Long-term remarkable remission of SAPHO syndrome in response to short-term systemic corticosteroids treatment in an immunoglobulin e elevated patient: A case report

Abstract

Rationale: Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare auto-inflammatory disease with no standardized treatment. Systemic corticosteroids are only transiently effective, but long-term use would bring complications and would not bring long-term remission. Bone scintigraphy is a first-line method for systematic evaluation of osteoarticular lesions but seems to show an "imprinting"pattern. Patient concerns: A 31-year-old female patient presented significant palmoplantar pustulosis and nail lesion as well as typical tracer accumulation feature on bone scintigraphy with normal hypersensitivity C-reactive protein and erythrocyte sedimentation rate, but an elevated serum immunoglobulin E level. Diagnosis: The diagnosis was made by dermatological manifestations and classical sign in bone scintigraphy in accordance with the diagnostic criteria proposed in 1988. Interventions: Methylprednisolone was given with a primary dose of 40mg/day for 1 week followed with a subsequent 20mg/day oral prednisone for another 1 week and then reduced in a rate of 5mg/week until the eventual cessation. Outcomes: Long-term remarkable remission on clinical manifestations, MRI performance, and quantitative analysis of bone scintigraphy was achieved. Lessons: Identification of specific subtype of SAPHO patient according to skin and nail manifestations as well as immunoglobulin E level may guide the selection of short-term systemic corticosteroids strategy, leading to remarkable long-term remission. Besides, the lesions on bone scintigraphy can hardly disappear in SAPHO patients, and instead, the quantitative analysis of bone scintigraphy and MRI performances may better reflect the change of disease condition and serve as indicator for treatment efficiency.