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:
- Ameer Kakaje M.D: Drafting, literature check, writing and revision,
and final submission.
- Rama Awad M.D: Acquiring and analysing the data and final revision of
the text.