Footnotes

• Handling editor Josef S Smolen

• Contributors SV wrote and revised the manuscript. EC draw the pictures and panel. LB, RP performed the immunological experiments and analysis. MT, IT, GS performed the genetic experiments (CGH array and exome sequencing) and analysis. LV performed CT scan and annotated panel B. TW supervised immunological work and reviewed the manuscript. AB is in charge of the patient and supervised the work. All authors critically reviewed the manuscript.

• Funding This study is funded by Agence Nationale de la Recherche (grant number: ANR LUMUGENE 2014).

• Competing interests None declared.

• Patient consent Obtained.

• Ethics approval Medical Ethics Committee of Sud Est III, Lyon, France.

• Provenance and peer review Not commissioned; externally peer reviewed.

• Data sharing statement No additional data available (raw data for genetic studies could be provided).