Abstract
Primary Sjögren's syndrome, like other autoimmune diseases, is a multisystemic disorder.
Trigeminal sensory neuropathy (TSN) has been recognized as a rare neurological complication of primary Sjögren's syndrome and the pathogenesis remains obscure. Previous reports have identified an association between primary Sjögren's syndrome and TSN in other countries but this relationship has been rarely reported in Japan.
We reported that six cases of 185 patients with primary Sjögren's syndrome complicated with TSN: one case with multiple cranial neuropathies including TSN, three cases with isolated TSN in the absence of other neurologic diseases, two cases with TSN as a part of peripheral neuropathy. In all cases TSN was considered to be peripherally involved.
All six cases were female, age range 34 to 58 years at onset of TSN. Neurological central nervous involvements and systemic vasculitis were not found in any cases. TSN preceded the diagnosis of primary Sjögren's syndrome in three cases, and in these cases TSN presented as an initial manifestation of primary Sjögren's syndrome.
Anti-RNP antibody was positive in four cases. Three cases out of four developed Raynaud's phenomenon. The interval between TSN and Raynaud's phenomenon in these cases was within two months. In one case with isolated TSN, thermography of the face was performed, and it showed that the temperature was low in the area of the involved trigeminal nerves.
These observations suggest that Raynaud's phenomenon and/or anti-RNP antibody may play a role in the pathogenesis of TSN in primary Sjögren's syndrome.
Our experience with six cases and review of the literature indicate that in patients with TSN, especially isolated TSN with Raynaud's phenomenon, the diagnosis of primary Sjögren's syndrome should be considered, even if sicca symptoms are absent, and that appropriate evaluation must be carried out.
