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Pancreatic Panniculitis and Polyarthritis

  • Orphan Diseases (B Manger, Section Editor)
  • Published:
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Abstract

Purpose of Review

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.

Recent Findings

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.

Summary

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.

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Authors and Affiliations

  1. Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nuremberg, Ulmenweg 18, 91054, Erlangen, Germany

    Sebastian Zundler, Deike Strobel, Markus F. Neurath & Dane Wildner

  2. Department of Medicine 3, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen, Germany

    Bernhard Manger

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  1. Sebastian Zundler
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Correspondence to Sebastian Zundler.

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Zundler, S., Strobel, D., Manger, B. et al. Pancreatic Panniculitis and Polyarthritis. Curr Rheumatol Rep 19, 62 (2017). https://doi.org/10.1007/s11926-017-0690-4

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