Case report: remarkable remission of SAPHO syndrome in response to Tripterygium wilfordii hook f treatment

Abstract

Rationale: SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is an autoinflammatory disease with no standardized treatment. Tripterygium wilfordii hook f (TwHF) is a Chinese herb with immunosuppressive effects and has been used to treat some chronic inflammatory diseases. However, it has not been reported as a therapeutic option in SAPHO syndrome. Here we present the first report in which a remarkable remission of SAPHO syndrome was achieved in response to TwHF.

Patient concerns: A 57-year-old female patient noted swelling and pain at the anterior chest wall and scattered rashes like psoriasis vulgaris. Bone scintigraphy demonstrated the classic "bull's head" sign and magnetic resonance images indicated bone marrow edema on T5.

Diagnoses: The diagnosis was made by dermatological and osteoarticular manifestations and classical "bull's head" sign in bone scintigraphy.

Interventions: TwHF with a priming dose of 20 mg 3 times per day and a gradual dose reduction of 20 mg per day in every 3 months. Four months later, methotrexate was added with 10 mg per week.

Outcomes: Osteoarticular symptoms and radiological abnormalities were improved dramatically.

Lessons: This case illustrates a promising strategy to treat SAPHO syndrome.

Figure 1

Imaging examination results. (A) 99mTc bone scintigraphy demonstrated the classic “bull's head” sign in the anterior chest wall (while circle). (B) MR image (Fat-suppression T2WI sequence) obtained before TwHF treatment revealed high signal intensity in T5 (white arrow), corresponding to bone marrow edema. (C) Bone marrow edema disappeared (white arrow) 4 months after the administration of TwHF.

Figure 2

Decrease of inflammatory condition parameters and disease activity indexes after administration of TwHF. Methotrexate (MTX) was added after 4-month treatment of TwHF (black arrow).