A 60-year-old man is hospitalized for acute pancreatitis. He reports swelling and tenderness in his jaw that appeared 6 months ago. He has no medical problems, takes no medications, and does not use tobacco, alcohol, or illicit drugs. There is no history of trauma to the abdomen. He does not have dry eyes or dry mouth. On admission, ultrasound of the abdomen shows no gallstones. CT of the abdomen reveals a 3x4-cm solid mass in the mid-portion of his pancreas with some surrounding edema. Chest CT reveals bilateral hilar adenopathy. He is started on intravenous fluids, opioids for pain control, and nothing by mouth, and his abdominal pain resolves.
On examination, he is well-nourished and appears comfortable. His blood pressure is 133/78 mm Hg, pulse is 90/min, and oxygen saturation is 98% on room air. There is bilateral enlargement of his parotid glands and some enlargement of his lacrimal glands. The remainder of his examination is within normal limits.
Laboratory results are as follows:

Serum chemistry and fasting lipid panels, liver function tests, urinalysis, and erythrocyte sedimentation rate are all within normal limits. HIV testing is negative.
Which of the following conditions is the most likely cause of this patient's presentation?

Question

A 60-year-old man is hospitalized for acute pancreatitis.  He reports swelling and tenderness in his jaw that appeared 6 months ago.  He has no medical problems, takes no medications, and does not use tobacco, alcohol, or illicit drugs.  There is no history of trauma to the abdomen.  He does not have dry eyes or dry mouth.  On admission, ultrasound of the abdomen shows no gallstones.  CT of the abdomen reveals a 3x4-cm solid mass in the mid-portion of his pancreas with some surrounding edema.  Chest CT reveals bilateral hilar adenopathy.  He is started on intravenous fluids, opioids for pain control, and nothing by mouth, and his abdominal pain resolves.
On examination, he is well-nourished and appears comfortable.  His blood pressure is 133/78 mm Hg, pulse is 90/min, and oxygen saturation is 98% on room air.  There is bilateral enlargement of his parotid glands and some enlargement of his lacrimal glands.  The remainder of his examination is within normal limits.
Laboratory results are as follows:

Serum chemistry and fasting lipid panels, liver function tests, urinalysis, and erythrocyte sedimentation rate are all within normal limits.  HIV testing is negative.
Which of the following conditions is the most likely cause of this patient's presentation?

Quizplus · Accepted Answer

11ec193a_96b5_ffd7_853d_3d34728fa0cc_MD0002_00 This patient's presentation of acute idiopathic pancreatitis, bilateral salivary and lacrimal gland enlargement, and lymphadenopathy suggests immunoglobulin IgG4-related disease (IgG4-RD), a fibro-inflammatory disease causing fibrosis and tumor-like swelling of affected organs. IgG4-RD is an emerging disease of unknown etiology. It most commonly affects middle-aged men and should be suspected in patients presenting with any of the following: Idiopathic pancreatitisSclerosing cholangitisBilateral salivary and/or lacrimal gland enlargement Idiopathic pancreatitis Sclerosing cholangitis Bilateral salivary and/or lacrimal gland enlargement Up to 40% of patients may have peripheral eosinophilia. Diagnosis is confirmed with tissue biopsy showing IgG4-positive plasma cells and lymphocytes. Elevated serum IgG4 can be helpful as well although serum levels can be normal. Patients typically respond to glucocorticoids. (Choice B) Patients with lymphoma can present with a pancreatic mass and lymphadenopathy, but they often present with B symptoms (eg, fever, weight loss, night sweats) as well. This patient's presentation of acute pancreatitis and bilateral parotid and lacrimal gland swelling is not typical for lymphoma. (Choice C) Alcoholism is sometimes associated with parotid gland enlargement in malnourished patients. Alcoholism sufficient to cause acute pancreatitis is often associated with increased mean corpuscular volume and elevated transaminases. This patient has none of these features and denies alcohol use. (Choice D) Autoimmune pancreatitis is a prototypical form of IgG4-RD that can mimic pancreatic cancer with a pancreatic mass and painless jaundice. However, there is usually no history of weight loss. (Choice E) Patients with Sjögren's syndrome usually complain of dry mouth and eyes, and/or arthralgia. They also have antibodies to SSA and SSB proteins and do not have recurrent episodes of pancreatitis, as is common in IgG4-RD. Patients with IgG4-related salivary and lacrimal gland disease do not have severe sicca symptoms despite glandular enlargement. Educational objective: IgG4-related disorders can present with autoimmune pancreatitis, lymphadenopathy, and bilateral salivary and/or lacrimal gland enlargement. Diagnosis is confirmed by peripheral eosinophilia, elevated serum IgG4 levels, and tissue biopsy showing IgG4-positive plasma cells. Treatment involves glucocorticoids and possibly azathioprine, mycophenolate mofetil, or rituximab for refractory disease. __________ References: Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. (http://www.ncbi.nlm.nih.gov/pubmed/22218969) Outcome of patients with type 1 or 2 autoimmune pancreatitis. (http://www.ncbi.nlm.nih.gov/pubmed/20736934)

A 60-Year-Old Man Is Hospitalized for Acute Pancreatitis