Marie-Eve Boucher1, Eric Dansin2, Mallorie Kerjouan3, Julien Mazieres4, Eric Pichon5, François Thillays6, Gilbert Massard7, Xavier Quantin8, Youssef Oulkhouir9, Virginie Westeel10, Luc Thiberville11, Christelle Clement-Duchene12, Franck Morin13, Pascal Thomas14, Nicolas Girard15, Benjamin Besse1
1Medical Oncology Department, Gustave Roussy, Villejuif, France;2Thoracic Oncology Department, Oscar Lambret, Lille, France;3Department of Pulmonology, Centre Hospitalier Universitaire de Rennes, Rennes, France;4Department of Pulmonology, Centre Hospitalier Universitaire de Toulouse, Toulouse, France;5Department of Pulmonology, Hôpital Bretonneau, Tours, France;6Radiation Oncology Department, Institut de Cancérologie de l’ouest, Rouen, France;7Thoracic Surgery Department, Centre Hospitalier Universitaire de Strasbourg, Strasbourg, France;8Department of Pulmonology and Addictology, Centre Hospitalier Universitaire de Montpellier, Montpellier, France;9Department of Pulmonology, Centre Hospitalier Universitaire de Caen, Caen, France;10Department of Pulmonology, Centre Hospitalier Universitaire de Besancon, Besancon, France;11Department of Pulmonology, Centre Hospitalier Universitaire de Rouen, Rouen, France;12Medical Oncology Department, Institut de Cancérologie de Lorraine, Nancy, France;13Clinical Research Department, Intergroupe francophone de cancérologie thoracique, Paris, France;14Thoracic Surgery Department, Hôpital Nord, Marseille, France;15Medical Oncology Department, Université Lyon 1, Institut Curie, Paris, France
Background: Thymic epithelial tumor (TET) has been associated with autoimmune disorders (AID) in up to 30% of patients. However, there have been wide variations in the reported prevalence of TET associated disorders based mostly on small single center series. RYTHMIC (Réseau tumeurs THYMiques et Cancer) is a French network mandated to systematically discuss every case of TET. Using our database, we aimed to describe the prevalence of AID in a large French TET population.
Methods: RYTHMIC database prospectively includes all consecutive patients with a diagnosis of TET discussed in our national tumor board. We calculated the prevalence and described epidemiologic, clinical and pathological characteristics of patients with TET’s related autoimmune diseases.
Results: From January 2012 to May 2017, 1,581 patients were included in the registry. Of these, 312 patients (19.7%) had autoimmune disorder. The mean age at diagnosis of TET was 56 years old and 52% were female. 233 had myasthenia gravis (65.8%), 19 Good syndrome (5.4%), 17 thyroiditis (4.8%), 16 systemic erythematous lupus (4.5%) and 14 pure red cell aplasia (4%). Some patients (10.3%) eventually developed more than 1 AID. Considering histologic characterization, 42.9% were B2 subtype, 17.1% AB subtype, 16.1% B3 subtype, 12.1% B1 subtype, 3.6% thymic carcinoma and 3.6% A subtype.
Conclusions: In our database of TET, the prevalence of autoimmune diseases was 11.8%, mostly in patients with B2, AB and B3 subtypes. This significant prevalence means that physicians must keep high awareness and systematically search for those comorbidities.
Keywords: Autoimmune diseases; thymoma
doi: 10.21037/med.2017.AB004