Steriod for Autoimmune pancreatitis complicating by gastric varices: A case report

lNTRODUCTlON

Autoimmune pancreatitis (AIP) is accepted as a unique clinical entity worldwide. The prevalence rate of AIP in Japan was 2.2 per 100000 people[1]. Using this prevalence, it was estimated that the number of AIP patients in China would be approximately 29 thousand[2]. A large multicenter study from 10 different countries reported that therapy with steroid could significantly induce clinical remission in 99% of type 1 AIP and 92% of type 2 AIP patients[3]. However, few studies have reported the role of steroid therapy in AIP complicated by gastric varices. In this study, we summarize the effects of steroid therapy in a case of AIP complicated by gastric varices.

No special previous medical history was reported.

CASE PRESENTATlON

Chief complaints

Based on these findings, autoimmune pancreatitis complicated by gastric varices was identified.

History of present illness

Type 1 AIP is considered a pancreatic manifestation of IgG4 related disease, which also includes a few extra-pancreatic organ diseases such as salivary sclerosingsialadenitis, interstitial nephritis, sclerosing cholangitis lung nodules, mediastinal fibrosis, adenopathy and chronic periaortitis[5,6]. The classical histopathological findings are dense lymphoplasmacytic infiltration, IgG4-positive plasma cell infiltration, storiform fibrosis, obliterative phlebitis and fibrosis without granulocytic infiltration[7]. A large multicenter study found that Type 1 AIP often presents at an older age and is more likely than type 2 AIP to manifest in men[3]. A systematic review in China found that the male to female ratio of type 1 AIP was 4.5:1. The main symptoms in type 1 AIP patients are jaundice (75%), abdominal pain (65%), and weight loss (42%)[2]. In this case report, the patient was a woman, and the main clinical presentation was up abdominal pain and weight loss.

History of past illness

習近平總書記認為“深厚的感情必須以深刻的認識做基礎”[注]習近平：《知之深愛之切》，石家莊：河北人民出版社，2015 年，(代序)第1頁。，愛國主義也是如此。愛國主義建立在深刻認識自己國家的基礎上，因此，踐行愛國主義必須要“樹立和堅持正確的歷史觀、民族觀、國家觀、文化觀”[注]中共中央宣傳部：《習近平總書記系列重要講話讀本(2016年版)》，北京：學習出版社，2016年，第203頁。。只有深刻認識祖國的悠久歷史和深厚文化，才會不斷增強中華民族的歸屬感、認同感、尊嚴感、榮譽感，不斷增強做中國人的骨氣和底氣。

Personal and family history

No special personal or family histories were found.

Physical examination

Epigastric tenderness without rebound or guarding was found. No other positive signs were found.

Laboratory examinations

Routine blood tests showed a hemoglobin (Hb) of 81 g/L (N:113-151 g/L), red blood cell of 2.88 × 10/L(N: 3.68-5.13 × 10/L). Other tests, such as white blood cell, platelet and neutrophil were all normal.Serum protein electrophoresis showed an increased incidence of polyclonal α1-globulinemia of 4.7% (N:1.1-3.7%), α2-globulinemia of 15.2% (N: 8.5-14.5%) and γ-globulinemia of 26.1% (N: 9.2–18.2%). Other blood chemistry parameters such as tumor markers, liver and kidney function, coagulation tests and amylase levels were all within normal limits. Serology for hepatitis B and C and autoimmune serology,including anti-neutrophil cytoplasmic antibody and antinuclear antibody were all negative. The serum level of IgG4 was detected and was significantly elevated at 3670 mg/L (N: 80-1400 mg/L).

Imaging examinations

Abdominal computed tomography (CT) revealed a diffusely enlarged pancreas with a capsule-like rim,an obstructed splenic vein, slight splenomegaly and collateral vessels from the gastric fundus(Figure 1A). Magnetic resonance cholangiopancreatography showed the irregular expansion of pancreatic duct in the neck and body of pancreas, and the swelling of pancreas (Figure 1B). Esophagogastroduodenoscopy (EGD) revealed that gastric varices in the partial gastric fundus and the red-color sign were positive (Figure 1C).

FlNAL DlAGNOSlS

A 59-year-old Chinese woman was admitted to the hospital with the symptoms of mild abdominal discontinuous pain for approximately 4 mo.

日本內閣2018年7月3日批準第五份基礎能源規劃，設定了到2030年的電力結構發展目標。根據規劃，核電仍將是一種重要能源，核發電量到2030年將占全國總發電量的20%～22%。

TREATMENT

通過前文的分析，本文得到的結論是人民幣升值會降低企業出口價格，企業垂直專業化水平上升擴大了出口價格下降的幅度，即企業出口價格的匯率彈性隨著垂直專業化水平的提高而增加。當企業按成本加成定價法確定出口價格時，出口價格由加成率和邊際成本兩部分組成，匯率變動對企業出口價格的影響取決于匯率變動對企業加成率和邊際成本的影響。理論模型部分在分析匯率變動對出口價格的影響以及垂直專業化對出口價格匯率彈性的調節作用時也主要從加成率和邊際成本的角度切入，為了檢驗這一影響機制，我們從實證角度考察匯率變動對企業加成率和邊際成本的影響以及垂直專業化對加成率匯率彈性和邊際成本匯率彈性的影響。

OUTCOME AND FOLLOW-UP

Two months after the initial treatment, an abdominal CT showed that the swelling in the pancreas had improved (Figure 2A). The obstructed splenic vein, splenomegaly and gastric varices with positive redcolor signs also showed a slight improvement (Figure 2B). The serum IgG4 had decreased to 1370 mg/L.Five months after the initial treatment, abdominal CT showed that the swelling of the pancreas, the obstructed splenic vein and splenomegaly had further improved (Figure 3A). EGD also showed that the gastric varices had improved, and the red-color signs were negative (Figure 3B). The serum IgG4 decreased to a normal level at 677mg/L. One year after the initial therapy, an abdominal CT showed that the pancreas, splenic vein and spleen were restored to the normal level (Figure 4A). EGD showed that the gastric vertices had disappeared (Figure 4B). The serum IgG4 had decreased to its normal level of 566 mg/L. Finally, the patient was asked to stop steroid therapy. After three years of follow-up, the patient did not experience a recurrent abdominal pain, and she did not have melena.

