SAPHO syndrome with rapidly progressing destructive spondylitis: two cases treated surgically

Area of Science:

• Rheumatology
• Orthopedic Surgery
• Dermatology

Background:

• Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare autoinflammatory disorder.
• Spinal involvement in SAPHO syndrome typically has a favorable prognosis and rarely leads to severe structural damage or neurological deficits.

Observation:

• Two cases of SAPHO syndrome with rapidly progressing destructive spondylitis are presented.
• Case 1: A 63-year-old female with palmoplantar pustulosis developed C4-C7 spinal cord compression and quadriplegia, treated with anterior and posterior decompression and reconstruction.
• Case 2: A 69-year-old female presented with T7-T9 spondylitis and spinal deformity, diagnosed with SAPHO syndrome based on hyperostosis, and treated with thoracic endoscopic curettage and reconstruction.

Findings:

• Both cases demonstrated severe spinal destruction and kyphotic deformity.
• Histopathology revealed non-specific inflammation in surgically obtained specimens.
• Surgical treatment improved neurological function and alleviated pain, preventing further spinal destruction.

Implications:

• SAPHO syndrome can manifest with severe, destructive spinal involvement, contrary to its usual good prognosis.
• Prompt surgical management is essential for patients with rapidly progressing destructive spondylitis secondary to SAPHO syndrome.
• Early diagnosis and intervention can prevent irreversible neurological damage and spinal deformities in affected individuals.