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 Table of Contents  
IMAGES IN RHEUMATOLOGY
Year : 2018  |  Volume : 13  |  Issue : 4  |  Page : 280-281

Acute pancreatitis presenting with polyarthritis


Department of Medicine, JIPMER, Puducherry, India

Date of Web Publication18-Nov-2018

Correspondence Address:
Dr. Shahanaze Javath Hussain
Department of Medicine, JIPMER, Gorimedu, Puducherry - 605 006
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/injr.injr_109_18

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Keywords: Pancreatitis, panniculitis, polyarthritis, septic arthritis


How to cite this article:
Hussain SJ, Amalnath D, Wyawahare M, Subrahmanyam D. Acute pancreatitis presenting with polyarthritis. Indian J Rheumatol 2018;13:280-1

How to cite this URL:
Hussain SJ, Amalnath D, Wyawahare M, Subrahmanyam D. Acute pancreatitis presenting with polyarthritis. Indian J Rheumatol [serial online] 2018 [cited 2019 Jan 20];13:280-1. Available from: http://www.indianjrheumatol.com/text.asp?2018/13/4/280/241784



A 47-year-old man presented with symmetrical polyarthritis affecting knees, ankles, wrist joint, and right thumb for 2 weeks [Figure 1] and [Figure 2]. On examination, the joints were swollen, erythematous, and tender. He was initially treated as septic arthritis. Thick pus was aspirated from the knee joints. He was empirically started on broad-spectrum intravenous antibiotics (meropenem and vancomycin) and had undergone arthrotomy and lavage of the knees for septic arthritis. Two months earlier, he had been treated conservatively for alcohol-related acute pancreatitis and computed tomography (CT) abdomen at that time showed an enlarged pancreas. He had also undergone surgery for pancreatic pseudocyst 5 years back.
Figure 1: Bilateral wrist joint and left metacarpophalangeal joint swelling

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Figure 2: Bilateral ankle joint swelling

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As there was no improvement we considered the possibility of pancreatitis panniculitis polyarthritis (PPP) syndrome. Although the patient did not have abdominal pain at presentation, CT abdomen revealed a pancreatic pseudocyst [Figure 3]. His serum amylase was elevated (3946 IU/L). Our patient did not have clinical findings of panniculitis, but his synovial fluid examination showed fat globules [Figure 4]. Magnetic resonance imaging (MRI) of his hands and feet showed intramedullary fat necrosis. The patient underwent a cystogastrostomy for the pancreatic pseudocyst 3 weeks after the onset of the polyarthritis. His subsequent serum amylase levels were 420 IU/L. His joint swelling subsided after 2 weeks with moderate residual restriction of joint movements.
Figure 3: Computed tomography abdomen showing pancreatic pseudocyst

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Figure 4: Synovial fluid wet mount showing fat globules

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PPP is a rare syndrome with <64 cases described in literature till date.[1] Patients are usually middle-aged men with a history of heavy alcohol consumption. Abdominal symptoms and sign are often absent. The prominent symptom is polyarthritis which is usually symmetrical and involves the metacarpophalangeal joints, wrist, ankle, and knee joints.[2] Panniculitis is another feature described, and it presents either as erythematous and tender cutaneous nodules or as intramedullary fat necrosis as in this case. As mentioned this syndrome is hypothesized to occur due to release of pancreatic enzymes into circulation in patients with pancreatitis with serum lipase being the major enzyme implicated.[3] Patients are investigated for the arthritis and panniculitis leading to a delay in diagnosis of underlying pancreatic disease. Synovial fluid has a creamy appearance resembling pus, and hence patients receive antibiotics and undergo unnecessary procedures which may delay recovery. Serum amylase and lipase levels are raised by several fold. Imaging of the abdomen by endoscopic ultrasound or CT may reveal a pseudocyst or pancreatic mass. MRI of the bone may reveal intramedullary fat necrosis seen as osteolytic lesions described as moth-eaten appearance which may support the diagnosis of this condition.[4] PPP syndrome is associated with a high morbidity and mortality which can be ameliorated by early diagnosis and appropriate treatment. In this case, drainage of the pancreatic pseudocyst led to resolution of symptoms but left the patient with permanent deformity due to a delay in diagnosis. PPP syndrome occurring in the background of pancreatic malignancy has a grave prognosis and a high mortality (24%) despite appropriate treatment.[5] About half of the patients with PPP syndrome suffer from a permanent disability.[6]

PPP syndrome is a rare presentation of acute pancreatitis. Not recognizing this uncommon condition may lead to a delay in the diagnosis of the underlying pancreatic disease and treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ferri V, Ielpo B, Duran H, Diaz E, Fabra I, Caruso R, et al. Pancreatic disease, panniculitis, polyarthrtitis syndrome successfully treated with total pancreatectomy: Case report and literature review. Int J Surg Case Rep 2016;28:223-6.  Back to cited text no. 1
    
2.
Kocabaş H, Melikoğlu MA, Sezer İ, Gürbüz Ü, Kaçar C, Bütün B. Acute pancreatitis presenting with polyarthritis and intraosseous fat necrosis: A case report. Arch Rheumatol 2010;25:221-4.  Back to cited text no. 2
    
3.
A Case of Pancreatitis, Panniculitis and Polyarthritis Syndrome: Elucidating the Pathophysiologic Mechanisms of a Rare Condition – ScienceDirect. Available from: http://www.sciencedirect.com/science/article/pii/S2213576615000445. [Last accessed on 2017 Nov 07].  Back to cited text no. 3
    
4.
Kang DJ, Lee SJ, Choo HJ, Her M, Yoon HK. Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome: MRI features of intraosseous fat necrosis involving the feet and knees. Skeletal Radiol 2017;46:279-85.  Back to cited text no. 4
    
5.
Opsomer D, Suttels V, De Keyser K, Spanoghe G, Goeteyn V, Deprez S, et al. Pancreatic disease, Panniculitis and Polyarthritis (PPP) syndrome: A case report and review of the literature. Belg J Med Oncol 2011;5:154-8.  Back to cited text no. 5
    
6.
Narváez J, Bianchi MM, Santo P, de la Fuente D, Ríos-Rodriguez V, Bolao F, et al. Pancreatitis, panniculitis, and polyarthritis. Semin Arthritis Rheum 2010;39:417-23.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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