Abstract
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 intermittentfavorable 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.
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References
Chamot AM, Benhamou CL, Kahn MF, et al. Acne-pustulosis-hyperostosisosteitis syndrome: results of a national survey. 85 cases [in French]. Rev Rhum Mal Osteoartic 1987; 54: 187–96
Levin J, Werth VP. Skin disorders with arthritis. Best Pract Res Clin Rheumatol 2006; 20 (4): 809–26
Hughes RA. Arthritis precipitated by isotretinoin treatment for acne vulgaris. J Rheumatol 1993; 20: 1241–2
Pedraz T, Martínez A, Pascual E. Acute hip monoarthritis in a patient treated with isotretinoin. J Clin Rheumato 2006; 12: 105–6
Rohekar G, Inman RD. Conundrums in nosology: synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome and spondylarthritis. Arthritis Rheum 2006; 55: 665–9
Olivieri I, Padula A, Ciancio G, et al. Successful treatment of SAPHO syndrome with infliximab: report of two cases. Ann Rheum Dis 2002; 6: 375–6
Wagner AD, Andresen J, Jendro MC, et al. Sustained response to tumor necrosis factor alpha-blocking agents in two patients with SAPHO syndrome. Arthritis Rheum 2002; 46: 1965–8
Deutschmann A, Mache CJ, Bodo K, et al. Successful treatment of chronic recurrent multifocal osteomyelitis with tumor necrosis factor-alpha blockage. Pediatrics 2005; 116: 1231–3
Moll C, Hernández MV, Cañete JD, et al. Ilium osteitis as the main manifestation of the SAPHO syndrome: response to infliximab therapy and review of the literature. Semin Arthritis Rheum 2008; 37: 299–306
Sabugo F, Liberman C, Niedmann JP, et al. Infliximab can induce a prolonged clinical remission and a decrease in thyroid hormonal requirements in a patient with SAPHO syndrome and hypothyroidism. Clin Rheumatol 2008; 27: 533–5
Wagner AD, Andresen J, Jendro MC, et al. Sustained response to tumor necrosis factor alpha-blocking agents in two patients with SAPHO syndrome. Arthritis Rheum 2002; 46: 1965–8
Eleftheriou D, Gerschman T, Sebire N, et al. Biologic therapy in refractory chronic non-bacterial osteomyelitis of childhood. Rheumatology (Oxford) 2010; 49: 1505–12
Widmer M, Weishaupt D, Brühlmann P, et al. Infliximab in the treatment of SAPHO syndrome: clinical experience and MRI response [abstract]. Ann Rheum Dis 2003; 62: 250–1A
Asmussen KH. Successful treatment of SAPHO syndrome with infliximab: a case report [abstract]. Arthritis Rheum 2003; 48 Suppl.: S621A
Kyriazis NC, Tachoula AV, Sfontouris CI. Successful treatment of refractory SAPHO syndrome with infliximab [abstract]. Ann Rheum Dis 2004; 63: 388–9A
Fruehauf J, Cierny-Modré B, Caelen Lel-S, et al. Response to infliximab in SAPHO syndrome. BMJ Case Rep. Epub 2009 May 20
Vilar-Alejo J, Dehesa L, de la Rosa-del Rey P, et al. SAPHO syndrome with unusual cutaneous manifestations treated successfully with etanercept. Acta Derm Venereol 2010; 90: 531–2
Hurtado-Nedelec M, Chollet-Martin S, Nicaise-Roland P, et al. Characterization of the immune response in the synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome. Rheumatology (Oxford) 2008; 47: 1160–7
Wolber C, David-Jelinek K, Udvardi A, et al. Successful therapy of sacroiliitis in SAPHO syndrome by etanercept [in German]. Wien Med Wochenschr 2011; 161: 204–8
Castellví I, Bonet M, Narváez JA, et al. Successful treatment of SAPHO syndrome with adalimumab: a case report. Clin Rheumatol 2010; 29: 1205–7
Ben Abdelghani K, Dran DG, Gottenberg JE, et al. Tumor necrosis factoralpha blockers in SAPHO syndrome. J Rheumatol 2010; 37: 1699–704
Arias-Santiago S, Sanchez-Cano D, Callejas-Rubio JL, et al. Adalimumab treatment for SAPHO syndrome. Acta Derm Venereol 2010; 90: 301–2
Massara A, Cavazzini PL, Trotta F. In SAPHO syndrome anti-TNF-alpha therapy may induce persistent amelioration of osteoarticular complaints, but may exacerbate cutaneous manifestations. Rheumatology (Oxford) 2006; 45: 730–3
Iqbal M, Kolodney MS. Acne fulminans with synovitis-acne-pustulosishyperostosis-osteitis (SAPHO) syndrome treated with infliximab. J Am Acad Dermatol 2005; 52: 118–20
Bassi E, Poli F, Charachon A, et al. Infliximab-induced acne: report of two cases. Br J Dermatol 2007; 156: 402–3
Galadari H, Bishop AG, Venna SS, et al. Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome treated with a combination of isotretinoin and pamidronate. J Am Acad Dermatol 2009; 61: 123–5
Dawson HD, Collins G, Pyle R, et al. The retinoic acid receptor-alpha mediates human T-cell activation and Th2 cytokine and chemokine production. BMC Immunol 2008; 9: 16
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No sources of funding were used to prepare this case report. The authors have no conflicts of interest that are directly relevant to the content of this case report.
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Garcovich, S., Amelia, R., Magarelli, N. et al. Long-Term Treatment of Severe SAPHO Syndrome with Adalimumab. Am J Clin Dermatol 13, 55–59 (2012). https://doi.org/10.2165/11593250-000000000-00000
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DOI: https://doi.org/10.2165/11593250-000000000-00000