Pancreatobiliary Versus Head and Neck Manifestations in Immunoglobulin G4-related Disease: Distinct Subsets of the Same Disease?

Abstract:

OBJECTIVES:We compared the clinical profiles and organ manifestations of the commonly encountered immunoglobulin G4-related diseases (IgG4-RDs) on either side of the diaphragm: head and neck (HN) versus pancreatobiliary (PB) in IgG4-RD. METHODS:From the Mayo Clinic, Rochester, database, we identified 53 HN and 88 PB IgG4-RD based on the first affected organ manifestation. RESULTS:Compared with HN IgG4-RD, subjects with PB IgG4-RD were likely to be older (median, 64.8 vs 50.2 years; P < 0.0001), male (83% vs 60.4%; P = 0.003), and with a shorter duration of follow-up (24.4 vs 48.7 months; P < 0.0001). In HN versus PB-IgG4-RD orbital, lacrimal gland, submandibular, parotid gland, asthma, and sinusitis manifestations were more common (77% vs 4.5%, 21% vs 0%, 32% vs 8%, 13% vs 0%, 36% vs 9%, and 51% vs 6.8%; P < 0.0001, respectively), whereas lung manifestations were similar (13.2% vs 5.6%; P = 0.12). In contrast, in PB versus HN IgG4-RD, pancreas and biliary were more frequent (98.8% vs 15%, 56.8% vs 3.7%; P < 0.0001), whereas renal lesions were similar (12.5% vs 7.5%; P = 0.36). CONCLUSION:Pancreatobiliary and HN IgG4-RD have distinct clinical profiles. Proximity matters in other organ involvement in IgG4-RD, and organs involved tend to cluster close to each.

journal_name

Pancreas

journal_title

Pancreas

authors

Mohapatra S,Sharma A,Chari ST

doi

10.1097/MPA.0000000000001332

subject

Has Abstract

pub_date

2019-07-01 00:00:00

pages

799-804

issue

6

eissn

0885-3177

issn

1536-4828

pii

00006676-201907000-00009

journal_volume

48

pub_type

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