Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis Research Articles

Pediatric SAPHO syndrome with pleural effusion: case report of a unique finding in a rare disease.

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare autoinflammatory disease characterized by bone inflammation and skin manifestations including acne, palmoplantar pustulosis, psoriasis, or hidradenitis suppurativa. SAPHO syndrome is considered on the same spectrum as chronic nonbacterial osteomyelitis/chronic recurrent multifocal osteomyelitis (CNO/CRMO), the former often being the nomenclature in adults and the latter in children. The diagnosis is made on patterns of clinical manifestations and is a diagnosis of exclusion. While skin and bone manifestations are commonly described with SAPHO syndrome, pleural involvement is rare, and few cases have been described in the literature, especially in pediatric patients. Herein we present a 14-year-old female with a past medical history of hidradenitis supprtiva, eczema, psoriasis, and a prior episode of culture-negative osteomyelitis who presented to the emergency room with chief complaints of right sided pain with inspiration and back pain. Exam revealed palmoplantar pustulosis, hidradenitis supprativa, psoriasis, and tenderness of vertebrae. Imaging showed a right sided pleural effusion and multiple sites of osteitis. Laboratory evaluation revealed elevated inflammatory markers, an exudative pleural effusion with neutrophilic predominance, and no evidence of malignancy, infection, or immunodeficiency. The patient was diagnosed with SAPHO syndrome and treated with naproxen, methotrexate, and golimumab with significant improvement including resolution of the pleural effusion. Pediatric SAPHO syndrome is a rare disease that classically causes osteitis and skin manifestations. This case highlights that pleural effusion can be a rare manifestation of pediatric SAPHO syndrome. Patients with suspected SAPHO syndrome with respiratory symptoms should be evaluated for pleural effusion.

Is JAK effective in treating recurrent SAPHO syndrome? TwHF might be a good choice.

Recently, JAKi has also been widely proved to be an effective alternative to conventional treatment for Synovitis acne pustulosis-hyperostosis-osteitis (SAPHO) cases, after failure of multiple drugs including those described above. But what to do when all these treatments fail? We report a case of remission from Tripterygium wilfordii Hook (TwHF) treatment. The patient was treated with nonsteroidal anti-inflammatory drugs, oral prednisone, minocycline, bisphosphonate injection, etanercept, and tofacitinib, but the symptoms did not change significantly. Treatment with TwHF (1.0 mg/kg/day, patient weight 60 kg) was started for 24 weeks. After 50 months of unsatisfactory treatment, this patient was finally treated with herbal TwHF, and after 6 months of treatment, the patient's magnetic resonance imaging and inflammatory indexes were significantly improved, indicating that the disease had been better controlled. In this study, TwHF was successful in treating a patient with refractory SAPHO syndrome who was refractory to multiple Western medications without significant adverse effects or toxicities, but further follow-up is needed to determine long-term efficacy. More case reports as well as clinical trials are still needed to confirm whether TwHF can effectively treat refractory SAPHO syndrome.

Chronic Nonbacterial Osteomyelitis, Hidradenitis Suppurativa, and SAPHO Syndrome

Introduction: Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome may represent one side of a spectrum of disease that also includes chronic nonbacterial osteomyelitis. Case: A 23-year-old male with bone pain in the anterior chest, shoulder, clavicles, left elbow, and left ankle presented to rheumatology clinic after multiple evaluations for infectious osteomyelitis. Imaging and deep bone biopsies of affected areas were consistent with chronic osteomyelitis, with labs notable for elevated erythrocyte sedimentation rate, C-reactive protein, and positivity for HLA-B27. A full skin exam was consistent with hidradenitis suppurativa, and he was started on infliximab and methotrexate with improvement in both his osteoarticular and skin symptoms. Conclusions: Patients presenting with features of follicular occlusion or neutrophilic dermatoses in conjunction with bone pain should be evaluated for SAPHO/CNO. In patients with SAPHO/CNO with vertebral involvement, bisphosphonates in addition to anti-inflammatory medications (such as methotrexate and TNF-inhibitors) can be effective.

Anterior Chest Wall Non-traumatic Arthropathies: A Crucial but Often Overlooked Site.

