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Joint Bone Spine
Volume 85, n° 6
pages 721-726 (décembre 2018)
Doi : 10.1016/j.jbspin.2018.01.014
accepted : 31 January 2018
Original Articles

Treatment and outcomes in patients with IgG4-related disease using the IgG4 responder index

Andreu Fernández-Codina a, b, Blanca Pinilla c, Iago Pinal-Fernández a, d, Cristina López c, Guadalupe Fraile-Rodríguez e, Eva Fonseca-Aizpuru f, Iago Carballo g, Pilar Brito-Zerón h, Carlos Feijóo-Massó i, Miguel López-Dupla j, Maria Cinta Cid h, Fernando Martínez-Valle a,

Spanish Registry of IgG4 Related Disease (REERIGG4) investigators

Autoimmune Diseases Group (GEAS)

Spanish Internal Medicine Society (SEMI)

a Internal medicine department hospital universitari Vall d’Hebron, 08035 Barcelona, Spain 
b Rheumatology division, department of medicine, university of Western Ontario, London, N6A 4V2 ON, Canada 
c Internal medicine department, hospital general universitario Gregorio Marañón, 28007 Madrid, Spain 
d Muscle disease unit, national institute of arthritis and musculoskeletal and skin diseases, Bethesda, 20892 Maryland, United States 
e Internal medicine department, hospital Ramón y Cajal, 28007 Madrid, Spain 
f Internal medicine department, hospital de Cabueñes, 33394 Gijón, Spain 
g Internal medicine department, hospital clínico universitario de Santiago, 15706 Santiago de Compostela, Spain 
h Internal medicine department, hospital Clínic i Provincial, 08036 Barcelona, Spain 
i Internal medicine department, consorci sanitari Parc Taulí, 08208 Sabadell, Spain 
j Internal medicine department, hospital Joan XXXIII, 43005 Tarragona, Spain 

Corresponding author at: Systemic autoimmune diseases unit, internal medicine department, hospital Vall d’Hebron. Passeig Vall d’Hebron 119-129, 08035 Barcelona, Spain.Systemic autoimmune diseases unit, internal medicine department, hospital Vall d’Hebron. Passeig Vall d’Hebron 119-129, 08035 Barcelona, Spain.

IgG4 responder index correlates with response to treatment in IgG4-related disease.
Glucocorticoids and rituximab are the only treatments with a minimum of evidence supporting their use.
Anti-rheumatic disease modifying drugs combined with glucocorticoids could be useful in cases of a relapsing disease.

The full text of this article is available in PDF format.

IgG4-related disease (IgG4-RD) is an autoimmune disease triggering an inflammatory cascade that leads to fibrosis. Outcome measures are limited and treatment options remain underexplored.


To assess the variation of the IgG4 responder index (IgG4-RI) in a cohort of IgG4-RD patients and to explore their treatments and outcomes.


We studied the clinical phenotype, severity of the disease and response to treatment in an ambispective multicenter cohort study including 14 different hospitals in Spain. All patients met the 2012 international consensus on pathology criteria for diagnosis.


Sixty-eight patients were included, with a mean age of 53.4 years and predominance of male sex. The most commonly involved tissues were: retroperitoneum (33%), orbital pseudotumor (28%) and maxillary and paranasal sinuses (24%). IgG4-RI values were higher in patients with multiorgan disease and before treatment. After being treated, IgG4-RI values were lower, in accordance with the high rates of treatment response. Most patients received: glucocorticoids (GC), surgery, azathioprine (AZA), mofetil mycophenolate or rituximab. GC alone, GC plus surgery and GC plus AZA were given in the most of the IgG4-RD disease activity episodes. All treatments had high response rates but relapses and flares were common.


IgG4-RI is a promising outcome measure in IgG4-RD, but still in development. Treatment algorithms are ill defined. GC and rituximab are the drugs with more evidence available. Disease modifying anti-rheumatic drugs may have a role in IgG4-RD and warrant more prospective studies.

The full text of this article is available in PDF format.

Keywords : IgG4-related disease, Outcomes, Treatment

© 2018  Société française de rhumatologie@@#104156@@