, the platelet levels in the peripheral blood recovered to within the normal range (16.4 9 104/ll). The levels of serum IgG, IgG4 and platelets are shown in Fig. 4. So far, symptoms and abnormal data suggesting the recurrence of AIP have not been observed for 5 months with a maintenance dose of prednisolone (10 mg/day). Fig. 1 Diffuse enlargement of pancreas was shown by ultrasonography (US)
1214 IU/l (normal range 115–359 IU/l), and c-GTP 847 IU/l (normal range 10–40 IU/l). Serum levels of IgG (normal range 870–1700 mg/dl) and IgG4 (normal range 4.8–105 mg/dl) were elevated at 2156 and 462 mg/dl, respectively. Antinuclear antibody (ANA) and rheumatoid arthritis particle agglutination (RAPA) were positive in the serum by 409 dilution, but other autoantibodies, such as anti-mitochondrial antibody (AMA), Sjo¨gren syndrome-A (SS-A) antibody and Sjo¨gren syndrome-B (SS-B) antibody were negative. Abdominal ultrasonography (US) revealed a sausage-like, diffuse enlargement of the pancreas (Fig. 1). Endoscopic retrograde cholangiopancreatography (ERCP) showed diffuse, irregular narrowing of the main pancreatic duct and stenosis of the lower common bile duct (Fig. 2a). Enhanced computed tomography (CT) showed enlargement of the pancreas and thickness of the anterior wall of the abdominal aorta (Fig. 3a). Pancreatic tissue obtained by endoscopic US-guided fine-needle aspiration biopsy (EUSFNAB) demonstrated histologically the infiltration and fibrosis of lymph-plasma cells. Meeting the Japanese criteria of AIP (2006), we started peroral steroid therapy with 30 mg/day of prednisolone. After 1 month, the serum markers responded well (IgG 1245 mg/dl and IgG4 296 mg/dl), and after 4 months the narrowing of the main pancreatic duct, stenosis of the bile duct and the thickness of the wall of the abdominal aorta all improved (Figs. 2b, 3b). We decreased the dosage to 10 mg/day by 5 mg/day every 2 weeks, and by 2 mg/day every 3 months. Steroid therapy was withdrawn after 16 months. A year after withdrawal of steroid therapy, the peripheral blood platelet levels gradually decreased to 5.8 9 104/ ll (32.6 9 104/ll at the first medical examination) in contrast to the elevation of serum IgG (1620 mg/dl). Although other laboratory data including hemoglobin,
123
Discussion AIP is associated with various autoimmune diseases and is considered to be a subgroup of the systemic IgG4-related diseases. Hamano et al. reported the frequency of extrapancreatic lesions that we often find (hilar lymph node swelling or gallium accumulation 80.4%, sclerosing cholangitis 73.9%, swelling of the lachrymal gland or the salivary gland 39.1%, hypothyroid state 20.2%, retroperitoneal fibrosis 12.5%) [4], and Ohara et al. [3] showed various extrapancreatic lesions that were occasionally reported, such as hypophysitis, liver inflammatory pseudotumor and interstitial nephritis. In our institute, we found the cases of AIP complicated by hypophysitis and liver inflammatory pseudotumor. AIP accompanied by ITP, as in our case, is also thought to be very rare. To date, only seven cases have been reported in the literature, including the cases that were complicated by immune thrombocytopenia [5–11] (Table 1). The relationship between AIP and ITP has not been clarified immunologically and epidemiologically. Interestingly, there is a difference in the epidemiology of ITP between the general population and cases of AIP. The prevalence of ITP is reported to be 5.5–11.5/100000 in the general Japanese population [12] and the number of patients with AIP was about 2800 in 2008. Taking the number of ITP cases with AIP (seven cases) and the number of AIP cases into consideration, prevalence of ITP among AIP is expected to be higher than the prevalence of ITP in the general population. A remarkable trend was also recognized in the gender of these seven cases: they were all men and no women, in contrast to a 1:3 man:woman ratio of ITP in the general population. These findings suggest some association between AIP and ITP. Further analysis is needed to identify molecular mechanisms or immunological factors commonly functioning in these diseases.
