Masaki et al proposed criteria for the IgG-4 positive multi-organ lymphoproliferative syndrome. The authors are from Kanazawa Medical University in Japan.
Criteria - all of the following:
(1) elevated serum IgG4 (> 1.35 g/L)
(2) tissue infiltrated by plasma cells with > 50% positive for IgG4
(3) presence of fibrosis and/or sclerosis
(4) exclusion of alternative diagnoses
Common findings:
(1) autoimmune pancreatitis
(2) allergic rhinitis
(3) elevated serum total IgG and IgE
(4) good clinical response to glucocorticoid therapy
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