Putative Infundibular And Pituitary Involvements Of Sapho Syndrome

Abstract

ABSTRACT: Objective: Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare, chronic, relapsing inflammatory osteoarticular disorder with dermatologic manifestations. SAPHO syndrome can be a rare cause of atypical symptoms including neurologic defects. We report a case with SAPHO syndrome with the endocrinologic defects most likely associated with SAPHO syndrome.Methods: Case report and review of the literature.Results: A 61-year-old Japanese female with SAPHO syndrome presented with polyuria and polydipsia. Pituitary magnetic resonance imaging (MRI) with gadolinium enhancement showed an enlarged infundibulum and pituitary with ring-enhancing masses located at the posterior pituitary and upper infundibulum. Extensive diagnostic workup excluded local and systemic infection, brain metastasis, and various secondary hypophysitis as the cause of the infundibular and pituitary lesions. There were spontaneous improvements in the MRI findings taken 4 months after her initial presentation. The patient underwent a biopsy of the anterior pituitary, which showed a sterile lymphocytic infiltration.Conclusion: There are rare cases of SAPHO syndrome patients in which transient neurologic symptoms associated with the self-limiting ring-enhancing lesions consisting of a sterile and nonspecific inflammation are described. Since cystic formation in the infundibulum caused by idiopathic lymphocytic hypophysitis has not been reported, to the best of our knowledge, we concluded that the endocrinologic defects in our patient were most likely associated with SAPHO syndrome. Thus, SAPHO syndrome should be considered as one of the causes not only for ring-enhancing lesions in the brain but also for secondary hypophysitis, especially in patients with dermatologic and osteoarticular manifestations.