SAPHO Syndrome Summary
What?
SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized by:
• Synovitis (inflammation of the joints)
• Acne (Acne conglobata or fulminans)
• Pustulosis (thick yellow blisters containing pus) often on the palms and soles
• Hyperostosis (increase in bone substance)
• Osteitis (inflammation of the bones)
Why?
• Etiology unknown: Autoinflammatory disorder
When to suspect?
• Pustular skin lesions + Rheumatic pain
Clinical Features:
• Synovitis: most often a nonerosive inflammatory arthritis
• Acne/Pustulosis: Variety of acneiform and neutrophilic dermatoses
• Skin findings: Hidradenitis suppurativa, palmar pustular pustulosis, pustular psoriasis, psoriasis vulgaris, Acne conglobata or fulminans
• Hyperostosis: Sclerotic bone changes with trabecular and cortical thickening
• Osteitis: pain, tenderness +/- sometimes swelling of the bone
• Bone and joint involvement: May involve anterior chest wall, parts of the axial skeleton-sacroiliac joint and spine, & medium to large lower-extremity joints
Diagnosis:
• A clinical diagnosis
• Rule out other causes such as infectious, malignant, or other etiology
• Laboratory findings: Nonspecific inflammatory changes: CRP, ESR
• Imaging:
- Plain radiographs of affected areas and if negative then whole-body scintigraphy.
- MRI: inflammation of the bone marrow or joints at characteristic sites such as the collar bone, breast bone, pelvis, heel, and lower jaw
Treatment:
• Osteoarticular manifestations: NSAIDs/glucocorticoid
• SAPHO syndrome with palmoplantar pustulosis: oral retinoid
• SAPHO syndrome with acne: antibiotics with antiinflammatory properties e.g Tetracyclines etc
TNF blockers had the best response rate in osteoarticular and skin disease. Other biologics reported to be efficacious include IL-1 inhibitors, IL-17 inhibitors, and IL-23 inhibitors.
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