Clin J Gastroenterol (2010) 3:243–247
245
Fig. 2 Diffuse, irregular narrowing of the main pancreatic duct and stenosis of the lower common bile duct were shown by endoscopic retrograde cholangiopancreatography (ERCP) before steroid therapy (a). These findings were improved after 4 months of steroid therapy (b)
Fig. 3 Thickness of the anterior wall of the abdominal aorta was shown by enhanced CT before steroid therapy (a). The wall thickness was improved after starting steroid therapy (b)
Fig. 4 The change in the levels of serum IgG and platelets
It was reported that cases of AIP with high levels of serum immunoglobulin frequently accompany other extrapancreatic lesions [3]. A similar trend was observed in the previous cases of AIP associated with ITP (Table 1) and the mean serum levels of IgG and IgG4 at the initial
diagnosis were extremely high [3830 mg/dl (range 2080–6196 mg/dl) and 455 mg/dl (range 337–553 mg/dl)]. We found a tendency for these data to be higher than serum immunoglobulin levels of AIP reported by the Ministry of Health in Japan (median level of IgG 2120 mg/dl and
123
246
Clin J Gastroenterol (2010) 3:243–247
Table 1 Details of cases of AIP complicated by ITP Author (reference)
Year
Age/sex
IgG/IgG4 (mg/dl)
Duration of onset between AIP and ITP
Seko et al. [5]
2000
66/M
6040/ND
14 days
Platelet (per ll)
PAIgG
Initial dose of PSL for AIP
Treatment for ITP
1000
(?)
Steroid pulse therapy
Synchronousa
Nakamura et al. [7]
2003
78/M
6196/ND
6 months
28000
(?)
ND
30 mg/day of PSL
Fukushima et al. [11] Nakazawa et al. [8]
2004 2004
80/M 70/M
3450/553 3730/ND
10 days 1 month
15000 5600
(?) ND
40 mg/day 30 mg/day
Synchronousa Synchronousa
Murase et al. [9]
2008
73/M
3160/468
Same time
10000
(?)
55 mg/day
Synchronousa
Miyatani et al. [10]
2008
64/M
2080/337
4 months
86000
(?)
30 mg/day
H. pylori eradication
Sawai et al.
2010
67/M
2156/462
28 months
58000
(?)
30 mg/day
25 mg/day of PSL
M male synchronous, ND no data in the report, PSL prednisolone a
Treatment of AIP and ITP was started synchronously
median level of IgG4 410 mg/dl) [13]. In such cases, we need to screen for extrapancreatic lesions [14], especially lesions which may cause severe physical dysfunction, such as inflammatory abdominal aneurysms [15], pituitary masses [16] and ureteric obstructions. In enhanced CT and PET, thickness of the anterior wall of the abdominal aorta was found in the current case, associated with retroperitoneal fibrosis. We did not obtain histological evidence as biopsy of the peri-aortic tissue was too invasive and the diagnostic ability of the CT scan was excellent (sensitivity 83.3%, specificity 99.7% and accuracy 93.7%) [17]. Incidence of peri-aortitis was not very rare and two out of 13 cases (15.4%) of AIP in our institution demonstrated this finding [14]. Early recognition is important to prevent severe secondary complications by retroperitoneal fibrosis, such as renal failure due to severe ureteric obstruction and the potentially fatal consequence of aortic rupture [15, 18]. We experienced AIP with the appearance of ITP after withdrawal of steroid therapy. Although rare, clinicians must monitor the number of platelets in peripheral blood and the possible occurrence of ITP in the clinical course of AIP. Acknowledgments The authors are grateful to Miss Mayumi Kishikawa and Mrs. Midori Numano for their assistance during endoscopic procedures.
References 1. Sarles H, Gerolami A. Chronic pancreatitis. Clin Gastroenterol. 1972;1:167–93. 2. Yoshida K, Toki F, Takeuchi T, Watanabe S, Shiratori K, Hayashi N. Chronic pancreatitis caused by an autoimmune abnormality. Dig Dis Sci. 1995;40:1561–8. 3. Ohara H, Nakazawa T, Sano H, Ando T, Okamoto T, Takada H, et al. Systemic extrapancreatic lesions associated with autoimmune pancreatitis. Pancreas. 2005;31:232–7.
123
4. Hamano H, Arakura N, Muraki T, Ozaki Y, Kiyosawa K, Kawa S. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol. 2006;41: 1197–205. 5. Seko S, Taniguti T, Hishikawa H, Azuma K, Asagoe K, Otani T. A case of autoimmune pancreatitis associated with immune thrombocytopenia. Gastroenterol Endosc. 2000;42:192–7. (in Japanese with English abstract). 6. Taniguchi T, Hamasaki A, Okamoto M. A case of suspected lymphocytic hypophysitis and organizing pneumonia during maintenance therapy for autoimmune pancreatitis associated with autoimmune thrombocytopenia. Endocr J. 2006;53:563–6. 7. Nakamura A, Funatomi H, Katagiri A, Katayose K, Kimura K, Seki T, et al. A case of autoimmune pancreatitis complicated with immune thrombocytopenia during maintenance therapy with prednisolone. Dig Dis Sci. 2003;48:1964–71. 8. Nakazawa T, Kobayashi K, Ohara H, Sano H, Ando T, Yamada T, et al. Autoimmune pancreatitis associated with biliary stricture, immune thrombocytopenic purpura and lung fibrosis. Dig Endosc. 2004;16:162–5. 9. Murase K, Matsunaga T, Hayashi T, Ishiwatari H, Araki N, Iyama S, et al. Successful treatment of autoimmune pancreatitis complicated with autoimmune thrombocytopenia and interstitial pneumonia by prednisolone. Intern Med. 2008;47:1033–8. 10. Miyatani H, Yoshida Y, Ikeda M, Sagihara N. Improvement of thrombocytopenia in a patient with autoimmune pancreatitis after Helicobacter pylori eradication. Intern Med. 2008;47:321–2. doi: 10.2169/internalmedicine.47.0399. 11. Fukushima H, Katou E, Nagayama K, Shirachi A, Sata M. A case of pancreatitis complicated with immune thrombocytopenia. J Gastroenterol. 2006;103:661–6. (in Japanese with English abstract). 12. Omine M. Epidemiology and long-term prognosis of ITP. Nippon Rinsho. 2003;61(4):655–63. (in Japanese with English abstract). 13. Kawa S, Hamano H. Serological markers for the diagnosis of autoimmune pancreatitis. Pancreas. 2007;22:641–5. (in Japanese with English abstract). 14. Matsubayashi H, Furukawa H, Maeda A, Matsunaga K, Kanemoto H, Uesaka K, et al. Usefulness of positron emission tomography in the evaluation of distribution and activity of systemic lesions associated with autoimmune pancreatitis. Pancreatology. 2009;9(5):694–9. 15. Jois RN, Gaffney K,Marshall T, Scott. DGI. Chronic periaortitis. Rheumatology. 2004;43:1441–6. 16. Shimatsu A, Oki Y, Fujisawa I, Sano T. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin
Clin J Gastroenterol (2010) 3:243–247 G4-related systemic disease: an emerging clinical entity. Endocr J. 2009;56(9):1033–41. 17. Iino M, Kuribayashi S, Imakita S, Takamiya M, Matsuo H, Ookita Y, et al. Sensitivity and specificity of CT in the diagnosis
247 of inflammatory abdominal aortic aneurysms. J Comput Assist Tomogr. 2002;26(6):1006–12. 18. Hellman DB, Grand DJ, Freischlag JA. Inflammatory abdominal aortic aneurysm. JAMA. 2007;297:395–400.
