MMP9 and triple-A syndrome: Despite the indisputable value of these observation in understanding TAA pathogenesis and their potential implications in diagnosis, it has to be noticed that those markers are not specific for the phenotype, and their levels are altered in several other processes such as AAA (MMP-2, MMP-9), cancer (MMP-9, TIMP-1, TIMP-2), renal disease (TIMP-2), and so on [74] and are currently not included in the recommended work-up for TAA diagnosis and management nor in the genetic screening.