The purpose of this study was to describe the distribution of Anterior Chest Wall (ACW) arthropathies in a tertiary care center and identify clinical, biological and imaging findings to differentiate osteoarthritis (OA) from non-osteoarthritis (N-OA) etiologies. Search from medical records from January 2009 to April 2022, including patients with manubriosternal and/or sternoclavicular and/or sternocostal joint changes confirmed by ultrasonography, computed tomography or magnetic resonance imaging. The final study group was divided into OA and N-OA subgroups. A total of 108 patients (34 males and 74 females, mean age: 47.3 ± 13 years) were included. Twenty patients had findings of OA, while 88 were diagnosed with N-OA pathologies. SpA was the most common etiology in the N-OA group (n = 75). The other N-OA etiologies were less common: rheumatoid arthritis (n = 4), Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome (n = 3), infectious arthritis (n = 3) and microcrystalline arthropathies (n = 3). Regarding the distinctive features, ACW pain was the inaugural manifestation in 50% of patients in OA group and 18.2% of patients in N-OA group (p = 0.003); high inflammatory biomarkers were more common in N-OA group (p = 0.033). Imaging findings significantly associated with OA included subchondral bone cysts (p < 0.001) and intra-articular vacuum phenomenon (p < 0.001), while the presence of erosions was significantly associated with N-OA arthropathies (p = 0.019). OA was independently predicted by the presence of subchondral bone cysts (p = 0.026). ACW pain is a common but often underestimated complaint. Knowledge of the different non-traumatic pathologies and differentiation between OA and N-OA etiologies is fundamental for appropriate therapeutic management.

SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis); A Case of Backache in an Adolescent in Pakistan

SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) syndrome is a group of rare auto-inflammatory disorders with a wide range of dermatological and osteoarticular manifestations. Different nomenclatures, like Chronic Recurrent Multifocal Osteoarthritis (CRMO) and Chronic Non-Bacterial Osteomyelitis (CNO), have been used in the past for the same disease, but SAPHO Syndrome has been the accepted terminology since 1990. An adolescent, 17 years of age, with a six-month history of inflammatory backache and fatigue, reported to a rheumatology clinic. His health assessment questionnaire- disability indexcame out to be two. Acne on physical examination raised inflammatory markers, and characteristic findings of SAPHO syndrome on bone scan suggest the disease. The patient was treated with an excellent response to bisphosphonates. The report highlights the importance of the evaluation of inflammatory backache in adolescents. SAPHO syndrome is an underdiagnosed disease. Therefore, more awareness of this disease among healthcare physicians is required.

Colitis induced by IL-17A-inhibitors

BackgroundInterleukin (IL)-17A is essential for intestinal mucosal integrity, contributing to the prevention of detrimental immunity such as infectious colitis and inflammatory bowel disease (IBD). Indeed, neutralization of IL-17A has been abandoned as a therapeutic principle in IBD because of increased disease activity. However, it is controversial whether IL-17A inhibitors increase the risk of developing colitis in patients who do not have underlying IBD. Here, we present two cases of different forms of colitis that occurred during treatment with two IL-17A inhibitors, secukinumab and ixekizumab.Case presentationsWe report the case of a 35-year-old female with SAPHO (synovitis–acne–pustulosis–hyperostosis–osteitis) syndrome who was admitted due to severe colitis with bloody diarrhea, fever, abdominal pain and weight loss after receiving secukinumab for 3 months as well as the case of a 41-year-old male with psoriatic arthritis who presented himself to the outpatient clinic with bloody stools, abdominal pain and nausea 5 months after changing his therapy from secukinumab to ixekizumab. In both patients, treatment with IL-17A-inhibitors was stopped and tumor necrosis factor inhibitors were started. Both patients recovered, are clinically stable and show no more signs of active colitis.ConclusionThe role of IL-17A inhibitors in the pathogenesis of infectious colitis and new-onset IBD is not fully understood and requires further research. Patients receiving IL-17A-inhibitor therapy should be carefully screened and notified of the possible side effects.

SAPHO Syndrome in Crohn’s Disease Successfully Treated with Ustekinumab: Case Report and Review of the Literature

The authors report a case of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome in a patient with Crohn’s disease (CD). SAPHO syndrome is a rare disease characterised by the association, even if not simultaneous, of joint and skin manifestations. A young patient with CD was admitted to the authors’ hospital for the onset of bloody diarrhoea, persistent chest pain, headache, and fever while on maintenance therapy with vedolizumab. At visit, sternocostoclavicular and temporomandibular joints were tender and painful. Magnetic resonance was performed, and showed bone oedema of involved joints, while ileocolonoscopy revealed ulcers in the transverse colon. At laboratory, tests marked phlogosis and Campylobacter jejuni infection was observed. A challenge in differential diagnosis arose: atypical drug-induced extraintestinal manifestations, reactive arthritis, or extraintestinal manifestation directly associated with intestinal flare? In relation to the patient’s age, the involved joints, and magnetic resonance findings, SAPHO syndrome was diagnosed. Systemic steroids were used with a rapid clinical improvement; vedolizumab was withdrawn and ustekinumab was started with sustained clinical response.

SAPHO Syndrome in Crohn’s Disease Successfully Treated with Ustekinumab: Case Report and Review of the Literature

The authors report a case of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome in a patient with Crohn’s disease (CD). SAPHO syndrome is a rare disease characterised by the association, even if not simultaneous, of joint and skin manifestations. A young patient with CD was admitted to the authors’ hospital for the onset of bloody diarrhoea, persistent chest pain, headache, and fever while on maintenance therapy with vedolizumab. At visit, sternocostoclavicular and temporomandibular joints were tender and painful. Magnetic resonance was performed, and showed bone oedema of involved joints, while ileocolonoscopy revealed ulcers in the transverse colon. At laboratory, tests marked phlogosis and Campylobacter jejuni infection was observed. A challenge in differential diagnosis arose: atypical drug-induced extraintestinal manifestations, reactive arthritis, or extraintestinal manifestation directly associated with intestinal flare? In relation to the patient’s age, the involved joints, and magnetic resonance findings, SAPHO syndrome was diagnosed. Systemic steroids were used with a rapid clinical improvement; vedolizumab was withdrawn and ustekinumab was started with sustained clinical response.

Diagnostic and therapeutic practices in adult chronic nonbacterial osteomyelitis (CNO)

BackgroundChronic nonbacterial osteomyelitis (CNO) is a rare, and impactful auto-inflammatory bone disease occurring in children and adults. Clinical care for CNO is challenging, as the condition lacks validated classification criteria and evidence-based therapies. This study aimed to map the current diagnostic and therapeutic practices for CNO in adults, as a first step towards a standardized disease definition and future consensus treatment plans.MethodsA primary survey was spread among global rheumatological/bone networks and 57 experts as identified from literature (May 2022), covering terminology, diagnostic tools (clinical, radiological, biochemical) and treatment steps. A secondary survey (sent to primary survey responders in August 2022) further queried key diagnostic features, treatment motivations, disease activity and treatment response monitoring.Results36 and 23 physicians completed the primary and secondary survey respectively. Diagnosis was mainly based on individual physician assessment, in which the combination of chronic relapsing-remitting bone pain with radiologically-proven osteitis/osteomyelitis, sclerosis, hyperostosis and increased isotope uptake on bone scintigraphy were reported indicative of CNO. Physicians appeared more likely to refer to the condition as synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome in the presence of joint and skin pathology. MRI was most frequently performed, and the preferred diagnostic test for 47%. X-rays were second-most frequently used, although considered least informative of all available tools. Typical imaging features reported were hyperostosis, osteitis, osteosclerosis, bone marrow edema, while degeneration, soft tissue calcification, and ankylosis were not regarded characteristic. Inflammation markers and bone markers were generally regarded unhelpful for diagnostic and monitoring purposes and physicians infrequently performed bone biopsies. Management strategies diverged, including indications for treatment, response monitoring and declaration of remission. Step-1 treatment consisted of non-steroidal anti-inflammatory drugs/COX-2 inhibitors (83%). Common step 2–3 treatments were pamidronate, methotrexate, and TNF-a-inhibition (anti-TNFα), the latter two regarded especially convenient to co-target extra-skeletal inflammation in SAPHO syndrome. Overall pamidronate and anti-TNFα and were considered the most effective treatments.ConclusionsFollowing from our survey data, adult CNO is a broad and insufficiently characterized disease spectrum, including extra-osseous features. MRI is the favoured imaging diagnostic, and management strategies vary significantly. Overall, pamidronate and anti-TNFα are regarded most successful. The results lay out current practices for adult CNO, which may serve as backbone for a future consensus clinical guideline.

Orphan Disease, Drugs, and Dentistry: A Cumulative Review

Orphan diseases also known as rare diseases (RDs) are typically hereditary in nature. According to World Health Organization, RDs have a prevalence of 1 in 1000 individuals. Few RDs such as Turner syndrome, Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO), Williams syndrome, neurofibromatosis type I, Gorlin–Goltz, Schimke immuno, and cherubism exhibit oral signs. The disease’s great complexity and relatively low occurrence necessitates a diagnostotherapeutic strategy based on the Integration of Several Specializations. Rare illnesses require a worldwide agreement about the planning of future interventions. Dentists are often the first medical professionals to recognize RDs because oral symptoms often appear several years before the corresponding systemic symptoms. The objective of this article is to showcase information about RDs and several activities undertaken over the past decade to address this worldwide issue.

Imaging of Osteoarticular Lesions in SAPHO Syndrome: A Case Report

Introduction: Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is an acronym for a variety of osteoarticular and dermatologic manifestations. Although the incidence and prevalence are probably underestimated, the syndrome is considered rare. Observation: A 45-year-old female patient, without any particular pathological history, presented with anterior chest pain, spinal pain and palmar pustulosis. The symptoms had been evolving for six months, in a context of apyrexia and conservation of the general state. The diagnosis of SAPHO syndrome was retained in view of the young age, the clinical presentation and the axial osteoarticular involvement on imaging. In addition, bone scintigraphy objectified the "bull's head sign". Discussion: The osteoarticular manifestations of SAPHO syndrome are characterized by osteosclerosis, hyperostosis, erosions, synovitis and synostosis. Some locations, especially the anterior chest wall, and some topographical associations are very suggestive. Clinical symptoms are non-specific, hence the interest of imaging. CT is the modality of choice to determine the extent of osteoarticular lesions. Magnetic resonance imaging has an important place. It allows to establish a correlation between the stage of the disease and the clinical symptoms. Conclusion: Knowledge of clinical manifestations and radiological features allows for early diagnosis and prevention of osteoarticular progression.

Bull's head sign in the scintigraphy of a young female with recurrent chest pain.

In this case we report a 38-year-old female patient with history of recurrent retrosternal chest pain lasting almost 5 years. Standard X-rays of chest and spine revealed no abnormalities. In a physical examination tenderness of anterior chest wall was observed, especially in sternoclavicular areas. SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome was taken into consideration despite of lack of typical skin lesions (acne, pustulosis). We decided to implement [99mTc]Tc-MDP scintigraphy. Increased osteoblastic activity (intense [99mTc]Tc-MDP) in manubriosternal and both sternoclavicular regions represents bull's head sign which is a rare finding, but pathognomonic to SAPHO syndrome. After a 3-month therapy with aceclofenac 100 mg, total remission was reached. If we rule out this rare condition like SAPHO based on lack of abnormalities in X-rays, the reason of symptoms could be still unrecognized. [99mTc]Tc-MDP scintigraphy is valuable to show even subclinical areas of involvement and to monitor treatment response in SAPHO syndrome. This case proved significant role of whole body scintigraphy to make diagnosis of SAPHO syndrome in patients with non-cardiac chest pain and lack of abnormalities in standard X-rays.

P43 A complicated road to diagnosis: Auto inflammatory bone disease

Introduction/BackgroundAuto inflammatory bone disorders are characterized by chronic non-infectious osteomyelitis and inflammation-induced bone resorption. It results from aberrant activation of components of the innate immune system. The auto inflammatory bone disorder includes chronic non-bacterial osteomyelitis (CNO) and its severe form chronic recurrent multifocal osteomyelitis (CRMO); synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome; Majeed syndrome; deficiency of interleukin-1 receptor antagonist (DIRA) and cherubism. The most common among this is CRMO/CNO. The disease can affect any part of the body but the metaphyseal region of the long bones, clavicle, and vertebra are the most commonly affected site.Description/MethodA 9 year old girl presented with five month history of swelling over right periocular and temporal region, associated with skin discoloration and difficulty in opening her eyes. She also reported headache more in the right frontal region. She had on and off exacerbations of skin discoloration and headaches. There was no history of fevers, weight loss, night sweats, skin lesions or arthritic symptoms. She did not have a personal or family history of inflammatory or autoimmune disorders. She is under ophthalmology elsewhere since the age of 3 months with a right divergent squint and history of right squint surgery at 6 years.Physical examination revealed swelling involving right infraorbital and temporal region, which was soft and not tender to touch with associated erythema in the same site. There were no signs of meningism or altered mental status. Musculoskeletal examination was unremarkable.Her blood test results showed mild increase in platelets (476 * 109), CRP 8mg/L, ESR (18mm/hr). IgG4 titres were mildly raised (1.16g/L). Other immunologic markers including rheumatoid factor, human leukocyte antigen B27, antinuclear antibody and anti-neutrophil cytoplasmic antibody were normal.Histopathological examination of bone and soft tissue of her right orbital rim showed low grade non-specific chronic inflammation with extensive appositional remodelling of bone suggestive of CRMO/SAPHO. No evidence of malignancy/infection.Later whole body MRI was performed to identify the extent of disease. MRI whole body was performed which showed multiple areas of bone marrow oedema and periostitis within the appendicular and axial skeleton, bilateral gluteal enthesitis and sacroilitis. There are some features that are atypical for CRMO (asymmetrical involvement, transphyseal involvement including epiphyseal involvement of the left humerus). Also noted to have right-sided periorbital oedema, oedema of the right masticator muscles and temporalis. Further planned to do dedicated MRI of left humerus with contrast.Discussion/ResultsIt can be difficult to consider what the problem is when a health care provider is presented with a group of seemingly disparate signs and symptoms with a history and time course that do not match classic (or commonly atypical) disease presentations. Common things happen commonly, so when symptoms occur without the usual co-occurring symptoms, unusual symptoms or time courses, it can be challenging.The current patient presented with periocular swelling with headache and skin discolouration. The differentials can be Langerhans cell histiocytosis, IgG4-related disease, osteoblastoma, and osteosarcoma. There were no hepatosplenomegaly, lymphadenopathy, also the mass was soft to palpate, and hence the above differentials were unlikely. Patient was initially evaluated at tertiary oncology centre and malignancy ruled out. The histopathology slides were reviewed and reported as mentioned above. In view of no skin manifestations, CRMO was the likely diagnosis considered. Hence MRI whole body performed.Barrani et al reported a similar type of unusual case of a 12-year-old girl with CRMO arising with recurrent episodes of left supraorbital headache, followed by the appearance of a periorbital dyschromia. Magnetic resonance imaging (MRI) of the skull and orbits revealed an important subacute inflammatory process. However, a few months later, the child presented a painful swelling of the left clavicle; the histological examination of the related biopsy allowed us to establish the diagnosis of CRMO.The follow up with our patient is quite a short duration and she might evolve later in the disease course giving more clarity!Key learning points/ConclusionIn evaluating a diagnostic dilemma, starting the laboratory evaluation helps to begin the process of trying to find an answer and can also help the families as “something is being done.”Chronic recurrent multifocal osteomyelitis can present with unifocal lesions, atypical locations or absence of recurrence.A high level of suspicion is paramount to avoid unnecessary biopsies and repeated antibiotic regimens.Families will often be quite concerned and support for them is also necessary and close communication and follow up is appropriate.

New Insights in the Treatment of SAPHO Syndrome and Medication Recommendations.

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare autoinflammatory disease characterized by dermatological disorders and osteoarticular inflammatory lesions. This article reviews the application of biologics and other treatments based on the therapeutic target and the size of molecules in SAPHO syndrome. We found that drugs, especially biologics, have different effects on bone, joint, and skin damage. This may relate to the different inflammatory pathways involved in the osteoarticular and cutaneous symptoms in SAPHO patients. In this study, we provide stratified medication recommendations for SAPHO syndrome. Patients with osteoarticular symptoms can consider tumor necrosis factor blockers, JAK inhibitor, interleukin (IL)-1 inhibitor, and IL-17 inhibitor. Patients with cutaneous symptoms should consider IL-17 and JAK inhibitors. Apremilast, Tripterygium wilfordii Hook F, and bisphosphonates are other effective treatments.

Cranial osteomyelitis associated with SAPHO syndrome: An underdiagnosed cause of chronic headache

AbstractA 50‐year‐old woman having palmoplantar pustulosis was admitted with a headache accompanied by occipital bulge. She had a history of cryptogenic cervical spondylitis which had not completely remitted despite treatment with nonsteroidal anti‐inflammatory drugs. Head magnetic resonance imaging revealed enhanced swelling in the soft tissues of the right occipital region corresponding to the bulging locus. Bone scintigraphy demonstrated increased uptake in the parieto‐occipital bone, cervical spine, and sternoclavicular joints. Considering the negative results of the culture tests, she was diagnosed with aseptic cranial osteomyelitis induced by synovitis‐acne‐pustulosis‐hyperostosis‐osteitis (SAPHO) syndrome; cervical osteitis might induce her unidentified spondylitis. This syndrome is unrecognized as a cause of secondary headache. Based on our review of previous reports and the current case, we suggest that a painful bulge, which is demonstrated as an enhanced swelling in the cephalic soft tissue, may be a diagnostic cue for SAPHO syndrome.

