Es. Husebye et al., AUTOANTIBODIES AGAINST AROMATIC L-AMINO-ACID DECARBOXYLASE IN AUTOIMMUNE POLYENDOCRINE SYNDROME TYPE-I, The Journal of clinical endocrinology and metabolism, 82(1), 1997, pp. 147-150
Patients with autoimmune polyendocrine syndrome type I(APS I) have aut
oantibodies against the enzyme aromatic L-amino acid decarboxylase (AA
DC) of pancreatic beta-cells. The aim of the present study was to inve
stigate the presence of anti-AADC antibodies in a large cohort of pati
ents with APS I, and in patients with isolated insulin-dependent diabe
tes mellitus (IDDM). We found autoantibodies against AADC in 35 of 69
patients (51%) with APS I but in none of 138 patients with isolated ID
DM or 91 healthy controls. Among the patients with APS I, anti-AADC an
tibodies were more often found in those with hepatitis (11/12, 92%), t
han in those without hepatitis (24/57, 42%) (P = 0.003). Similarly, of
15 patients with vitiligo, 12 (80%) had anti-AADC antibodies, compare
d with 23/54 (43%) without vitiligo (P = 0.021). Of the 9 APS I patien
ts with IDDM, 5 had antibodies against both AADC and glutamate decarbo
xylase, 2 against AADC only, and 2 against glutamate decarboxylase onl
y. Interestingly, AADC is present in relatively large amounts in the l
iver, where its function is unknown. Thus, an autoimmune reactivity ag
ainst AADC may be involved in the pathogenesis of autoimmune chronic a
ctive hepatitis and vitiligo in APS I patients, whereas the role of AA
DC in the development of IDDM in these patients remains to be determin
ed.