Heerfordt's syndrome (HS) consists in its complete form of uveitis, parotid or 
salivary gland enlargement and cranial nerve palsy. The objective of the present 
study was to analyse if there are also links between HLA-DRB1* alleles and HS, 
as it is a specific phenotype of sarcoidosis. 1,000 patients with sarcoidosis, 
out of whom 83 had symptoms associated with HS, were included in the study 
together with a group of 2,000 healthy individuals from the same population, 
matched for sex and age. HLA-DRB1* allelic groups were determined for all 
individuals, and comparisons were made between different disease subgroups and 
between patients and healthy controls. We found that the HLA-DRB1*04 allele was 
overrepresented in patients with symptoms associated with HS. 83 (8.3%) of all 
patients had one or more of the symptoms and 46 (55%) of them were HLA-DRB1*04 
positive. 44 (55%) of the patients with ocular sarcoidosis, i.e. the most common 
symptom associated with HS, were HLA-DRB1*04 positive, compared with 35.9% of 
healthy controls (p=0.0008), and only 26.6% of the whole group of sarcoidosis 
patients (p<0.0001). HLA-DRB1*04 seems to protect against overall sarcoidosis 
but appears to be a significant risk factor for ocular sarcoidosis as well as 
for other manifestations associated with HS.