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UniSR at the forefront for the 5th International Symposium on IgG4-Related Disease

12 April 2024
Scientific events

A team of clinicians and researchers from Università Vita-Salute San Raffaele and IRCCS Ospedale San Raffaele has organized the Fifth International Symposium on IgG4-Related Disease, for the first time hosted in Italy. San Raffaele is an Italian and European reference institute for this under-diagnosed rare disease, thanks to the largest case series of affected patients.

A multidisciplinary group of international experts has been brought together at the 5th International Symposium on IgG4-Related Disease to inform, debate, and discuss new aspects of the field. Topics on the agenda have included organ-specific manifestations of IgG4-Related Disease, diagnostic modalities, the latest immunopathogenesis findings, and present and future therapeutic approaches. The "unmet" needs of patients with this disease have also been discussed and identified during the opening ceremony.

Among them have been numerous clinicians and researchers from Università Vita-Salute San Raffaele and IRCCS Ospedale San Raffaele (immunologists, rheumatologists, gastroenterologists, hematologists, and radiologists), brought together in dedicated multidisciplinary teams to ensure the best patient care.

IgG4-Related Disease

IgG4-Related Disease is named after a particular group of antibodies called IgG4 ("Immunoglobulins of subclass 4"), which are present at very high levels in the blood of most patients. In addition, they show in the affected organs an abundant infiltrate of B lymphocytes, the cells of the immune system that secrete IgG4.

Left: Hematoxylin eosin staining of a pancreas affected by IgG4-Related Disease (autoimmune pancreatitis). Bundles of cells (fibroblasts) and collagen fibers are observed, drawing arabesques in the pancreatic tissue completely replaced by fibrotic phenomena. This type of fibrosis is called “storiform” from the Latin storea = mat. Right: immunohistochemistry performed on the aorta wall of a patient affected by IgG4-Related Disease. In darker brown, a rich infiltrate of B lymphocytes producing IgG4 antibodies is observed, typical of this pathology. Courtesy of Dr. Della Torre.

This is an emerging pathologic condition in the field of immune rheumatology, clinically characterized by expansive tumor-like lesions and dense fibrosis (i.e., hardening and thickening) of the affected tissues; if not treated promptly, these can lead to organ failure. IgG4-related disease was first described in 2001 in the pancreas ("autoimmune pancreatitis") and then progressively recognized in a variety of organs and systems. It is considered a rare condition, although incidence and prevalence of this condition are largely underestimated. To date, the pathogenetic mechanisms underlying the disease remain largely undefined.

The International Symposium on IgG4-Related Disease

After the United States and Japan, the International Symposium on IgG4-Related Disease has been hosted for the first time in Europe, and in particular in Italy and Milan, as evidence of the international scientific value of the work coordinated by UniSR doctors and researchers.

Scientific contact person for the Symposium is Dr. Emanuel Della Torre, rheumatologist, allergist and immunologist of the Clinical Unit of Immunology, Rheumatology, Allergology and Rare Diseases at IRCCS Ospedale San Raffaele and Researcher in Rheumatology UniSR. After years of experience in the United States, he returned to Italy in 2018 thanks to a Cariplo Foundation grant aimed at understanding some of the mechanisms underlying IgG4-Related Disease to identify targeted therapeutic targets. The prestigious Expertscape platform considers Dr. Della Torre a "World Expert" in this disease.

Comments Dr. Della Torre, European coordinator also of the study group dedicated to the disease in the context of the European Reference Networks (ERNs):

Thanks to the clinical experience gained over the years on a large court of patients, San Raffaele is the Italian reference institute for IgG4-Related Disease, one of the European reference centers and among the top three groups in the world in terms of publications and resonance. Organizing and hosting the conference here in Milan is a great honor and testifies to the value of the clinical and scientific commitment that we have invested for almost 20 years in trying to best treat our patients.

Dr. Della Torre continues:

During the three-day event, opportunities for multidisciplinary work on current and future studies have been highlighted. In addition, for the first time, an entire morning has been devoted to patients, patient associations, and patient-focused initiatives. Finally, an international collaborative effort has been initiated to define a clinimetric index of IgG4-Related Disease activity to guide therapeutic decisions. In an extraordinarily short time, IgG4-Related Disease has been identified and characterized, and effective targeted treatments that could gain rapid regulatory approval to improve patient care now appear possible.

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