Long-term treatment of severe SAPHO syndrome with adalimumab: case report and a review of the literature.
SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome defines an association of inflammatory cutaneous disorders with osteoarticular manifestations and represents a clinical and therapeutic challenge. We report a case of severe SAPHO syndrome with acne conglobata and a diffuse involvement of the anterior chest wall and sacroiliac joints that required treatment with isotretinoin and adalimumab, a new fully human anti-tumor necrosis factor (TNF)-α monoclonal antibody. Combination treatment determined a complete clinical remission of cutaneous and osteoarticular manifestations after 48 weeks. Despite maintenance of clinical remission, follow-up imaging studies after 24 months of adalimumab monotherapy revealed osteoarticular disease progression, with features of inflammatory osteitis. TNFα antagonists have been used as third-line therapy for SAPHO syndrome in single case reports or case series, but these lack consistent long-term follow-up. SAPHO syndrome can present an intermittent-favorable course in the majority of cases as well as a chronic-progressive course, the latter requiring aggressive combination treatment with TNFα antagonists and conventional systemic agents.
Department of Internal Medicine and Specialist Sciences, Institute of Dermatology, A. Gemelli University Hospital, Catholic University, Rome, Italy. simgarko@yahoo.it
Simone
S
Garcovich
Rosanna
R
Amelia
Nicola
N
Magarelli
Venanzio
V
Valenza
Pierluigi
P
Amerio
D020083:Acquired Hyperostosis Syndrome / Q000188:drug therapy*; D000068879:Adalimumab; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000494:pharmacology / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized / Q000494:pharmacology / Q000627:therapeutic use*; D003879:Dermatologic Agents / Q000627:therapeutic use*; D006801:Humans; D015474:Isotretinoin / Q000627:therapeutic use*; D008297:Male; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
D000893:Anti-Inflammatory Agents; D061067:Antibodies, Monoclonal, Humanized; D003879:Dermatologic Agents; D014409:Tumor Necrosis Factor-alpha; D015474:Isotretinoin; D000068879:Adalimumab