Demystifying seronegative autoimmune pancreatitis

Gokulakrishnan Balasubramanian, Aravind Sugumar, Thomas C. Smyrk, Naoki Takahashi, Jonathan E. Clain, Ferga C. Gleeson, Phil A. Hart, Michael J. Levy, Randall K. Pearson, Bret T. Petersen, Mark D. Topazian, Santhi S. Vege, Suresh T. Chari

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


Background: Autoimmune pancreatitis (AIP) has been classified into type 1 and type 2 subtypes. Serum immunoglobulin G4 (IgG4) elevation characterizes type 1 AIP. Type 2 AIP and a subset of type 1 AIP are seronegative, i.e., have normal serum IgG4 levels. Aim: We compared the profiles of the three subsets of AIP to identify the unique characteristics of seronegative type 1 AIP and type 2 AIP. Methods: We compared the clinical profiles of 69 seropositive type 1 AIP patients, 21 seronegative type 1 AIP patients and 22 type 2 AIP patients. Results: Among type 1 AIP, seronegative group had similar clinical profiles when compared to seropositive group except that they were more likely to undergo surgical resection than seropositive patients (p < 0.001). Seronegative type I AIP patients were older (61.9 ± 13.7 vs 45.3 ± 17.4; p < 0.004), and differed in the occurrence of other organ involvement (OOI) (71.4% vs 0%; p lt; 0.001) and disease relapse (33.3% vs 0%; p < 0.005) when compared with type 2 AIP. All seronegative type 1 AIP patients had at least one of the following eOOI, disease relapse, and age >50 years while none of the type 2 AIP had OOI or disease relapse. Conclusions: Seronegative and seropositive type 1 AIP patients have similar clinical profiles, which are distinct from that of type 2 AIP. Among the seronegative AIP group, patients are more likely to have type 1 AIP rather than type 2 AIP if they are older than 50 years or have OOI or disease relapse.

Original languageEnglish (US)
Pages (from-to)289-294
Number of pages6
Issue number4
StatePublished - Jan 1 2012


  • Autoimmune
  • Chronic pancreatitis
  • IgG4
  • Pancreatitis
  • Steroid therapy

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Hepatology
  • Gastroenterology


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