Wakabayashi Tokio, Kawaura Yukimitsu, Satomura Yoshitake, Fujii Tomoharu, Motoo Yoshiharu, Okai Takashi, Sawabu Norio
Department of Gastroenterology, Saiseikai Kanazawa Hospital, Kanazawa, Japan.
Pancreas. 2002 Oct;25(3):283-9. doi: 10.1097/00006676-200210000-00011.
Main pancreatic duct (MPD)-narrowed chronic pancreatitis (CP) may be an autoimmune abnormality. It also has been called autoimmune pancreatitis and sclerosing pancreatitis. It is unclear whether cases with focal pancreatographic changes are part of the same clinical entity as cases with diffuse MPD changes.
We reviewed seven cases of chronic pancreatitis (CP) with focal narrowing of the main pancreatic duct (MPD), evidenced by endoscopic retrograde cholangiopancreatography (ERCP), and swelling of one or two segments of the pancreas, evidenced by ultrasonography (US) /computed tomography (CT), and indicated the clinicopathologic features of focal-type MPD-narrowed CP.
The patient group comprised six men and one woman, and their age range was 28-75 years, with a mean of 63.7 years. Affected sites were in the head in two patients, the body in one patient, the tail in one patient, and the body and tail in three patients; ERP showed narrowing in six patients and obstruction in one. Stricture of the lower portion of the common bile duct (CBD) that caused obstructive jaundice was shown by ERC in two cases in which the pancreas head was affected. In all six patients, a dynamic study by CT or MRI homogeneously showed delayed enhancement of involved segments of the pancreas. Serum levels of pancreatic enzyme were elevated in five patients, but only one subject had pancreatitis-like epigastric pain. Serological evidence suggestive of autoimmune abnormality was detected in only three patients with hypergammaglobulinemia (> or =2.0 g/dL) or positive titers of antinuclear antibody (ANA; > or =80). Histological assessment was available for five patients, who characteristically had dense lymphocytic or plasmocytic infiltration with severe fibrosis that caused luminal narrowing. The clinical, serologic, and histologic findings as described above were comparable to those for 12 CP patients with diffuse narrowing of the MPD, diagnosed during the same period. Surgical resection was performed in 5 patients, in 2 of whom a similar inflammatory process recurred in the remnant head of the pancreas, whereas pancreatitis no longer developed in the other 3 patients. One patient was initially treated with steroids, with clinical remission, although there was neither hypergammaglobulinemia nor positive ANA.
These results indicate that CP with focal narrowing of the MPD is part of the same clinical spectrum as CP with diffuse narrowing of the MPD, and whether the distribution is diffuse or focal seems to be related to the stage or the extent of the disease. It is therefore important to recognize the possible existence of this focal variant to avoid unnecessary surgery.
主胰管（MPD）狭窄的慢性胰腺炎（CP）可能是一种自身免疫异常。它也被称为自身免疫性胰腺炎和硬化性胰腺炎。尚不清楚局灶性胰管造影改变的病例是否与弥漫性MPD改变的病例属于同一临床实体。
我们回顾了7例经内镜逆行胰胆管造影（ERCP）证实为主胰管（MPD）局灶性狭窄、超声（US）/计算机断层扫描（CT）证实为胰腺一或两个节段肿胀的慢性胰腺炎（CP）病例，并指出了局灶型MPD狭窄性CP的临床病理特征。
患者组包括6名男性和1名女性，年龄范围为28 - 75岁，平均63.7岁。受累部位：2例在胰头，1例在胰体，1例在胰尾，3例在胰体和胰尾；ERCP显示6例狭窄，1例梗阻。在2例胰头受累的病例中，ERC显示胆总管（CBD）下段狭窄导致梗阻性黄疸。在所有6例患者中，CT或MRI动态研究均显示胰腺受累节段均匀延迟强化。5例患者血清胰酶水平升高，但只有1例有胰腺炎样上腹部疼痛。仅在3例高球蛋白血症（≥2.0 g/dL）或抗核抗体（ANA）滴度阳性（≥80）的患者中检测到提示自身免疫异常的血清学证据。5例患者有组织学评估，其特征为密集的淋巴细胞或浆细胞浸润伴严重纤维化导致管腔狭窄。上述临床、血清学和组织学表现与同期诊断的12例MPD弥漫性狭窄的CP患者相似。5例患者接受了手术切除，其中2例胰腺残端头部再次出现类似炎症过程，而其他3例患者未再发生胰腺炎。1例患者最初接受类固醇治疗，临床缓解，尽管既无高球蛋白血症也无ANA阳性。
这些结果表明，MPD局灶性狭窄的CP与MPD弥漫性狭窄性CP属于同一临床谱，其分布是弥漫性还是局灶性似乎与疾病的阶段或范围有关。因此，认识到这种局灶性变异的可能存在以避免不必要的手术很重要。
