Background The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare disease (<1/10,000) with multiple cutaneous and musculoskeletal manifestations [1]. The most common dermatological manifestations are palmoplantar pustulosis (PPP) and severe acne (SA). Musculoskeletal manifestations are diverse, including the thoracic wall, spine, temporomandibular joint, and peripheral joints [2]. It is considered as part of spondyloarthropathies, however, no significant relationship with HLA-B27 has been found [3]. For treatment, non-steroidal anti-inflammatory drugs (NSAIDs), disease-modifying drugs (DMARDs) and bisphosphonates have been traditionally used. More recently, new therapies such as apremilast and biological DMARDs (4) are being used more frequently.

Objectives To examine the characteristics of individuals diagnosed with SAPHO syndrome during follow-up at multiple Rheumatology Services in Spain.

Methods This is a descriptive, cross-sectional, multi-center study, carried out in the Rheumatology Services of several hospitals in Spain. A convenience sampling was carried out, including all patients with SAPHO under active follow-up.

Results 50 patients were included (76% women), with a mean age of 48.56 (±12,28) years. The mean BMI was 24.84 (±4.31). Regarding cardiovascular risk factors, 36.2% and 23.4% were smokers and ex-smokers, respectively. 18% had hypertension, 28% had dyslipidaemia and 8% were diabetic.

Regarding of the musculoskeletal involvement, the most affected region was the anterior chest wall (74%), followed by peripheral arthritis (56%) and the spine (34%). 24% presented enthesitis and only 6% dactylitis. The most frecuent skin involvement was palmpplantar pustulosis in 54% (27), the distribution of dermatological manifestations is shown in Figure 1.

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Figure 1.

Skin manifestations in SAPHO syndrome.

In laboratory tests at diagnosis, acute phase reactants were slightly elevated, with a mean of CRP of 11.13 (±17.34) and ESR of 21.21 (±24.56). HLA-B27 and CW6 were positive in 12% (6) and 6% (3) of patients, respectively. The most commonly used imaging tests in the diagnosis and follow-up were plain x-rays (90%), MRI (76%), bone scintigraphy (72%), and CT scan (52%). The least used test was ultrasound, used in 17 patients (34%).

The treatment was mostly based in NSAIDs, 94% of patients received it at some point. 60% used DMARDsc (mostly methotrexate). 50% of patients received bDMARDs, and 9 patients received more than two bDMARDs throughout the follow-up. The distribution of the first bDMARD used is shown in Table 1.

The course of the disease was mostly recurrent (54%) with asymptomatic periods between flare-ups. About half of the patients (44%) in follow-up currently have active disease.

Conclusion Our cohort presented axial involvement like other series and significant peripheral involvement. The most frequent skin condition was PPP. Half of the patients in our cohort required bDMARD, with ADL being the most widely used.

References [1]Heldmann F, Kiltz U, Baraliakos X, Braun J. [SAPHO syndrome]. Z Rheumatol. Octubre de 2014;73(8):729-41.

[2]Hayem G, Bouchaud-Chabot A, Benali K, Roux S, Palazzo E, Silbermann-Hoffman O, et al. SAPHO syndrome: a long-term follow-up study of 120 cases. Semin Arthritis Rheum. diciembre de 1999;29(3):159-71.

[3]Colina M, Govoni M, Orzincolo C, Trotta F. Clinical and radiologic evolution of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: A single center study of a cohort of 71 subjects. Arthritis Care Res. 2009;61(6):813-21.

[4]Daoussis D, Konstantopoulou G, Kraniotis P, Sakkas L, Liossis S-N. Biologics in SAPHO syndrome: A systematic review. Semin Arthritis Rheum. febrero de 2019;48(4):618-25.

Acknowledgements To the Sociedad de Reumatología de la Comunidad de Madrid (SORCOM).

Disclosure of Interests Boris Anthony Blanco Cáceres Speakers bureau: MSD, Janssen, Novartis, UCB, Grant/research support from: Gebro, Pfizer, Novartis, Janssen, MSD, África Andreu-Suárez: None declared, Marta Valero: None declared, Diego Benavent: None declared, María Sanz: None declared, José Campos Esteban: None declared, Olga Rusinovich: None declared, Juan Molina Collada: None declared, patricia Castro: None declared, Vega Jovani: None declared, María Jesús Montesa: None declared, Eva Tomero Muriel: None declared, Álvaro García Martos: None declared, RAQUEL ALMODOVAR: None declared, Fernando Lozano Morillo: None declared.