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CASE REPORT
A case of autoimmune pancreatitis with metachronous appearance of idiopathic thrombocytopenic purpura Hiroaki Sawai • Hiroyuki Matsubayashi Masaki Tanaka • Yuichiro Yamaguchi • Hiroyuki Ono
•
Received: 25 February 2010 / Accepted: 7 July 2010 / Published online: 10 August 2010 Ó Springer 2010
Abstract Autoimmune pancreatitis (AIP) is often associated with systemic disorders, but a case accompanied with idiopathic thrombocytopenic purpura (ITP) is very rare. A 67-year-old man was referred to our institution with complaints of abdominal pain and jaundice. Multiple images showed diffuse enlargement of the pancreas, narrowing of the main pancreatic duct, stenosis of the lower common bile duct and thickness of the anterior wall of the abdominal aorta. Serum levels of IgG and IgG4 were elevated and a diagnosis of AIP was made based on the Japanese criteria. The pancreatic lesion and serum markers responded well to steroid therapy. A year after withdrawal of steroids, the peripheral blood platelet levels gradually decreased to 5.8 9 104/ll. With elevated serum PAIgG levels and exclusion of other causes, we diagnosed ITP. Immediately after restarting steroid therapy, the platelet level in the peripheral blood recovered. Keywords Autoimmune pancreatitis Idiopathic thrombocytopenic purpura IgG4 Steroid Retroperitoneal fibrosis
Introduction Primary inflammatory sclerosis of the pancreas, which may occur with an autoimmune mechanism, was first reported by Sarles et al. [1]. Yoshida et al. [2] proposed the concept of autoimmune pancreatitis (AIP) in 1995. The Japan Pancreas Society then proposed diagnostic criteria for autoimmune pancreatitis in 2002 and revised it in 2006. The diagnostic criteria include: diffuse or segmental narrowing of the main pancreatic duct with enlargement of the pancreas; high levels of serum gammaglobulin (c-globulin), IgG, or IgG4, or the presence of autoantibodies; and lymphoplasmacytic sclerosing pancreatitis (LPSP). A unique feature of AIP is an association with systemic disorders and autoimmune diseases such as sialaodenitis, sclerosing cholangitis and diabetes mellitus [3]. Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder characterized by production of an autoreactive antibody to platelet antigens, resulting in both accelerated destruction of platelets and reduced platelet production. It is considered that the onset of ITP is associated with autoimmune mechanism. So far, only a small number of reports have demonstrated cases of AIP accompanied with ITP.
Case report This case report is ethically approved by institutional review board of Shizuoka Cancer Center. H. Sawai (&) H. Matsubayashi M. Tanaka Y. Yamaguchi H. Ono Division of Endoscopy, Shizuoka Cancer Center, 1007, Shimonagakubo, Nagaizumi, Suntogun, Shizuoka 411-8777, Japan e-mail: [email protected]
A 67-year-old Japanese man was referred to our institution for further examination of a pancreatobiliary disorder, found under endoscopic biliary drainage (EBD) placement in the previous hospital. His physical findings were not remarkable. Laboratory tests showed elevated levels of hepatobiliary enzymes, i.e., AST 99 IU/l (normal range 10–40 IU/l), ALT 322 IU/l (normal range 5–40 IU/l), ALP
123
244
Clin J Gastroenterol (2010) 3:243–247
leukocyte, coagulation factor and hepatobiliary enzymes were within normal range, serum PAIgG was elevated to 58.2 ng/107 cells. By excluding other causes, such as drugs, radiation and viral infection, we diagnosed ITP. Helicobacter pylori (H. pylori) was not detected by culture of the gastric mucosa obtained by endoscopic biopsy. Taking his warfarization for atrial fibrillation into the consideration, we restarted steroid therapy (25 mg/day of prednisolone) to prevent hemorrhage. In 4 weeks, the platelet levels in the peripheral blood recovered to within the normal range (16.4 9 104/ll). The levels of serum IgG, IgG4 and platelets are shown in Fig. 4. So far, symptoms and abnormal data suggesting the recurrence of AIP have not been observed for 5 months with a maintenance dose of prednisolone (10 mg/day). Fig. 1 Diffuse enlargement of pancreas was shown by ultrasonography (US)
