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Synovitis-Acne-Pustulosis- ABSTRACT Spondylitis, OR 10 (95% CI: 3.3-25, Hyperostosis-Osteitis Background and Objectives

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Synovitis-Acne-Pustulosis- ABSTRACT Spondylitis, OR 10 (95% CI: 3.3-25, Hyperostosis-Osteitis Background and Objectives Clinical and Experimental Rheumatology 2007; 25: 125-128. BRIEF PAPER Synovitis-acne-pustulosis- ABSTRACT spondylitis, OR 10 (95% CI: 3.3-25, hyperostosis-osteitis Background and objectives. Patients p < 0.0001). with psoriatic arthritis (PsA) as well as Conclusions. Psoriasis/PsA and SAP- syndrome and psoriatic those with synovitis, acne, pustulosis, HO syndrome show a different immuno- arthritis exhibit a different hyperostosis, osteitis (SAPHO) syn- genetic background, however the genet- immunogenetic profi le drome share some common features, and ic basis of SAPHO syndrome remains in fact, for many authors the SAPHO unknown. R. Queiro, P. Moreno, concept fi ts well into the broader concept C. Sarasqueta*, M. Alperi, of PsA. However, some clinical features Introduction J.L. Riestra, J. Ballina are unique to the SAPHO syndrome, and Since 1959 there have been several re- in the other hand, these patients do not ports of patients affected by the com- Rheumatology Department, Hospital show the known association between the bination of cutaneous lesions such as Universitario Central de Asturias (HUCA), HLA-B27 antigen and the spondyloar- acne, palmo-plantar pustulosis (PPP), Oviedo, Spain; *Clinical Epidemiology thropathies. To date, there are no studies hidradenitis suppurativa or pyoderma Unit, Complejo Hospitalario de Donostia, comparing the immunogenetic profi le of gangrenosum, with osteoarticular man- San Sebastián, Spain. these two conditions, so the main objec- ifestations including peripheral syno- Rubén Queiro,Queiro, PhD;PhD; PuertoPuerto Moreno,Moreno, MD;MD; tive of the present report was to analyse vitis, sacroiliitis, hyperostosis, anterior Cristina Sarasqueta, PhD; Mercedes whether or not both entities may share chest wall swelling or sterile osteitis (1, Alperi, MD; José LuisLuis Riestra,Riestra, PhD;PhD; the same genetic basis. 2). Such heterogeneous conditions were Javier Ballina, PhD. Patients and methods. All patients grouped by French investigators in the Please address correspondence and with SAPHO syndrome (n = 25) seen in mid-1980s under the SAPHO (synovi- reprint requests to: Dr. R. Queiro, a single university hospital from 1985 tis, acne, pustulosis, hyperostosis, os- Staff rheumatologist, HUCA, C/ Celestino Villamil s/n, 33006, to 2005 were recruited and followed teitis) rubric, and since then, the term Oviedo, Spain. up in standardised manner in order to SAPHO has been used as a convenient E-mail: [email protected] study their main characteristics and descriptor in clinical reports and case Received on March 22, 2007; accepted in HLA profi le. The HLA-Cw6, DR and reports worldwide (3). Although both revised form on September 19, 2007. B27 antigen distribution of these cases the SAPHO syndrome acronym and © CopyrightCopyright CLINICAL AND was compared to that of 50 patients with classifi cation criteria were proposed in EXPERIMENTAL RHEUMATOLOGY 2008.2008. psoriasis vulgaris, 120 with PsA, and 1987 by Chamot et al., these criteria 170 healthy blood donors. PsA patients have not been validated in comparison Key words: Psoriasis, psoriatic were classifi ed in accordance with their with closely related conditions, partic- arthritis, SAPHO syndrome, predominant pattern observed in the ularly spondyloarthopathies (SpA), al- HLA antigens. last 5 years of disease evolution. Odds though some authors have included the ratios (OR) values were calculated to SAPHO syndrome among the variants measure the strength of the association of psoriatic arthritis (PsA) (3, 4). between HLA antigens and disease, On the other hand, this disease group- while the statistical signifi cance of the ing appears to have a different genetic association was assessed with a two- basis, with neither the HLA DR3/DR4 tailed Fisher’s exact test. P < 0.05 val- association of connective tissue dis- ues were considered signifi cant. eases (CTD)/ rheumatoid arthritis Results. No association was found be- (RA) nor the B27 association of SpA tween HLA-Cw6, B27, or DR antigens, (5). Thus, the SAPHO acronym still re- and SAPHO syndrome. HLA-Cw6 was mains a good term to describe a group strongly associated with psoriasis, of arthritides which share common fea- OR 12 (95% CI: 5.6-26, p < 0.0001) tures, but there is still a need to clearly and PsA, OR 10 (95% CI: 5.4-19.5, elucidate whether this group is a sin- p< 0.0001), however this antigen was gle disease or syndrome, or in contrast, equally distributed among the three whether it could be assigned to the articular categories of PsA. HLA-DR4 spondyloarthropathy group in general, was found under-represented in PsA and to PsA in particular. patients compared to controls, OR 0.4 The present study was undertaken to (95% CI: 0.2-0.7, p = 0.002). HLA- analyse the clinical picture and the DR7 correlated well with psoriatic HLA profi le of a series of patients with oligoarthritis, OR 9.6 (95% CI: 2.9-28, SAPHO syndrome, and in order to com- p< 0.0001), HLA-DR8 was found asso- pare the HLA profi le of these patients to ciated with polyarthritis, OR 6.7 (95% that of PsA patients, analysing if these CI: 2-25, p = 0.002), while HLA-B27 two conditions may show a similar or a Competing interests: none declared. was over-represented in psoriatic different genetic background. 