To describe the first case of human T-cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy, Sjögren syndrome, and lymphocytic pneumonitis in a nonendemic area.
Retroviruses are implicated in the pathogenesis of autoimmune diseases, including Sjögren syndrome. Asymptomatic lymphocytic pneumonitis is prevalent in HTLV-1-associated myelopathy. There are 7 case reports with the combination of HTLV-1-associated myelopathy, Sjögren syndrome, and lymphocytic pneumonitis, all of them in endemic areas for HTLV-1.
Case report and literature review.
A 40-year-old Creole woman from New Orleans, La, presented with progressive spastic paraparesis and exertional dyspnea. Review of systems revealed chronic complaints consistent with sicca syndrome. She was found to have HTLV-1-associated myelopathy by polymerase chain reaction in the cerebrospinal fluid. Increased levels of SSA, positive results on a Schirmer test, and the findings of biopsy of the minor salivary gland were consistent with Sjögren syndrome. A lung biopsy specimen showed marked lymphocytic infiltration.
The present case raises questions about the role of HTLV-1 in the development of autoimmunity. It also happens to be a unique occurrence in a nonendemic area.