Primary Sjögren's syndrome with diffuse cerebral vasculitis and lymphocytic interstitial pneumonitis

A 30-year-old black Haitian man with primary Sjögren's syndrome presented with minimal sicca symptoms, negative results of serologic testing, and predominant systemic involvement, including chronic bilateral lymphoplasmocytic pulmonary infiltrates and diffuse central nervous system vasculitis. This case illustrates the recognized fact that a paucity of sicca symptoms can be overshadowed by the predominance of other systemic manifestations, delaying the diagnosis of this disease. The angiographic results in this patient have not been reported before and support the role of vasculitis in the pathogenesis of the central nervous system manifestations in primary Sjögren's syndrome.