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一位 50 岁的胰腺炎患者,表现为胰腺炎、脂膜炎和多发性关节炎综合征——病例报告及文献复习。

A 50-year-old patient with pancreatitis revealing pancreatitis, panniculitis, and polyarthritis syndrome-a case report and review of the literature.

机构信息

2nd Medical Department, Division of Rheumatology, Korneuburg-Stockerau Hospital, Lower Austrian Centre for Rheumatology, Landstraße 18, 2000, Stockerau, Austria.

Department for Radiology, Korneuburg-Stockerau Hospital, Stockerau, Austria.

出版信息

Wien Med Wochenschr. 2024 Nov;174(15-16):350-354. doi: 10.1007/s10354-024-01055-7. Epub 2024 Sep 25.

DOI:10.1007/s10354-024-01055-7
PMID:39320395
Abstract

The combination of pancreatitis, panniculitis, and polyarthritis (PPP) is a rare systemic syndrome that occurs in patients with acute or chronic pancreatitis or pancreatic malignancies. A 50-year-old male patient presented with polyarthritis increasing for 1 week and consequent inability to walk unaided. In addition, the patient had several isolated nodules on the lower extremities without any tenderness to pressure. Laboratory tests showed elevated pancreatic enzymes indicative of pancreatitis, which was thereafter confirmed by abdominal CT scan, with signs of chronic pancreatitis and concrements in the pancreatic duct. The diagnosis of panniculitis was established by dermatological consultation. Considering all these clinical features, PPP syndrome was diagnosed. In accordance with the pre-existing literature, oral glucocorticoid therapy and nonsteroidal anti-inflammatory drugs (NSAIDs) were given but failed to improve pain and polyarthritis. In the further clinical course, due to the persistent increase in lipase and amylase, endoscopic retrograde cholangiopancreatography (ERCP) was performed, showing small concrements in the pancreatic duct. During the intervention, the pancreatic duct was widened, the small concrements were removed, and a pancreatic duct stent was then implanted. Following ERCP, an instant decrease in pancreatic enzymes was observed, accompanied by a clear and sustained improvement of joint swellings and pain. With the typical triad of clinical findings in mind, one should consider PPP syndrome in the context of acute or chronic pancreatitis as well as in pancreatic malignancies. All involved disciplines (gastroenterology, dermatology, and rheumatology) should be familiar with this rare but severe condition. The prognosis depends on the extent of the functional deficit of the extremities and the progression of the underlying disease.

摘要

胰腺炎、脂膜炎和多发性关节炎(PPP)三联征是一种罕见的系统性综合征,发生于急性或慢性胰腺炎或胰腺恶性肿瘤患者。一位 50 岁男性患者因多发性关节炎加重 1 周,继而出现无法独立行走而就诊。此外,患者下肢有几个孤立性结节,无压痛。实验室检查显示胰腺酶升高,提示胰腺炎,腹部 CT 扫描证实为慢性胰腺炎,并可见胰管内结石。皮肤科会诊诊断为脂膜炎。鉴于所有这些临床特征,诊断为 PPP 综合征。根据现有文献,给予口服糖皮质激素和非甾体抗炎药(NSAIDs)治疗,但未能改善疼痛和多发性关节炎。在进一步的临床过程中,由于脂肪酶和淀粉酶持续升高,进行了内镜逆行胰胆管造影(ERCP),显示胰管内有小结石。在介入过程中,胰管扩张,小结石被取出,然后植入胰管支架。ERCP 后,胰腺酶立即下降,关节肿胀和疼痛明显且持续改善。考虑到典型的三联征临床表现,在急性或慢性胰腺炎以及胰腺恶性肿瘤的情况下,应考虑 PPP 综合征。所有相关学科(消化内科、皮肤科和风湿科)都应熟悉这种罕见但严重的疾病。预后取决于四肢功能缺陷的程度和基础疾病的进展。

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本文引用的文献

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The Pancreatitis, Panniculitis, and Polyarthritis (PPP) Syndrome: Subcutaneous Nodular Fat Necrosis, a Cutaneous Marker of Pancreatic Neoplasia.胰腺炎、脂膜炎和多关节炎(PPP)综合征:皮下结节性脂肪坏死,胰腺肿瘤的皮肤标志物。
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Clinical characteristics, treatment, and outcome of pancreatitis, panniculitis, and polyarthritis syndrome: a case-based review.胰腺炎、脂膜炎和多发性关节炎综合征的临床特征、治疗和转归:基于病例的综述。
Clin Rheumatol. 2021 Apr;40(4):1625-1633. doi: 10.1007/s10067-020-05333-8. Epub 2020 Aug 10.
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Pancreatitis, Panniculitis, and Polyarthritis Syndrome Simulating Cellulitis and Gouty Arthritis.
模拟蜂窝织炎和痛风性关节炎的胰腺炎、脂膜炎和多关节炎综合征
Korean J Gastroenterol. 2019 Sep 25;74(3):175-182. doi: 10.4166/kjg.2019.74.3.175.
4
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J Clin Rheumatol. 2020 Aug;26(5):e90. doi: 10.1097/RHU.0000000000000914.
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Int J Surg Case Rep. 2017;31:170-175. doi: 10.1016/j.ijscr.2017.01.037. Epub 2017 Jan 18.
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A case of pancreatitis, panniculitis and polyarthritis syndrome: Elucidating the pathophysiologic mechanisms of a rare condition.一例胰腺炎、脂膜炎和多关节炎综合征:阐明一种罕见病症的病理生理机制。
J Pediatr Surg Case Rep. 2015 May;3(5):223-226. doi: 10.1016/j.epsc.2015.03.014.
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Radiographics. 2016 May-Jun;36(3):710-34. doi: 10.1148/rg.2016150149.
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