1214 IU/l (normal range 115–359 IU/l), and c-GTP 847 IU/l (normal range 10–40 IU/l). Serum levels of IgG (normal range 870–1700 mg/dl) and IgG4 (normal range 4.8–105 mg/dl) were elevated at 2156 and 462 mg/dl, respectively. Antinuclear antibody (ANA) and rheumatoid arthritis particle agglutination (RAPA) were positive in the serum by 409 dilution, but other autoantibodies, such as anti-mitochondrial antibody (AMA), Sjo¨gren syndrome-A (SS-A) antibody and Sjo¨gren syndrome-B (SS-B) antibody were negative. Abdominal ultrasonography (US) revealed a sausage-like, diffuse enlargement of the pancreas (Fig. 1). Endoscopic retrograde cholangiopancreatography (ERCP) showed diffuse, irregular narrowing of the main pancreatic duct and stenosis of the lower common bile duct (Fig. 2a). Enhanced computed tomography (CT) showed enlargement of the pancreas and thickness of the anterior wall of the abdominal aorta (Fig. 3a). Pancreatic tissue obtained by endoscopic US-guided fine-needle aspiration biopsy (EUSFNAB) demonstrated histologically the infiltration and fibrosis of lymph-plasma cells. Meeting the Japanese criteria of AIP (2006), we started peroral steroid therapy with 30 mg/day of prednisolone. After 1 month, the serum markers responded well (IgG 1245 mg/dl and IgG4 296 mg/dl), and after 4 months the narrowing of the main pancreatic duct, stenosis of the bile duct and the thickness of the wall of the abdominal aorta all improved (Figs. 2b, 3b). We decreased the dosage to 10 mg/day by 5 mg/day every 2 weeks, and by 2 mg/day every 3 months. Steroid therapy was withdrawn after 16 months. A year after withdrawal of steroid therapy, the peripheral blood platelet levels gradually decreased to 5.8 9 104/ ll (32.6 9 104/ll at the first medical examination) in contrast to the elevation of serum IgG (1620 mg/dl). Although other laboratory data including hemoglobin,
123
Discussion AIP is associated with various autoimmune diseases and is considered to be a subgroup of the systemic IgG4-related diseases. Hamano et al. reported the frequency of extrapancreatic lesions that we often find (hilar lymph node swelling or gallium accumulation 80.4%, sclerosing cholangitis 73.9%, swelling of the lachrymal gland or the salivary gland 39.1%, hypothyroid state 20.2%, retroperitoneal fibrosis 12.5%) [4], and Ohara et al. [3] showed various extrapancreatic lesions that were occasionally reported, such as hypophysitis, liver inflammatory pseudotumor and interstitial nephritis. In our institute, we found the cases of AIP complicated by hypophysitis and liver inflammatory pseudotumor. AIP accompanied by ITP, as in our case, is also thought to be very rare. To date, only seven cases have been reported in the literature, including the cases that were complicated by immune thrombocytopenia [5–11] (Table 1). The relationship between AIP and ITP has not been clarified immunologically and epidemiologically. Interestingly, there is a difference in the epidemiology of ITP between the general population and cases of AIP. The prevalence of ITP is reported to be 5.5–11.5/100000 in the general Japanese population [12] and the number of patients with AIP was about 2800 in 2008. Taking the number of ITP cases with AIP (seven cases) and the number of AIP cases into consideration, prevalence of ITP among AIP is expected to be higher than the prevalence of ITP in the general population. A remarkable trend was also recognized in the gender of these seven cases: they were all men and no women, in contrast to a 1:3 man:woman ratio of ITP in the general population. These findings suggest some association between AIP and ITP. Further analysis is needed to identify molecular mechanisms or immunological factors commonly functioning in these diseases.
