Zundler Sebastian, Strobel Deike, Manger Bernhard, Neurath Markus F, Wildner Dane
Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nuremberg, Ulmenweg 18, 91054, Erlangen, Germany.
Department of Medicine 3, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany.
Curr Rheumatol Rep. 2017 Aug 26;19(10):62. doi: 10.1007/s11926-017-0690-4.
Polyarthritis can have numerous reasons and may thus constitute a challenge for differential diagnosis. One rare potential reason for sterile polyarthritis is underlying pancreatic disease with systemic hyperlipasemia, most often accompanied by painful skin lesions caused by a subcutaneous inflammatory process known as panniculitis. Systematic evidence on pancreatic panniculitis and polyarthritis is limited, particularly regarding its feature as facultative paraneoplasia with underlying intra- or even extra-pancreatic malignancy. Therefore, we will summarize the current knowledge about this orphan disease including epidemiological, pathophysiological, diagnostic, and treatment aspects in the present review.
Although direct evidence is lacking, it is highly probable that pancreatic polyarthritis and panniculitis are caused by peripheral lipolytic activity of lipase systemically circulating due to benign (e.g., acute or chronic pancreatitis) or malign (e.g., acinar cell carcinoma (ACC) or adenocarcinoma) pancreatic disease. In the latter case, pancreatic polyarthritis and panniculitis are associated with poor outcome. Pancreatic polyarthritis and panniculitis should always be included into diagnostic considerations, and once suspected, a thorough work-up to identify the underlying disease has to be performed.
多关节炎可能有多种原因,因此可能构成鉴别诊断的挑战。无菌性多关节炎的一种罕见潜在原因是潜在的胰腺疾病伴系统性高脂血症,最常见的是伴有由皮下炎症过程(称为脂膜炎)引起的疼痛性皮肤病变。关于胰腺脂膜炎和多关节炎的系统性证据有限,特别是关于其作为潜在的胰腺内甚至胰腺外恶性肿瘤的潜在副肿瘤特征。因此,我们将在本综述中总结关于这种罕见疾病的当前知识,包括流行病学、病理生理学、诊断和治疗方面。
尽管缺乏直接证据,但胰腺多关节炎和脂膜炎很可能是由良性(如急性或慢性胰腺炎)或恶性(如腺泡细胞癌(ACC)或腺癌)胰腺疾病导致的全身循环脂肪酶的外周脂解活性引起的。在后一种情况下,胰腺多关节炎和脂膜炎与不良预后相关。胰腺多关节炎和脂膜炎应始终纳入诊断考虑范围,一旦怀疑,必须进行全面检查以确定潜在疾病。