DlSCUSSlON

The patient suffered from symptoms of mild abdominal discontinuous pain for approximately four months, accompanied by abdominal distension and poor appetite. The patient’s intake was decreased by almost 1/2 compared with normal, and induced a weight loss of 5 kg. In the last month, the symptoms of abdominal pain and distension were worse than before, accompanied by fatigue, but without nausea and vomiting.

Gastric varices are a relatively common complication in chronic pancreatitis[8]. Chronic pancreatitis leads to splenic vein obstruction and induces sinistral portal hypertension. Finally, gastric varices are formed along the fundus and the greater curvature of the stomach, since the blood flow in the short gastric veins or the gastroepiploic vein is significantly increased. However, AIP complicated by gastric varices has rarely been reported. Ishikawa[9] retrospectively reviewed 54 patients who met the international disease diagnostic criteria for AIP and found that 24 of the patients had AIP complicated by peripancreatic vascular involvement. Among these, only one patient (1/54, 1.9%) had gastric varices,22 patients (22/54, 40.7%) had splenic vein occlusion or stenosis, and 13 patients (13/54, 24.1%) had superior mesenteric-portal vein occlusion or stenosis. In this case, the patient was diagnosed with AIP complicated by splenic vein obstruction and gastric varices.

柯式模型能夠全方位對培訓進行有效的評估，能夠很好的反映醫學專碩研究生參與住院醫師規范化培訓的效果。科學有效地評估對于醫學院校充分了解培訓投入的效果、培訓對醫學院校發展的積極作用以及專碩研究生能力提升等方面有著重要的作用和意義。

All study participants, or their legal guardian, provided informed written consent prior to study enrollment.

CONCLUSlON

In summary, we reported a case of AIP complicated by gastric varices in this study. After 1 year of steroid therapy, the patient did not have any symptoms. The pancreas was significantly improved, and the gastric varices disappeared.

Oral administration of 24 mg/d methylprednisolone was started for two weeks. Then the treatment was tapered by 4 mg every two weeks and until a maintenance dose of 8 mg/d was reached.

FOOTNOTES

Hao NB and Li CZ designed the research study; Hao NB, Li Χ, Hu WW and Zhang D performed the research; Li Χ, Χie J and Wang ΧL contributed new reagents and analytic tools; Hao NB and Li CZ analyzed the data and wrote the manuscript; all authors have read and approve the final manuscript.

Steroids are considered a risk factor for gastrointestinal bleeding. More studies are needed to evaluate the merits and disadvantages of using steroid therapy for AIP complicated by peripancreatic vascular involvement, especially gastric varices[10]. Ishikawa and colleagues reported that vascular lesions were significantly improved in 14 of 16 patients who received steroid therapy for AIP complicated by peripancreatic vascular involvement[9]. In contrast, the vascular lesions were exacerbated in 2 of 4 patients who were not treated with steroid therapy after two years. These results indicated that steroid therapy was meaningful. Furthermore, Goto and colleagues reported 3 cases of AIP complicated by gastric varices[11]. In case 1 without splenomegaly, steroid therapy significantly improved the enlarged pancreas and the splenic vein was reperfused. Finally, the gastric varices disappeared after six months of therapy. However, in cases 2 and 3 with splenomegaly, steroid therapy was not very effective. One case showed that steroid therapy only improved AIP but had no influence on gastric varices. In another case, it was found that steroid therapy had no role in either AIP or gastric varices after five months of therapy. These cases revealed that the reason for these patients’ congestive splenomegaly might have been the long duration for which they were affected by sinistral portal hypertension, and the splenomegaly finally led to irreversible spleen vein obstruction[11,12]. In this case, at the time of diagnosis, the patient had a diffusely enlarged pancreas with a capsule-like rim, an obstructed splenic vein, slight splenomegaly and gastric varices. After 1 year of steroid therapy, the capsule-like rim and gastric varices had disappeared, the spleen vein was reperfused, and the spleen was reduced.

The authors declare that they have no conflict of interest.

林兆華導表演藝術與話劇“中國學派”的探索發展 ……………………………………………… 張 赟（1．73）

The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).

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China

Ning-Bo Hao 0000-0002-9315-8813; Χue Li 0000-0002-3298-7036; Wen-Wei Hu 0000-0001-8368-6199; Dan Zhang 0000-0001-9248-6195; Jing Χie 0000-0001-5650-8901; Χiao-Lin Wang 0000-0002-8074-3175; Chang-Zheng Li 0000-0001-6994-573Χ.

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格雷在他1970年出版的《國際旅行國際貿易》一書中，提出了休閑旅行（即旅游）的兩種驅動力：一種是內在的“推動”因素（push factor）、一種是存在于異地的“拉動”因素（pull factor）［1］106－107。按照他的解釋，“推動”因素是人類的一種內在的本質特征，它催發著人們離開熟悉的環境和事物而前往異域他鄉去觀賞令人激動的異域文化和事物的欲望②。

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