Clin J Gastroenterol (2010) 3:243–247
245
Fig. 2 Diffuse, irregular narrowing of the main pancreatic duct and stenosis of the lower common bile duct were shown by endoscopic retrograde cholangiopancreatography (ERCP) before steroid therapy (a). These findings were improved after 4 months of steroid therapy (b)
Fig. 3 Thickness of the anterior wall of the abdominal aorta was shown by enhanced CT before steroid therapy (a). The wall thickness was improved after starting steroid therapy (b)
Fig. 4 The change in the levels of serum IgG and platelets
It was reported that cases of AIP with high levels of serum immunoglobulin frequently accompany other extrapancreatic lesions [3]. A similar trend was observed in the previous cases of AIP associated with ITP (Table 1) and the mean serum levels of IgG and IgG4 at the initial
diagnosis were extremely high [3830 mg/dl (range 2080–6196 mg/dl) and 455 mg/dl (range 337–553 mg/dl)]. We found a tendency for these data to be higher than serum immunoglobulin levels of AIP reported by the Ministry of Health in Japan (median level of IgG 2120 mg/dl and
123
246
Clin J Gastroenterol (2010) 3:243–247
Table 1 Details of cases of AIP complicated by ITP Author (reference)
Year
Age/sex
IgG/IgG4 (mg/dl)
Duration of onset between AIP and ITP
Seko et al. [5]
2000
66/M
6040/ND
14 days
Platelet (per ll)
PAIgG
Initial dose of PSL for AIP
Treatment for ITP
1000
(?)
Steroid pulse therapy
Synchronousa
Nakamura et al. [7]
2003
78/M
6196/ND
6 months
28000
(?)
ND
30 mg/day of PSL
Fukushima et al. [11] Nakazawa et al. [8]
2004 2004
80/M 70/M
3450/553 3730/ND
10 days 1 month
15000 5600
(?) ND
40 mg/day 30 mg/day
Synchronousa Synchronousa
Murase et al. [9]
2008
73/M
3160/468
Same time
10000
(?)
55 mg/day
Synchronousa
Miyatani et al. [10]
2008
64/M
2080/337
4 months
86000
(?)
30 mg/day
H. pylori eradication
Sawai et al.
2010
67/M
2156/462
28 months
58000
(?)
30 mg/day
25 mg/day of PSL
M male synchronous, ND no data in the report, PSL prednisolone a
Treatment of AIP and ITP was started synchronously
median level of IgG4 410 mg/dl) [13]. In such cases, we need to screen for extrapancreatic lesions [14], especially lesions which may cause severe physical dysfunction, such as inflammatory abdominal aneurysms [15], pituitary masses [16] and ureteric obstructions. In enhanced CT and PET, thickness of the anterior wall of the abdominal aorta was found in the current case, associated with retroperitoneal fibrosis. We did not obtain histological evidence as biopsy of the peri-aortic tissue was too invasive and the diagnostic ability of the CT scan was excellent (sensitivity 83.3%, specificity 99.7% and accuracy 93.7%) [17]. Incidence of peri-aortitis was not very rare and two out of 13 cases (15.4%) of AIP in our institution demonstrated this finding [14]. Early recognition is important to prevent severe secondary complications by retroperitoneal fibrosis, such as renal failure due to severe ureteric obstruction and the potentially fatal consequence of aortic rupture [15, 18]. We experienced AIP with the appearance of ITP after withdrawal of steroid therapy. Although rare, clinicians must monitor the number of platelets in peripheral blood and the possible occurrence of ITP in the clinical course of AIP. Acknowledgments The authors are grateful to Miss Mayumi Kishikawa and Mrs. Midori Numano for their assistance during endoscopic procedures.
