New Catalogue Sorts IgG4RD From Sjögren's

June 24, 2014
Lois Wingerson

(EULAR 2014) IgG4-related disorder and Sjögren syndrome share some symptoms, but have different treatments. A new study involving registry and clinical research data shows how to tell them apart.

The mucosal dryness of sicca, enlarged glands, and systemic involvement are features of both Sjögren syndrome and the rare, often deadly condition known as IgG4-related disease (IgG4RD). The wrong diagnostic call, obviously, would lead to the wrong treatment.

A multicenter team from Spain, with input from IgG4-RD expert Jon Stone MD of Harvard, has come up with a solution.  Matching data from the 921 patients in the GEAS-SS multicenter registry who fulfill classification criteria for Sjögren syndrome with another 662 patients included in altogether 23 clinical studies of IgG4RD, they have come up with a list of signs and symptoms that taken together depict the differences between the two.

The results were presented at the annual meeting of the European Union League Against Rheumatism (EULAR) in Paris by Pilar Brito Zeron MD of Hospital Clinic in Barcelona. We offer them below in chart form.

Note that certain laboratory values (IgG4 levels and the antibodies ANA, Ro/SS-A and La/SS-B) almost perfectly distinguish the two conditions. Also, in these data biliary tract and retroperitoneal involvement occurred only among patients with IgG4RD and only those with Sjögren syndrome showed joint involvement.

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