Discussion:
BD lacks specific histological or laboratory findings [2] and is very polymorphous and commonly fatal. The patient had BD triad which is diagnostic according to ICBD 2014 criteria [3]. BD etiology and pathophysiology are controversial with conflicting research results; BD is believed to cause immunological disturbances which affect immune regulation, making AID more likely to occur [4]. Autoinflammatory origin is also suggested for BD [5], with the result that BD can resemble Crohn’s disease by having polygenic auto-inflammation where IL-1β increases during attacks [6]. This made the fact that the sister of the patient in our case had Crohn’s disease seem relevant. In contrast, although BD typically causes hyper- vascularization to approximately all endocrine glands, an autoimmune etiology is suggested as the underline cause for BD rather than direct attacks from vasculitis [7]. There is also controversial data about thyroid functions (TSH, fT4, and T3) in BD patients. However, immunological mechanisms are suggested to be involved as auto-reactive T cells play a major role in BD which may explain thyroid dysfunction in BD [8]. Furthermore, few reports correlate BD with AD. One suggested a partially dysfunctional adrenal glands [9]. In our case, the patient suffered from multiple AID which were HD, AD and CD.
When multiple endocrines are being targeted by the immune system in one individual, he may have polyglandular autoimmune syndrome (PGA). The most common type is PGA-ll which is when a patient has AD with either autoimmune thyroid disease or diabetes type 1 or both. It may correspond with other AID such as CD. This is termed in combination as Schmidt syndrome [10]. In our case, the patient did not carry out any genetic test and family history was negative for HD, AD, diabetes type 1, BD and CD despite having 4 siblings in the family. This makes hereditary PGA-ll unlikely. Moreover, it is not evident whether ceasing the Immune-modulating drugs caused this AID in the BD patient, caused PGA-ll to be symptomatic, or was irrelevant.
In conclusion, we can neither confirm nor deny that our patient could have presented with PGA-ll and BD in unrelated manner. However, this is the first-reported case in that manner for best of our knowledge and further research is required.
Author contributions: