Synovitis, Acne, Pustulosis, Hyperostosis And Osteitis Research Articles

Current and future advances in practice: SAPHO syndrome and chronic non-bacterial osteitis (CNO).

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare, underdiagnosed disease with a wide clinical spectrum. Sterile bone inflammation, predominantly of the anterior chest, and skin manifestations (palmoplantar pustulosis, psoriasis vulgaris and acne) are the key features of SAPHO, which shares certain similarities with SpA. SAPHO is closely related to paediatric chronic non-bacterial osteitis (CNO), a spectrum of autoinflammatory bone diseases. The aetiology of SAPHO is considered multifactorial based on a complex interplay of genetic, immune and infectious factors. Despite the increasing awareness of SAPHO/CNO, diagnostic delay is common, as validated classification and diagnostic criteria are lacking. Treatment of SAPHO represents a challenge and includes anti-inflammatory drugs, antibiotics, bisphosphonates, synthetic conventional DMARDs and off-label use of anti-cytokine biologics and Janus kinase inhibitors. This review summarizes the current diagnostic and practical treatment approach to SAPHO/CNO and highlights the ongoing research endeavours concerning the definition and validation of diagnostic criteria, core domains and treatment.

SAPHO syndrome in the mandible: A 17-patient-based experience

SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis) syndrome is a rare inflammatory bone disorder with a remarkably low incidence. The condition's impact on the mandible is exceptionally uncommon, often resulting in a high rate of misdiagnosis and an extended duration of illness. The objective of this study was to assess patients with SAPHO syndrome in the mandible in across various stages and to dissect their distinctive features, aiming to provide future clinical experience for the disease.Methods: A retrospective analysis was performed on a cohort of 17 patients diagnosed with SAPHO syndrome affecting the mandible at the Second Affiliated Hospital of Zhejiang University from January 2020 to March 2023. Data including clinical presentations, imaging characteristics, and laboratory results were collected.Results: The median age at disease onset was 25, with a diagnostic interval of 26 months. Notably, seven individuals were prepubescent (under the age of 14). Seven patients (41.18 %) exhibited polyostotic involvement, while eight patients (47.06 %) presented with dermatological manifestations either concurrently with or subsequent to the osseous lesions. Condyle involvement was identified in six patients (35.29 %), and bilateral mandibular affection was noted in an equivalent number. The majority of patients (sixteen patients, 94.12 %) reported symptomatic relief following treatment with nonsteroidal anti-inflammatory drugs (NSAIDs). Glucocorticoids were instrumental in managing severe pain and extreme limitations in mouth opening. For patients with refractory disease, tumor necrosis factor-alpha (TNF-α) inhibitors, Janus kinase (JAK) inhibitors and bisphosphonates were employed. Ultimately, effective pain management was achieved in the entire cohort.Conclusion: The diagnosis of SAPHO syndrome involving the mandible is exclusionary. It is important to improve diagnostic accuracy among oral and maxillofacial surgeons (OMFS), dentists, and rheumatologists to avoid unnecessary surgery and tooth extraction. TNF-α inhibitors, JAK inhibitors and bisphosphonates are recommended as third-line drugs.

Update on ocular manifestations of the main monogenic and polygenic autoinflammatory diseases.

Autoinflammatory diseases include disorders with a genetic cause and also complex syndromes associated to polygenic or multifactorial factors. Eye involvement is present in many of them, with different extent and severity. The present review covers ophthalmological lesions in the most prevalent monogenic autoinflammatory diseases, including FMF (familial Mediterranean fever), TRAPS (TNF receptor-associated periodic syndrome), CAPS (cryopyrin-associated periodic syndromes), Blau syndrome, DADA2 (deficiency of adenosine deaminase 2), DITRA (deficiency of the interleukin-36 receptor antagonist), other monogenic disorders, including several ubiquitinopathies, interferonopathies, and the recently described ROSAH (retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and headache) syndrome, and VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. Among polygenic autoinflammatory diseases, ocular manifestations have been reviewed in Behçet's disease, PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) syndrome, Still's disease and autoinflammatory bone diseases, which encompass CRMO (chronic recurrent multifocal osteomyelitis) and SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome.

SAPHO Syndrome Complicated by Myocardial Infarction

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome occasionally has rare cases of heart involvement. There is a case of a male patient with SAPHO syndrome complicated by myocardial infarction (MI), which discovered that myocardial infarction is also influenced by smoking and alcohol consumption. This case provided a reference for the clinical diagnosis and treatment of SAPHO syndrome and myocardial infarction.

SAPHO syndrome: current clinical, diagnostic and treatment approaches.

This review provides an overview of SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis), a rare autoinflammatory disease that primarily affects bones, skin, and joints. We conducted a search on Medline/PubMed using keywords such as SAPHO syndrome, chronic recurrent multifocal osteitis/osteomyelitis, and related terms. SAPHO syndrome is rare, with a reported frequency of 1 in 10,000 in the Caucasian population. However, the actual incidence of SAPHO syndrome is unknown, and the incidence of the disease is likely higher. The pathogenesis of SAPHO syndrome remains incompletely understood. Current evidence suggests that SAPHO results from a complex interplay between immune dysregulation, genetic susceptibility, and environmental factors. It's not clear if SAPHO syndrome is an autoimmune disease or an autoinflammatory disease, but current evidence suggests that it's more likely an autoinflammatory disease because of things like neutrophil hyperactivity, fewer natural killer (NK) cells, high levels of interleukin (IL)-1, and a good response to treatments that block IL-1. Osteo-articular (OA) involvement is a key clinical feature of SAPHO. It affects the anterior chest wall, axial skeleton, peripheral joints, mandible, long bones of the extremities, and pelvis. Dermatological involvement is a common target in SAPHO, with lesions observed in 60-90% of cases. Common skin lesions include psoriasis and acne, with hidradenitis suppurativa and neutrophilic dermatoses being less commonly seen. Other clinical findings include constitutional symptoms caused by systemic inflammation, such as fever, weight loss, and fatigue. There is no specific laboratory finding for SAPHO syndrome. However, during active disease, there may be an increase in positive acute phase markers, such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complement levels, mild leukocytosis, and thrombocytosis. Diagnosis is crucial for SAPHO syndrome, which lacks a specific diagnostic finding and is often underrecognized. A comprehensive evaluation of a patient's medical history and physical examination is crucial. Treatment options include non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, conventional and synthetic disease-modifying agents (cDMARDs and sDMARDs), biological therapies, bisphosphonates, and antibiotics. Biological treatments have emerged as a viable alternative for SAPHO patients who do not respond to conventional treatments.

A Bull’s Head Sign in SAPHO

A syndrome of synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) is a rare autoimmune disease. It occurs in genetically predisposed individuals by immune activation triggered by possible infectious agents. A 60-year-old man was presented with musculoskeletal and cutaneous manifestations. After exclusion of differential diagnoses, he was diagnosed with SAPHO and successfully treated with adalimumab. He had a typical image defined as a bull’s head sign in bone scintigraphy with 99mTcmethylene diphosphonate. Positron emission tomography also revealed increased inflammatory activity in related anterior chest joints and soft tissue. We want to emphasize diagnostic radiological images in patients with SAPHO to increase the awareness of clinicians.

Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Juvenile Spondyloarthritis (JSpA): To What Extent Are They Related?

Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disease occurring mainly in the pediatric age group (before 16 years) and generally presents as a separate entity. Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome combines osteoarticular and cutaneous involvement, similar to CRMO, and falls into the spectrum of spondyloarthritis (SpA). The fact that a patient can progress from one disease to another raises the question of whether CRMO, like SAPHO, could fall within the spectrum of SpA, ranging from a predominantly osteoarticular form to an enthesitic form with more or less marked skin involvement. In this review, we set out to discuss this hypothesis by highlighting the differences and similarities between CRMO and juvenile SpA in clinical, radiological and pathophysiological aspects. A common hypothesis could potentially consider intestinal dysbiosis as the origin of these different inflammatory diseases. Interindividual factors such as gender, environment, genetics and/or epigenetic background could act as combined disease modifiers. This is why we suggest that pathophysiology, rather than clinical phenotype, be used to reclassify these diseases.

SAPHO Syndrome: About an Observation and Review of the Literature

Introduction: SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) is autoinflammatory rheumatism of the spondyloarthritis group. It is rare, it prevalence is 1/10000 inhabitants. The average age at diagnosis is 38 years, with no gender predominance, first described by Chamot and colleagues in 1987. Objective: To present observation and review of the literature on SAPHO syndrome which is rarely diagnosed in our daily practice. Observation: This was a 28 year old female patient, Senegalese, living in Dakar, followed in obstetrics gynecology for the desire of maternity, the family context of sickle cell disease who consulted for diffuse mechanical and articular inflammatory pains involving the proximal interphalangeal (IPP) of the left 5th toe, the left shoulder, the sternocostal and dorsolumbar joints evolving for 3 years. The clinical examination found: A painful swelling with a visual analog scale (VAS) of 6/10 of the right sternocostal joint and of the interphalangeal joint (IP) of the right hallux, diffuse acne, no palmoplantar pustulosis, no psoriasis, no gluteal or talalgia. The biology shows an inflammatory syndrome (VS at 37 mn at the 1st hour and CRP at 41mg/l), a decrease of 25-OH-Vitamin D3 at 22 ng/ml (N: 25-30 ng/ml), the positive latex test, Ac anti-CCP: 43 IU/ml (N < 5 IU/ml), positive Ac anti-HBc , Ac anti HBs > 1000 IU/l on the other hand blood calcium, blood phosphorus, G6PD, PTH, TSH us, free T4, anti ECT Ac, HLA-B27 Ag with no particularities. The scintigraphy showed hyper fixation of the right 1st toe, sacroiliac joints, dorsolumbar spine, left shoulder, manubrium sternal junction, and bilateral sternoclavicular junction realizing a classical bull horn image in SAPHO syndrome. Because of this clinical and paraclinical manifestation helped by the 1994 diagnostic criteria of Kahn at 1/3 (chronic joint involvement associated with severe acne), the SAPHO syndrome was retained and the treatment instituted after the normal pre-therapeutic analysis and systematic deworming was: Salazopyrine 2000mg/day (dosage reached by increments of 500 mg/week); Uvedose (vitamin D3 ) 100000 IU / month; bisphosphonates 70 mg /week and paracetamol + codeine 1g x 2/day as needed for 3 months with a favorable follow-up by the regression of signs (pain VAS at 2/10). Conclusion: The SAPHO syndrome is the prerogative of the young adult subject, bone scintigraphy is very valuable for its diagnosis, its better knowledge would avoid diagnostic and therapeutic erratic. Keywords: SAPHO syndrome, bone scan, Dakar, Senegal.

Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome with Cutis Verticis Gyrata: Case Report and Review of Literature

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare disease clinically characterized by a wide range of cutaneous and osteoarticular manifestations. Here, we report a case of SAPHO syndrome with cutis verticis gyrata (CVG) and investigated the genetic causes in the four members of this pedigree. After failure of conventional treatments, a recombinant human TNF-α receptor II:IgG Fc fusion protein (rhTNFR:Fc, YISAIPU®) achieved good control of the disease at the 2-year follow-up. We did not identify any pathogenic mutation in this pedigree. We also summarized the clinical and therapeutic characteristics of 83 patients with SAPHO syndrome through the China National Knowledge Infrastructure (CKNI) database from 2016 to 2021. Patients with acne were young and predominantly male. About 45.8% patients were treated with biological therapies or traditional Chinese medicine (TCM), 84.2% of which showed positive effects against cutaneous and osteoarticular manifestations. We report a case of SAPHO syndrome with CVG that was successfully treated with rhTNFR:Fc. Our results reveal the genetic heterogeneity involved. Biologics and TCM are likely alternative options for the treatment of SAPHO syndrome.

Off-label Use of Secukinumab: A Potential Therapeutic Option for SAPHO Syndrome

We read the recent article by Wang et al with great interest.1 The authors described a cohort of 4 patients with SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome who showed substantial improvement in skin lesions, clinical conditions, and whole-body magnetic resonance imaging before and after treatment with secukinumab without concomitant conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), nonsteroidal antiinflammatory drugs (NSAIDs), or other biologics, and suggested a potential benefit of secukinumab in the treatment of SAPHO syndrome. However, there are some details that need further clarification.

Dr. Li et al reply.

We appreciate Wang et al1 for their interest in our article2 and for highlighting the pathogenic role of cytokine dysregulation in SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome. The authors also address the possible mechanism of secukinumab in the treatment of SAPHO syndrome: blocking the expression of Th17 cell–related cytokines.

SAPHO Syndrome Mimicking Infectious Spondylodiscitis and Bone Metastasis

The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) includes diseases with similar osteoarticular manifestations and skin conditions. Making this diagnosis is not always obvious, especially when the clinical presentation does not fit the typical pattern of the disease or it occurs in a particular field. We described three cases where the diagnosis was difficult. A 46 year-old woman presented with cervical pain. The cervical X-ray showed the aspect of an ivory C5 vertebra. The patient had, however, preserved general condition, no signs of underlying neoplasia, nor other joint complaints. Blood analysis was normal. Tomography did not find any suspect lesion but showed sclerosis and hyperostosis of the manubrium. Scintigraphy showed the characteristic “bullhead” appearance. A 61-year-old woman had thoracic and lumbar pain. MRI showed spondylodiscitis in D3-D4, D4-D5, D5-D6, D6-D7, and L1-L2 with paraspinal soft tissue involvement, simulating infectious spondylodiscitis. Infectious investigations and discovertebral biopsy performed twice were negative. SAPHO syndrome was then suspected. Bone scintigraphy showed uptake in the chondrosternal articulations and D4 to D7 vertebrae. The diagnosis of SAPHO was established. The third case was a 46-year-old man with a lung adenocarcinoma. Staging for metastatic disease, a TAP tomography was performed and showed osteosclerosis of D8 to D12 and intra-articular bridges in the sacroiliac joints. MRI and scintigraphy eliminated malignancy and confirmed the diagnosis of SAPHO. In our cases, imaging findings could facilitate differentiating SAPHO syndrome from other diseases.

Clinical and Radiological Remission of Osteoarticular and Cutaneous Lesions in SAPHO Patients Treated With Secukinumab: A Case Series.

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare chronic inflammatory disease involving bone, joints, and skin1 No consensus has been reached on the treatment of SAPHO syndrome and the current options may lead to variable outcomes2.

Comparative analysis and differentiation between SAPHO syndrome and spondyloarthropathies using whole-spine MRI

To determine the imaging characteristics of SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome and seronegative spondyloarthropathies (SpAs) on whole-spine magnetic resonance imaging (WS-MRI) and evaluate the role of WS-MRI in the diagnosis and differentiation of the two diseases. Twenty-eight patients with SAPHO and 44 with SpAs were included. All patients were symptomatic and clinically diagnosed with SAPHO or SpAs, and all underwent WS-MRI for comparison of imaging characteristics. The mean age of the SAPHO patients was 48.7±12.7 years, while that of the SpA patients was 34.7±12.3 years (p<0.001). WS-MRI showed that the frequency of cervical, thoracic, and lumbar spine involvement was 53.6% versus 52.3%, 75% versus 88.6%, and 60.7% versus 63.6%, respectively (p=0.70, 0.13, and 0.80). The frequency of sacroiliac joint involvement was 7.1% and 100% (p<0.001). Continuous spinal involvement accounted for 50% versus 43.2%, 60.7% versus 84.1%, and 39.3% versus 40.9% in the cervical, thoracic, and lumbar vertebrae, respectively (p=0.03). WS-MRI showed that bone marrow oedema of spinal anterior corner was observed in 50% versus 75% (p=0.03). Vertebral body and posterior attachment involvement accounted for 85.7% versus 93.2% and 14.3% versus 34.1% (p=0.3, 0.06). The frequency of bone erosion in mobile spine was 75% and 36.4%, respectively (p=0.02). The frequency of intervertebral disc, endplate, anterior thoracic wall, and paraspinal soft-tissue swelling was 42.9% versus 18.2%, 53.6% versus 22.7%, 85.7% versus 42.2%, and 50% versus 11.4% (p=0.02, 0.00). Factors differentiating the two groups at WS-MRI were bone marrow oedema of the spinal anterior corner, bone erosion, and swelling of the intervertebral disc, endplate, anterior thoracic wall, and paraspinal soft-tissue.

Serum Sp17 Autoantibody Serves as a Potential Specific Biomarker in Patients with SAPHO Syndrome

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome shows a wide variability in musculoskeletal and cutaneous manifestations, and it is therefore underrecognized and misdiagnosed in the clinic due to a lack of specific markers. In this study, we aimed to identify specific biomarkers by screening serum autoantibodies in SAPHO patients with a 17K human whole-proteome microarray. The serum anti-Sp17 autoantibody was identified and verified to be a specific biomarker in patients with SAPHO syndrome. Indeed, the level of the anti-Sp17 autoantibody was significantly increased in patients with active SAPHO compared to patients with an inactive disease and healthy controls (P < 0.05). Additionally, serum anti-Sp17 autoantibody levels correlated with those of serum hypersensitive C-reactive protein (hsCRP), the erythrocyte sedimentation rate (ESR), and β-crosslaps (β-CTx) in patients with active SAPHO disease. Moreover, anti-Sp17 autoantibody levels were markedly decreased after anti-inflammatory treatment with pamidronate disodium, which downregulated levels of hsCRP and ESR in patients with active SAPHO. Thus, serum levels of the anti-Sp17 autoantibody might serve as a specific biomarker for the diagnosis of SAPHO syndrome or for monitoring the disease status.

Orofacial manifestations of SAPHO syndrome: a systematic review of case reports.

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a poorly known disease with cutaneous and osteo-articular manifestations requiring a multidisciplinary care. The aim of this study was to review the case reports that have described oral manifestations in patients suffering for this syndrome. A systematic review of case reports was performed on PubMed and Science Direct on January 2020 among all the articles dealing with the disease. In vitro, preclinical, and clinical studies have not been included to select only the case reports. Eighteen articles, published between 1999 and 2019, were included. All the patients presented mandibular osteomyelitis or sclerosis, associated with various other symptoms such as trismus, temporomandibular joint arthritis, or dysphagia. The data highlight the high variability in the disease's manifestations between people and also in the treatments applied. Knowing the orofacial signs of the SAPHO syndrome, the dental surgeon has a crucial role in the diagnosis procedure and must take place in the multidisciplinary medical team involved in the patient following. Some care adaptations are needed for oral interventions in these patients, depending on their treatments and their handicap. Key Points • Orofacial manifestations of SAPHO syndrome mainly occur on the mandible. • In cases of mandible sclerosis, decorticalization surgeries may be performed. • Oral care are encouraged, especially the preventive treatments to limit the necessity of surgeries. • The complexity in the management of patients suffering for a SAPHO syndrome concerns the oral manifestations, the patient general health and the treatments he has to take every day.

P194 Non-bacterial osteitis syndromes: characterising an adult population

Abstract Background Inflammatory bone syndromes, such as SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) and CRMO (chronic recurrent multifical osteomyelitis) are rare and have not been extensively described in the existing literature. Although there have been proposed classification and diagnostic criteria, there is still limited consensus and uniformity of approach in identifying and treating these patients. This may be due to a lack of data and understanding about the nature and prognosis of these conditions. Attempts to characterise, especially in adult populations, has been limited thus far. As a tertiary metabolic bone service with an allied regional sarcoma service, we present further data on these conditions in an adult population. Methods Retrospective analysis of notes of patients, with a diagnosis of SAPHO and CRMO, that were registered on a departmental database was performed. Data on demographics, symptom onset and character, serum and radiological tests and treatment were analysed. Results 77 adults were identified with a diagnosis of SAPHO and CRMO, with a 60:40 % split respectively. Female preponderance was noted with 74% of this cohort represented. SAPHO patients tended to present slightly later with a mean age of symptom onset of 37 years as compared to 24 years in the CRMO group. Diagnostic delay was evident with mean time to diagnosis averaging 34 months. Pain and bony swelling were the most common symptoms (99% and 39% respectively), with the clavicle, sternum and spine being the most common sites involved. Osteitis was a universal feature in both groups, whereas skin and inflammatory joint involvement was more of a feature of SAPHO patients (59% vs 39%). There is limited consensus on appropriate diagnostic tests. This was reflected in this cohort with variable testing of inflammatory markers, serological testing of HLA B27 and extent and modality of imaging. Non-steroidal anti-inflammatories (NSAIDS), intravenous bisphosphonates, steroids and anti-microbials were all employed as treatment options, although efficacy was difficult to establish in this retrospective analysis, a significant source of referrals were from the local Sarcoma Unit; this may have introduced a degree of bias in our results. Conclusion As rare conditions, promoting awareness, having an index of suspicion and early referral to specialist centres would help address current issues faced by this patient population. Several national and international disease registries are in development and will undoubtly help to standardise data collection in order to guide further work in this area. Future studies examining treatment efficacy in current as well as emerging biological therapies, such as anti-tumour necrosis factor, anti- interleukin 1 and 17 agents, would be of significant value. Disclosures T. Gill None. J. Bubbear None. R. Keen None.

Exosomal proteome from the serum, bone marrow, and palm and toe pustular skin tissues of a single patient with SAPHO syndrome

Exosome proteomic analysis may reveal differentially abundant proteins that are of significance for clarifying the pathogenesis of SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis) syndrome. Exosomes were isolated from the serum, bone marrow and skin tissue of the palm and toe pustular areas in a unique patient with SAPHO syndrome. The exosomes were not different from those of healthy subjects in size (114.1 ± 73.7 nm) or morphology. Label-free exosome proteomic analysis identified 198 more abundant proteins and 183 less abundant compared with those of healthy subjects. Gene ontology enrichment analysis revealed that these proteins were involved in binding with a variety of biological molecules and participated in biological processes related to autoimmunity or inflammation. A total of 243 KEGG (Kyoto Encyclopedia of Gene and Genomes) pathways were enriched, of which 43 were related to immune function. It was speculated that five differentially abundant proteins, Mitogen-activated protein kinase 1 (MAPK1/MK01), Tyrosine protein kinase (SYK), Integrin beta-3 (ITB3), Serine/threonine-protein phosphatase 2a catalytic subunit alpha isoform (PP2AA) and Serine/threonine-protein phosphatase 2a 65 kDa regulatory subunit A beta isoform (2AAB), associated with multiple KEGG pathways, forms an interaction network that may be involved in the occurrence, development and prognosis of SAPHO syndrome. SignificanceExosomes of SAPHO syndrome patient were not significantly different from those of healthy subjects in size and morphology. Label-free proteomic analysis of exosomal proteins in patient with SAPHO syndrome speculated 5 proteins MAPK1, SYK, ITB3, PP2AA and 2AAB, which may be involved in the occurrence, development and prognosis of SAPHO syndrome by binding with other biological molecules. It is speculated for the first time that proteins Histone H2A type 1-J and Histone H4 were related to SAPHO syndrome.Clinic relevance. Exosome proteomics can suggest novel pathological data in patients with SAPHO.

SAPHO syndrome: pathogenesis, clinical presentation, imaging, comorbidities and treatment: a review.

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a constellation of dermatological and osteoarticular symptoms. The pathogenesis of SAPHO is unknown, but infectious, genetic, immunological and environmental factors may play a role. SAPHO is classified along two different spectrums: pustulo-psoriatic hyperostotic spondyloarthritis and chronic recurrent multifocal osteomyelitis. The typical skin lesions are palmoplantar pustulosis and acne. The sign of arthritis is involvement of the anterior chest wall, most often the sternoclavicular joints. There are no standard treatment recommendations, but nonsteroidal anti-inflammatory drugs, sulfasalazine, methotrexate, antibiotics and biological drugs can be considered.

Systemic Amyloidosis with Renal Failure: A Challenging Diagnosis of SAPHO Syndrome

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare, unrecognized and chronic inflammatory disorder characterized by distinct cutaneous and osteoarticular manifestations. Renal complications are uncommon. We describe the unusual case of a patient with severe refractory and chronic hidradenitis suppurativa (HS) that progressed to chronic renal failure due to secondary amyloidosis, presenting with mandibular pain and renal failure. The challenging diagnosis of SAPHO syndrome was made. The purpose of this case report is to emphasize the need for vigilance, timely recognition and multidisciplinary treatment. The fundamental management of AA amyloidosis and SAPHO syndrome requires an individualized approach with control of the underlying inflammatory disease.LEARNING POINTSSystemic amyloidosis is a serious but rare complication of chronic inflammatory disorders such as hidradenitis suppurativa and should be periodically considered.SAPHO syndrome should be suspected in patients with cutaneous and osteoarticular manifestations, and is a challenging diagnosis with exclusion of neoplastic, autoimmune and infectious diseases.The cornerstone of management of AA amyloidosis is vigilance, timely recognition and sometimes aggressive immunosuppressive treatment.