SJÖGREN SYNDROME: AN UNDIAGNOSED ETIOLOGY FOR FACIAL PAIN: CASE SERIES WITH REVIEW OF NEUROLOGICAL MANIFESTATION OF SJÖGREN SYNDROME
- Kam A. Newman, United States of America
Abstract
Background and Aims
Facial pain is a challenging entity in medicine, and has a wide range of differential diagnoses. Therefore, different disciplines including neurology, otolaryngology, pain medicine, dentistry and neurosurgery may be involved in diagnosis and management of these individuals although rheumatologic etiology of facial pain has been less discussed and recognized.
Methods
In this case series, we catalogued eleven facial pain patients: 7 were diagnosed with trigeminal neuralgia, 3 with atypical facial pain, and 1 with persistent idiopathic facial pain. All patients were evaluated by several specialists including neurologists, dentists, endodontists, otolaryngologists, pain specialists and neurosurgeons. Two patients underwent craniotomy for microvascular decompression with diagnosis of trigeminal neuralgia with no relief.
Results
All patients were female with negative Sjögren’s syndrome antibody but proven by salivary gland biopsy. All but one were positive for ANA. ESR was normal in all patients, but CRP was elevated in three patients. Brain and trigeminal nerve MRI were unremarkable in all patients. Minor salivary gland biopsy confirmed lymphocytic sialadenitis. Lymphocytic sialadenitis is considered histologic hallmark of Sjögren’s syndrome. Lymphocytic sialadenitis is characterized by presence of significant perivascular or periductular lymphocytic infiltrate. All patients but one complained of sicca syndrome. Age of onset for facial pain in all patients except one was before 50.
Conclusions
CNS and cranial nerves involvement, particularly trigeminal nerve involvement that may present with facial pain or headaches, as the main pSS presenting manifestation has been underestimated and under-looked. We recommend painful trigeminal neuropathy secondary to pSS should be considered in differential diagnosis of facial pain particularly in patients who carry PIFP diagnosis.