IgG4 Related Disease (IgG4 RD)_ERN Re-CONNET
WHAT IS?
WHAT ARE THE MANIFESTATIONS of IgG4-RD AND HOW IS IT RECOGNIZED
IgG4-RD typically presents with symptoms and signs arising because of the compression exerted by the fibrotic mass on adjacent anatomical structures. Due to its mass forming aspect and to the lack of accurate disease biomarkers, IgG4-RD is classically misdiagnosed with a solid or haematological tumor. Serum IgG4, for instance, are increased in the majority of patients but, if taken alone, have relevant shortcomings for diagnostic and follow-up purposes. Similarly, imaging studies such as Computed Tomography, Magnetic Resonance, and 18-Fluorodeoxyglucose PET scan are useful to have a complete picture of the organs involved by IgG4-RD, but definitive diagnosis still requires histological examination of affected organs.
WHO IS AFFECTED
HOW DO WE TREAT IT Patients with IgG4-RD referred to San Raffaele Hospital are followed via the national health care system in a dedicated Clinic (The “IgG4-RD Clinic”) by immunologists and rheumatologists with years of expertise in the field. Our team was, in fact, the first to describe IgG4-RD and its multifaceted manifestations in Italy and presently follows-up the largest cohort of Italian IgG4-RD patients. Our group published more than 100 scientific works on peer-reviewed international journals dealing with IgG4-RD, contributed to the development of international diagnostic and therapeutic guidelines, and received financial support to address the molecular bases of this rare disorder. Because IgG4-RD can affect many different organs, each case is periodically discussed in multidisciplinary teams within San Raffaele Hospital to provide the better therapeutic options for each patient. The multidisciplinary team includes gastroenterologists, hepatologists, ENT specialists, ophthalmologists, pulmonologists, neurologists, pancreatic, biliary and thoracic surgeons among others. Being recognized as a national and international referral center for IgG4-RD, the IgG4-RD Clinic at San Raffaele Hospital also offers the possibility to be enrolled in research projects, to participate in clinical trials, to benefit from up-to-date off-label treatments, and to discuss social and psychological aspects of IgG4-RD. at Ospedale San Raffaele |