References 1. Sarles H, Gerolami A. Chronic pancreatitis. Clin Gastroenterol. 1972;1:167–93. 2. Yoshida K, Toki F, Takeuchi T, Watanabe S, Shiratori K, Hayashi N. Chronic pancreatitis caused by an autoimmune abnormality. Dig Dis Sci. 1995;40:1561–8. 3. Ohara H, Nakazawa T, Sano H, Ando T, Okamoto T, Takada H, et al. Systemic extrapancreatic lesions associated with autoimmune pancreatitis. Pancreas. 2005;31:232–7.
123
4. Hamano H, Arakura N, Muraki T, Ozaki Y, Kiyosawa K, Kawa S. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol. 2006;41: 1197–205. 5. Seko S, Taniguti T, Hishikawa H, Azuma K, Asagoe K, Otani T. A case of autoimmune pancreatitis associated with immune thrombocytopenia. Gastroenterol Endosc. 2000;42:192–7. (in Japanese with English abstract). 6. Taniguchi T, Hamasaki A, Okamoto M. A case of suspected lymphocytic hypophysitis and organizing pneumonia during maintenance therapy for autoimmune pancreatitis associated with autoimmune thrombocytopenia. Endocr J. 2006;53:563–6. 7. Nakamura A, Funatomi H, Katagiri A, Katayose K, Kimura K, Seki T, et al. A case of autoimmune pancreatitis complicated with immune thrombocytopenia during maintenance therapy with prednisolone. Dig Dis Sci. 2003;48:1964–71. 8. Nakazawa T, Kobayashi K, Ohara H, Sano H, Ando T, Yamada T, et al. Autoimmune pancreatitis associated with biliary stricture, immune thrombocytopenic purpura and lung fibrosis. Dig Endosc. 2004;16:162–5. 9. Murase K, Matsunaga T, Hayashi T, Ishiwatari H, Araki N, Iyama S, et al. Successful treatment of autoimmune pancreatitis complicated with autoimmune thrombocytopenia and interstitial pneumonia by prednisolone. Intern Med. 2008;47:1033–8. 10. Miyatani H, Yoshida Y, Ikeda M, Sagihara N. Improvement of thrombocytopenia in a patient with autoimmune pancreatitis after Helicobacter pylori eradication. Intern Med. 2008;47:321–2. doi: 10.2169/internalmedicine.47.0399. 11. Fukushima H, Katou E, Nagayama K, Shirachi A, Sata M. A case of pancreatitis complicated with immune thrombocytopenia. J Gastroenterol. 2006;103:661–6. (in Japanese with English abstract). 12. Omine M. Epidemiology and long-term prognosis of ITP. Nippon Rinsho. 2003;61(4):655–63. (in Japanese with English abstract). 13. Kawa S, Hamano H. Serological markers for the diagnosis of autoimmune pancreatitis. Pancreas. 2007;22:641–5. (in Japanese with English abstract). 14. Matsubayashi H, Furukawa H, Maeda A, Matsunaga K, Kanemoto H, Uesaka K, et al. Usefulness of positron emission tomography in the evaluation of distribution and activity of systemic lesions associated with autoimmune pancreatitis. Pancreatology. 2009;9(5):694–9. 15. Jois RN, Gaffney K,Marshall T, Scott. DGI. Chronic periaortitis. Rheumatology. 2004;43:1441–6. 16. Shimatsu A, Oki Y, Fujisawa I, Sano T. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin
Clin J Gastroenterol (2010) 3:243–247 G4-related systemic disease: an emerging clinical entity. Endocr J. 2009;56(9):1033–41. 17. Iino M, Kuribayashi S, Imakita S, Takamiya M, Matsuo H, Ookita Y, et al. Sensitivity and specificity of CT in the diagnosis
247 of inflammatory abdominal aortic aneurysms. J Comput Assist Tomogr. 2002;26(6):1006–12. 18. Hellman DB, Grand DJ, Freischlag JA. Inflammatory abdominal aortic aneurysm. JAMA. 2007;297:395–400.
123