Pulmonary Vasculitis on Dual-Phase 18F-FDG PET/CT in SAPHO Syndrome.

Pulmonary vasculitis is an uncommon type of vasculitis that may cause pulmonary artery stenosis with ensuing ischemic damage to lung tissue. 99mTc-macroaggregated albumin scintigraphy and iodine lung perfusion mapping are useful imaging modalities for assessment of lung perfusion but are not useful for assessing disease activity. On the other hand, 18F-FDG PET/CT is widely used for assessment of vasculitis activity but cannot provide perfusion information. We reported the clinical utility of dual-phase 18F-FDG PET/CT for pulmonary vasculitis in patients with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome.

O13 A case of chronic recurrent multifocal osteomyelitis

O13 A case of chronic recurrent multifocal osteomyelitis

SAPHO Syndrome: An Unusual Cause of Dysphagia.

Case PresentationThis case describes a 51-year-old male who presented to the emergency department with a complaint of two weeks of progressively worsening dysphagia as well as the emergence of superficial fluid collections on the anterior chest and leg during the same period. Computed tomography showed retropharyngeal and paratracheal fluid collections with adjacent vertebral osteitis; however, biopsies were negative for any infectious or mycobacterial source, and instead showed chronic inflammatory changes.DiscussionSynovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare rheumatic disorder that presents with multifocal osteitis and sterile neutrophilia. SAPHO syndrome may be easily mistaken for a diffuse infectious process on initial evaluation and imaging; however, it is treated with anti-inflammatory medications, including non-steroidal anti-inflammatory drugs and corticosteroids. Although most patients achieve remission of symptoms with treatment, the location of the fluid collections and resultant bony destruction may be life-threatening if undiagnosed.

Recent advances in palmoplantar pustulosis.

Palmoplantar pustulosis (PPP) is a chronic inflammatory condition where crops of sterile pustules with erythematous keratotic lesions causing bleeding and pain appear on the palms and soles. Recently, the European Rare and Severe Expert Network considered PPP as a variant of pustular psoriasis with or without psoriasis vulgaris.The prevalence of PPP varies from 0.050 to 0.12%. PPP occurs more frequently in women and the highest prevalence occurred between the ages of 50 and 69 years. Nail psoriasis seems to be frequent in PPP, ranging from 30 to 76%, and psoriatic arthritis in 8.6 to 26% of PPP patients.Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome and pustulotic arthro-osteitis are considered PPP-associated disorders. PPP has been reported with other co-morbidities such as psychiatric disorders, thyroid-associated disease, altered calcium homeostasis, gluten sensitivity diabetes, obesity, and dyslipidemia, but larger studies are required to prove such associations. Environmental exacerbating factors might contribute to the onset or worsening of PPP such as cigarette smoking, stress, focal infections, metal allergies, and drug intake. Genetic predisposition plays an important role in PPP.In PPP, both the innate and the adaptive immune systems are activated. The acrosyringeal expression of IL-17 has been demonstrated, indicating that the eccrine sweat gland is an active component of the skin barrier and an immune-competent structure. Increased levels of several inflammatory molecules, including IL-8, IL-1α, IL-1β, IL-17A, IL-17C, IL-17D, IL-17F, IL-22, IL-23A, and IL-23 receptor, have been detected in PPP biopsies. Increased serum levels of TNF-α, IL-17, IL-22, and IFN-γ have been detected in patients with PPP in comparison to healthy subjects, suggesting a similar inflammatory pattern to psoriasis vulgaris. Oral and tonsillar infections serve as trigger factors for PPP.Long-term therapy is required for many patients, but high-quality data are limited, contributing to uncertainty about the ideal approach to treatment.

Establishing core domain sets for Chronic Nonbacterial Osteomyelitis (CNO) and Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO): A report from the OMERACT 2020 special interest group

ObjectiveA working group was established to develop a core domain set (CDS) for Chronic Nonbacterial Osteomyelitis (CNO) and Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) following the OMERACT filter 2.1. MethodsA scoping review to identify disease-related manifestations was performed, followed by a special interest group (SIG) session at OMERACT2020 to begin the CNO/SAPHO CDS framework. ResultsCandidate items were identified from the scoping review and most fell under Life Impact and Pathophysiology Manifestation core areas. A SIG agreed on the need to develop a CDS for CNO and SAPHO (100%) and for children and adults (91%). ConclusionBased on candidate items identified, qualitative research and Delphi surveys will be performed as next steps.