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A Patient with Autoimmune Pancreatitis Type 1 with Previously Known Lymphadenopathy, Both in the Context of IgG4-related Disease

Unusual clinical course, Challenging differential diagnosis, Diagnostic / therapeutic accidents, Unusual setting of medical care, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)

Fazil M. Alidjan, Faiz Karim, Rob M. Verdijk, Joost W. van Esser, Marianne J. van Heerde

Netherlands Department of Internal Medicine, Erasmus Medical Centre, Rotterdam, Netherlands

Am J Case Rep 2015; 16:790-793

DOI: 10.12659/AJCR.895059

Available online:

Published: 2015-11-05


BACKGROUND: Autoimmune pancreatitis (AIP) is an important clinical pathologic concept of IgG-4-related disease. AIP is a rare cause of chronic pancreatitis, characterized by a fibroinflammatory process by lymphoplasmacytic infiltrates, storiform fibrosis, obliterative phlebitis, and increased IgG4+ plasma cells, leading to dysfunction of the pancreas. Affected patients with AIP frequently have disease affecting other organs or sites with similar histologic changes, elevated IgG4+ plasma cell infiltrate, and good response to corticosteroid therapy. These diseases often are not limited to the pancreas and the pancreas may not be involved at all.
CASE REPORT: We report a 62-year-old man with obstructive jaundice with pre-existent submandibular lymphadenopathy. Diagnosis of AIP was based on diagnostic criteria by the HISORT-criteria in combination with elevated IgG-4 serum levels. CT revealed a focal enlargement of the head of the pancreas, as well as mesenteric peripancreatic and mediastinal lymphadenopathy. He was treated with high-dose steroid in combination with azathioprine and showed good clinical response.
CONCLUSIONS: We report a case with pre-existent submandibular lymphadenopathy and obstructive jaundice based on AIP type 1, both in the context of IgG4-related disease.

Keywords: Antibodies, Anti-Idiotypic - immunology, Autoimmune Diseases - immunology, Biopsy, Diagnosis, Differential, Immunoglobulin G - immunology, Lymphatic Diseases - diagnosis, Pancreatitis, Chronic - immunology, Tomography, X-Ray Computed



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