Delplanque M, Galicier L, Oziol E, et al. - Researchers report the French cases of primary immune deficiencies (PID)-related AA amyloidosis (AAA), as well as ascertained its main characteristics and predisposing factors by conducting a systematic literature review. This analysis involved 40 patients, including 2 new French cases. At PID diagnosis, the mean age was 22.2 ± 16.02 years. AAA most commonly manifested as renal involvement (80%). Extremely heterogenous infections were identified; the most frequent was bacterial infection with pulmonary involvement. Especially common was bronchiectasis (52,5%). A delay of 16.18 ± 7 years was reported between the first symptoms of PID and AAA diagnosis. Follow-up revealed the deaths of 20 patients. Overall, experts concluded AAA as a rare life-threatening complication of PID, particularly in cases of long diagnostic and therapeutic delays. They suggested considering bronchiectasis as a warning sign of chronic inflammation as well as a raised risk of AAA.