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Cureus Jun 2020Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an under-recognized, benign fibroinflammatory disease most commonly seen in the submandibular gland of older...
Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an under-recognized, benign fibroinflammatory disease most commonly seen in the submandibular gland of older adult males. Sialolithiasis or bacterial infection was first suspected as an etiology, but CSS is now considered an immunoglobulin G4-related disease (IgG4-RD). IgG4-RD can affect almost every organ in the body, characterized by organ fibrosis with IgG4-positive plasmacytes. Numerous autoimmune-related diseases have been unified under IgG4-RD, including Mikulicz disease (MD), autoimmune pancreatitis, Reidel's thyroiditis, and others. In any organ, IgG4-RD can present similar to malignancy. Due to the ability to mimic malignancy, it is crucial to be aware of this under-recognized clinical entity. CSS is currently of broad and high clinical interest due to increased understanding, multiorgan involvement, and more clearly defined criteria. To increase awareness of this disease, we describe a rare presentation of CSS with a literature review.
PubMed: 32754389
DOI: 10.7759/cureus.8846 -
Clinical Case Reports Jun 2024This case highlights the importance of a definite diagnosis of an IgG4-related chronic sclerosing duodenitis based on histological and radiological findings to rule out...
KEY CLINICAL MESSAGE
This case highlights the importance of a definite diagnosis of an IgG4-related chronic sclerosing duodenitis based on histological and radiological findings to rule out any malignancy in the mass. While dealing with patients having concentric duodenal thickening resulting in stricture formation, one should think of inflammatory etiology as well. IgG4-related disease is one of these inflammatory disorders where we see soft tissue thickening without a large mass or any associated lymphadenopathy as in our case.
ABSTRACT
Immunoglobulin G4-related disease (IgG4-RD) is distinguished as an infiltration of IgG-4-positive plasmacytes involving inflammatory lesions across multiple organs which is accompanied by raised IgG4 levels in the serum. Several inflammatory disorders are recognized as part of the IgG4-RD family based on shared histopathological features, which include Mikulicz's disease, chronic sclerosing sialadenitis, or Riedel's thyroiditis. Our case highlights a distinctive presentation of IgG4-related diseases; a 58-year-old man presenting with duodenal stricture highly suspicious of a duodenal mass/ampullary mass later found to be due to IgG4-related sclerosing duodenitis with negative malignancy on biopsy. We present the diagnostic challenges faced and relevant findings noted.
PubMed: 38808195
DOI: 10.1002/ccr3.8980