125 BRIEF PAPER SAPHO syndrome and HLA antigens / R. Queiro et al. Patients and methods group, irrespective of the presence of Table I. Diagnostic criteria for SAPHO All patients from a single teaching hos- peripheral synovitis (n = 42). This lat- syndrome. pital fulfi lling the SAPHO criteria pro- ter defi nition for axial disease in PsA Sterile multifocal chronic osteitis: posed by Chamot et al. (3) (Table I) is not universally accepted but may Involvement of the chest, spine, pelvis or long were recruited and their clinical records represent an appropriate view for clas- bones reviewed for the purposes of the present sifi cation purposes. Distal interphalan- Absence of skin lesions study. Most patients were sent to our geal joint (DIP) disease and “mutilans” Acute or chronic arthritis associated with: department by general practitioners and forms were also recorded, however, Palmoplantar pustulosis dermatologists. All cases were evalu- these forms could be assigned to the Palmoplantar pustular psoriasis Severe acne ated by dermatologists of our hospital in oligoarticular, polyarticular or spondy- Hidradenitis supurativa order to establish an accurate diagnosis litic groups over time. of the cutaneous lesions. All remarkable HLA-Cw6 was investigated by the Sterile mono or polyosteitis associated with: Palmoplantar pustulosis data were collected in a standard man- PCR-SSOP method, while HLA-B27 Palmoplantar pustular psoriasis ner, and this included age at onset, sex, and DR antigens were analysed by clas- Severe acne disease duration, family history, medi- sical serological methods. This HLA Hidradenitis supurativa cal history, physical examination, acute profi le was analysed in the SAPHO Folliculitis phase reactants, cultures and serological group, in those with chronic stable From ref.3 studies to rule out the presence of ha- plaque psoriasis, in PsA patients, and in bitual pathogens causing reactive arthri- 170 healthy blood donors from our gen- tis, type of skin lesions, osteoarticular eral population. The HLA distribution Table II. Demographic, clinical, radiologic, manifestations, form of onset, evolution was then compared between patients and treatment characteristics in a series of and response to treatment. Radiologi- and controls, and between the three 25 patients with SAPHO syndrome. cal study included chest, cervical and groups of diseases. This distribution Variable SAPHO n = 25 lumbar column, pelvis (with sacroiliac was also compared among the three ar- views), as well as projections of all af- ticular categories defi ned for PsA. Age (mean ± SD) 44 ± 8.6 fected joints and bones. When neces- The strength of the association between Gender ratio (female/male) 7/1 Disease duration (mean ± SD) 8.4 ± 6 sary, a CT scan, scintigraphy and/or antigens and disease was evaluated by Synchronous onset (%) 60 MRI was used to evaluate the extension odds ratios (OR), while the statistical Cutaneous fi rst (%) 32 of the joint/bone lesions. The presence signifi cance of the association was as- Osteoarticular fi rst (%) 8 of psoriasis and other extraskeletal man- sessed with a two-tailed Fisher’s exact PPP (%) 80 Pustular psoriasis (%) 12 ifestations associated with the group of test. P < 0.05 values were considered Psoriasis vulgaris (%) 8 SpA was also recorded. Autoimmunity signifi cant. Acne (%) 8 tests included RF, ANA, immunoglobu- All patients were informed about the ob- Hidradenitis suppurativa (%) 4 lins and complement proteins. jectives of the study and informed con- Onychopathy (%) 4 Chest wall involvement (%) 48 In order to test the hypothesis that pa- sent sheets were obtained. The ethical Synovitis (%) 60 tients with SAPHO syndrome may dif- committee of our institution approved Sacroiliitis (%) 20 fer from those suffering psoriasis and the fi nal version of the present study. Discitis (%) 8 PsA, 50 patients with chronic stable Sterile osteitis/osteomyelitis (%) 8 Hyperostosis (%) 40 plaque psoriasis and 120 with PsA were Results NSAIDs use (%) 72 randomly selected from the general da- From 1985 to 2005, 25 patients fulfi lled Antibiotic use (%) 12 tabase of the Dermatology and Rheu- the criteria proposed by Chamot et al. DMARDs use (%) 32 matology departments of our hospital. for the SAPHO syndrome (3). The main Biphosphonate use (%) 12 Anti-TNF alfa use (%) 4 Patients with PsA were divided into clinical, radiological, and demographic Corticosteroid use (%) 20 disease subgroups, as originally cited features, as well as the strategies used by Moll and Wright (6), however as to treat these patients, are shown in See text for a more detailed explanation. PsA patients may change their articular Table II. Most patients were middle- PPP: palmo-plantar pustulosis; DMARDs: Dis- pattern with time, this cohort was divid- aged women (7/1). The main cutane- ease Modifi ying Anti-Rheumatic Drugs.
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