Minor salivary gland inflammation in Devic’s disease and longitudinally extensive myelitis

Abstract

Devic’s disease is often considered as a variant of multiple sclerosis (MS). However, evidence suggests that Devic’s disease may be distinct from MS. Devic’s disease can coexist with connective tissue diseases, particularly Sjögren’s disease, but this association is rare with MS. Diagnosis of Sjögren’s disease in patients with neurological symptoms is often difficult. During early stages of Sjögren’s disease, patients may not fulfill all criteria for Sjögren’s disease. A high percentage of patients with Sjögren’s disease have inflammatory infiltrates in minor salivary glands, and this may be a reliable indicator of early or subclinical disease. We show high prevalence (80%) of salivary gland inflammation in Devic’s disease and longitudinally extensive transverse myelitis (LETM). We diagnosed 16 patients with Devic’s disease, and 2 of these satisfied criteria for Sjögren’s disease as did 2 of 9 patients with LETM. Anti-SSA/B titers were infrequently elevated. Although most did not satisfy criteria for Sjögren’s disease. 9 of 12 Devic’s disease patients and 7 of 8 LETM patients had severe salivary gland inflammation. Thus: (1) patients with Devic’s disease or with LETM who have positive labial biopsies but do not satisfy criteria for Sjögren’s disease could have subclinical Sjögren’s diseases. Alternatively, (2) as patients with Devic’s disease have elevated titers of several autoantibodies, so there may exist a set of antibodies that react with antigens in minor salivary glands and cause inflammation. Minor salivary gland biopsy is more sensitive than anti-SSA/B serology in providing histological evidence for possible Sjögren’s disease with CNS lesions.