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Diffuse Lung Disease |

A Woman With Lymphangioleiomyomatosis and Acute Pancreatitis

Dhanu Bhat, MD; Syed Mudassar Naqshbandi, MD; Rajiv Dhand, MD; Naveed Sheikh, MD
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University of Tennessee Medical Center, Knoxville, TN


Chest. 2014;145(3_MeetingAbstracts):228A. doi:10.1378/chest.1836377
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Abstract

SESSION TITLE: ILD Case Report Posters I

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM

INTRODUCTION: Lymphangioleiomyomatosis(LAM) is an extremely rare lung disease affecting Caucasian women of childbearing age. There are approximately 1300 known patients with LAM in North America. There has been only one previously reported case of LAM involving the pancreas1. Here we discuss an unusual case of acute pancreatitis in a LAM patient.

CASE PRESENTATION: A 50-year-old female with biopsy proven LAM, chronic respiratory failure on domiciliary oxygen presented to the Emergency Department with sudden onset of acute epigastric pain, nausea, vomiting, fevers and chills. She also complained of increased shortness of breath than at her baseline. On physical examination, she was tachycardic with normal heart sounds, abdominal exam revealed diffuse tenderness on palpation, and lung examination showed fine rales. A serum lipase level was increased to 1672 mg/dl. CT scan of the abdomen with contrast revealed acute necrotic pancreatitis. Also, the chest CT scan showed diffuse cystic disease consistent with LAM in both lungs, a pulmonary embolism (PE) in the right upper lobe and a right pleural effusion. The patient was managed with aggressive volume resuscitation; oral intake was stopped and heparin anticoagulation for PE was started. An ultrasound of the abdomen showed no evidence of gall stones, blood alcohol level and lipid profile were normal, and lower extremity dopplers showed no evidence of deep venous thrombosis. The patient’s condition improved and lipase level returned to normal. She was advised to follow up with gastroenterology for ERCP in 3 weeks after discharge.

DISCUSSION: To the best of our knowledge this is the first case of acute pancreatitis reported in a patient with LAM. The relationship of LAM to acute pancreatitis is not well established. In the absence of known common causes of acute pancreatitis, such as gall stone disease, alcohol intake, or drug reaction, in our patient we could speculate that atypical smooth muscle proliferation around the pancreatic duct could have caused the obstruction that lead to the acute pancreatitis. A similar process of atypical smooth muscle cell proliferation around the bronchovascular structures and interstititum creates a ball valve obstruction that leads to the distension of the terminal airspaces and cyst formation in the lung that is so characteristic of pulmonary involvement in LAM.

CONCLUSIONS: Though extrapulmonary manifestations in LAM have been reported, they are extremely rare. This case is unique as it illustrates an unusual presentation of likely extrapulmonary LAM as acute pancreatitis.

Reference #1: Solitary lymphangioleiomyoma of pancreas mimicking pancreatic pseudocyst--a case report. J Gastrointest Cancer. 2012 Jun;43(2):336-9

DISCLOSURE: Rajiv Dhand: Consultant fee, speaker bureau, advisory committee, etc.: Speaker bureau - GSK, Consultant fee, speaker bureau, advisory committee, etc.: Advisory Committee - Mylan, Cardeas Pharma, Other: Research studies - Bayer, Mylan, Other: International Physician Coordinator for multi-center clinical trial - Astra-Zeneca The following authors have nothing to disclose: Dhanu Bhat, Syed Mudassar Naqshbandi, Naveed Sheikh

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