Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 2.899
Filtrar
2.
Dermatol Clin ; 42(2): 247-265, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38423685

RESUMO

Hidradenitis suppurativa (HS) is an autoinflammatory skin disorder of the terminal hair follicle, which can present in sporadic, familial, or syndromic form. A classification has been proposed for the latter, distinguishing cases associated with a known genetic condition, with follicular keratinization disorders or with autoinflammatory diseases. This review focuses on the clinical and genetic features of those entities (ie, pyoderma gangrenosum [PG], acne and HS; PG, acne, pyogenic arthritis and HS; psoriatic arthritis, PG, acne and HS; synovitis, acne, pustulosis, hyperostosis, osteitis; and so forth) for which the collective term HS-related autoinflammatory syndromes is proposed.


Assuntos
Acne Vulgar , Artrite , Hidradenite Supurativa , Hiperostose , Osteíte , Pioderma Gangrenoso , Sinovite , Humanos , Hidradenite Supurativa/diagnóstico , Hidradenite Supurativa/genética , Hidradenite Supurativa/terapia , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/genética , Pioderma Gangrenoso/terapia , Osteíte/complicações , Acne Vulgar/diagnóstico , Acne Vulgar/genética , Acne Vulgar/complicações , Síndrome , Sinovite/complicações , Hiperostose/complicações , Artrite/complicações
3.
J Med Case Rep ; 18(1): 57, 2024 Feb 16.
Artigo em Inglês | MEDLINE | ID: mdl-38360816

RESUMO

INTRODUCTION: The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare condition. Its treatment remains a challenge for clinicians, and often yields mixed results. CASE: We report the case of a 51-year-old Caucasian woman who presented with SAPHO syndrome with mainly axial involvement. She had been treated with sulfasalazine and anti-inflammatory drugs for many years without any success. A few weeks after starting treatment with tofacitinib, both clinical and biological parameters dramatically improved. Imaging also showed considerable regression of the vertebral and pelvic lesions. However, tofacitinib had to be discontinued due to the occurrence of pulmonary embolism. Consequently, recurrence of bone pain and biologic inflammation was rapidly observed. CONCLUSIONS: Anti-JAKs are an interesting treatment option in the management of SAPHO syndrome that need further clinical trials and assessment for validating response.


Assuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Hiperostose , Osteíte , Piperidinas , Pirimidinas , Sinovite , Feminino , Humanos , Pessoa de Meia-Idade , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico
5.
Diagn Microbiol Infect Dis ; 108(3): 116170, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38176301

RESUMO

BACKGROUND: Mediastinitis and sternal osteitis are critical complications in cardiac surgery. Cases of these complications caused by Mycoplasma hominis are extremely rare. CASE PRESENTATION: We present a case of mediastinitis and sternal osteitis caused by M. hominis infection following ascending aortic replacement surgery. Whole gene sequencing analysis suggested the genitourinary tract as the most likely source of this M. hominis infection. Successful infection control was achieved through a regimen of moxifloxacin treatment. Additionally, a notable correlation was observed between serum levels of interleukin-6 and M. hominis infection. CONCLUSIONS: The significance of M. hominis as a potential cause of postoperative infection in cardiac surgery is still not fully recognized. Special attention should be paid to patients with bacteriologically negative infections, as M. hominis should not be disregarded, despite its rarity.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Mediastinite , Infecções por Mycoplasma , Osteíte , Humanos , Mycoplasma hominis/genética , Mediastinite/diagnóstico , Mediastinite/tratamento farmacológico , Mediastinite/etiologia , Osteíte/diagnóstico , Osteíte/tratamento farmacológico , Osteíte/complicações , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/tratamento farmacológico , Infecções por Mycoplasma/diagnóstico , Infecções por Mycoplasma/tratamento farmacológico
6.
Exp Dermatol ; 33(1): e15016, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38284206

RESUMO

Pustulotic arthro-osteitis (PAO) is an infrequent condition, with its manifestation in children being even rare. Some reports propose an association between genetic variants and the onset of PAO. Currently, no definitive treatment protocol exists for paediatric patients with PAO. In this study, we present the paediatric case of PAO with an IL36RN variant who was successfully treated with tonsillectomy.


Assuntos
Osteíte , Psoríase , Tonsilectomia , Humanos , Criança , Osteíte/etiologia , Tonsilectomia/efeitos adversos , Psoríase/complicações , Interleucinas
8.
Int J Rheum Dis ; 27(1): e14878, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37592395

RESUMO

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) is a rare chronic inflammatory disease that develops in adults. We present a case of SAPHO syndrome in a 37-year-old male presenting with gradually worsening back and neck pain for a 7-year period. The episodes were preceded by a history of pustular skin eruptions, which first appeared on the upper trunk and then involved his face and were pustular and scarring. The purpose of presenting this case report from Iraq is to raise awareness about this rare condition, which is frequently misdiagnosed and under-recognized.


Assuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Osteíte , Sinovite , Masculino , Adulto , Humanos , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Sinovite/diagnóstico , Dor nas Costas/diagnóstico , Dor nas Costas/etiologia , Pele , Acne Vulgar/diagnóstico
9.
Int J Rheum Dis ; 27(1): e14880, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37694521

RESUMO

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is an uncommon clinical syndrome with the signs of skin problems and osteoarthropathy as its main features. The pathogenesis of SAPHO syndrome has not been fully elucidated, and multiple complications may be present, including thrombosis. A 39-year-old male patient was diagnosed with SAPHO syndrome, complicated by multiple venous thrombosis of the left lower limb. We conducted a brief review of the current available literature on thrombosis in patients with SAPHO syndrome and speculated that the presence of lower extremity thrombosis in this patient with SAPHO syndrome may be related to physiological structure or antiphospholipid syndrome. Whether positive lupus anticoagulant has an effect on thrombosis in patients with SAPHO syndrome remains to be investigated.


Assuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Síndrome Antifosfolipídica , Osteíte , Sinovite , Trombose Venosa , Masculino , Humanos , Adulto , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Sinovite/complicações , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/tratamento farmacológico , Trombose Venosa/etiologia , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Acne Vulgar/complicações
10.
Clin Rheumatol ; 43(1): 561-568, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37755548

RESUMO

OBJECTIVES: We aimed to analyze the clinical characteristics and outcomes of patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. METHODS: The clinical records of 64 patients with SAPHO syndrome were collected, and the treatment and outcomes of 27 patients were followed up. The patients were divided into three groups according to the site of bone lesions: only anterior chest wall (ACW) involvement, only spinal involvement, and bone lesion involvement at both sites. The clinical characteristics and outcomes were compared. The clinical characteristics of the patients with and without peripheral joint involvement were compared. RESULTS: Among all patients, 31.25% (20/64) had only ACW involvement, 15.63% (10/64) had only spinal involvement, and 53.12% (34/64) had both ACW and spinal involvement. Peripheral joint involvement was observed in 25.00% (16/64) of the patients. Patients with only spinal involvement were older than those with only ACW involvement (p = 0.006). Patients with both ACW and spinal involvement were older than those with only ACW involvement (p = 0.002) and had a longer diagnosis delay (p = 0.015). Patients with peripheral joint involvement were younger than those without peripheral joint involvement (p = 0.028). During follow-up, 88.89% (24/27) of patients had good outcomes. Twenty-two patients were treated with non-steroidal anti-inflammatory drugs + Iguratimod (IGU), and the outcomes of 90.91% (20/22) improved. CONCLUSIONS: A relationship may exist between the sites of bone lesions and clinical characteristics of patients with SAPHO syndrome. The clinical outcomes of these patients may be good, and IGU may be effective in treating SAPHO syndrome. Key Points • This study is the first long-term follow-up on the effectiveness of iguratimod in treating patients with SAPHO. • This study revealed that patients with SAPHO and different bone lesion sites may present with different clinical characteristics.


Assuntos
Síndrome de Hiperostose Adquirida , Doenças Ósseas , Osteíte , Humanos , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Síndrome de Hiperostose Adquirida/patologia , Estudos de Coortes , Osteíte/diagnóstico , Prognóstico
12.
RMD Open ; 9(4)2023 Dec 26.
Artigo em Inglês | MEDLINE | ID: mdl-38151265

RESUMO

BACKGROUND AND OBJECTIVES: Synovitis acne pustulosis hyperostosis osteitis (SAPHO) is a rare heterogeneous disease of unknown aetiopathology. Externally validated and internationally agreed diagnostic criteria or outcomes and, as a result, prospective randomised controlled trials in SAPHO are absent. Consequently, there is no agreed treatment standard. This study aimed to systematically collate and discuss treatment options in SAPHO. METHODS: Following 'Preferred Reporting Items for Systematic Reviews and Meta-Analyses' guidance, a systematic literature search was conducted using PubMed, Scopus and Web of Science databases. Prospective clinical studies and retrospective case collections discussing management and outcomes in SAPHO involving five or more participants were included. Articles not published in English, studies not reporting defined outcomes, and studies solely relying on patient-reported outcomes were excluded. RESULTS: A total of 28 studies (20 observational, 8 open-label clinical studies) reporting 796 patients of predominantly European ethnicity were included. Reported therapies varied greatly, with many centres using multiple treatments in parallel. Most patients (37.1%) received non-steroidal anti-inflammatory drugs alone or in combination. Bisphosphonates (22.1%), conventional (21.7%) and biological (11.3%) disease-modifying antirheumatic drugs were the next most frequently reported treatments. Reported outcomes varied and delivered mixed results, which complicates comparisons. Bisphosphonates demonstrated the most consistent improvement of osteoarticular symptoms and were associated with transient influenza-like symptoms. Paradoxical skin reactions were reported in patients treated with TNF inhibitors, but no serious adverse events were recorded. Most treatments had limited or mixed effects on cutaneous involvement. A recent study investigating the Janus kinase inhibitor tofacitinib delivered promising results in relation to skin and nail involvement. CONCLUSIONS: No single currently available treatment option sufficiently addresses all SAPHO-associated symptoms. Variable, sometimes descriptive outcomes and the use of treatment combinations complicate conclusions and treatment recommendations. Randomised clinical trials are necessary to generate reliable evidence.


Assuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Hiperostose , Osteíte , Sinovite , Humanos , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Síndrome de Hiperostose Adquirida/etiologia , Osteíte/complicações , Osteíte/diagnóstico , Osteíte/tratamento farmacológico , Estudos Retrospectivos , Estudos Prospectivos , Sinovite/tratamento farmacológico , Hiperostose/complicações , Hiperostose/tratamento farmacológico , Acne Vulgar/complicações , Acne Vulgar/tratamento farmacológico , Difosfonatos/uso terapêutico
13.
Nat Rev Rheumatol ; 19(12): 818-827, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37919337

RESUMO

Since the original description of spondyloarthritis 50 years ago, results have demonstrated similarities and differences between ankylosing spondylitis (AS) and axial psoriatic arthritis (PsA). HLA-B27 gene carriage in axial inflammation is linked to peri-fibrocartilaginous sacroiliac joint osteitis, as well as to spinal peri-entheseal osteitis, which is often extensive and which provides a crucial anatomical and immunological differentiation between the AS and PsA phenotypes. Specifically, HLA-B27-related diffuse bone marrow oedema (histologically an osteitis) and bone marrow fatty corners detected via magnetic resonance imaging, as well as radiographic changes such as sacroiliitis, vertebral squaring, corner erosions and Romanus lesions, all indicate initial bone phenotypes in HLA-B27+ axial disease. However, in much of PsA with axial involvement, enthesitis primarily manifests in ligamentous soft tissue as 'ligamentitis', with characteristic lesions that include para-syndesmophytes and sacroiliac joint bony sparing. Like axial PsA, diffuse idiopathic skeletal hyperostosis phenotypes, which can be indistinguishable from PsA, exhibit a thoracic and cervical spinal ligamentous soft-tissue tropism, clinically manifesting as syndesmophytosis that is soft-tissue-centric, including paravertebral soft-tissue ossification and sacroiliac soft-ligamentous ossification instead of joint-cavity fusion. The enthesis bone and soft tissues have radically different immune cell and stromal compositions, which probably underpins differential responses to immunomodulatory therapy, especially IL-23 inhibition.


Assuntos
Artrite Psoriásica , Osteíte , Espondilite Anquilosante , Humanos , Espondilite Anquilosante/diagnóstico , Espondilite Anquilosante/genética , Antígeno HLA-B27/genética , Ligamentos/patologia
14.
BMJ Case Rep ; 16(10)2023 Oct 13.
Artigo em Inglês | MEDLINE | ID: mdl-37832974

RESUMO

Osteitis pubis is a rare, inflammatory condition involving the pubic symphysis. While osteitis pubis has been reported following many urological procedures, including those addressing bladder outlet obstruction such as transurethral resection of the prostate, it has never been reported after holmium laser enucleation of the prostate (HoLEP). Here, we detail the clinical course of a patient found to have osteitis pubis following HoLEP. This patient presented several weeks after surgery with non-specific, persistent symptoms of groin pain and difficulty ambulating, alerting our clinicians to consider osteitis pubis which was confirmed on MRI of the pelvis. While the majority of osteitis pubis cases are managed with locally invasive techniques, our patient's symptoms were successfully managed conservatively with Foley catheter placement, oral antibiotics and close follow-up. At 9 months postoperative, the patient has reported complete resolution of symptoms and continues to be followed closely.


Assuntos
Artrite , Terapia a Laser , Lasers de Estado Sólido , Osteíte , Hiperplasia Prostática , Ressecção Transuretral da Próstata , Masculino , Humanos , Osteíte/diagnóstico por imagem , Osteíte/etiologia , Osso Púbico/diagnóstico por imagem , Próstata , Lasers de Estado Sólido/uso terapêutico , Artrite/cirurgia , Terapia a Laser/efeitos adversos , Resultado do Tratamento , Hiperplasia Prostática/cirurgia
17.
Pediatr. catalan ; 83(3): 114-117, Juliol - Setembre 2023. ilus, tab
Artigo em Catalão | IBECS | ID: ibc-227795

RESUMO

Introducció. L’osteomielitis crònica no bacteriana (OCNB)és una entitat poc freqüent en pediatria. Es tracta d’unamalaltia inflamatòria no infecciosa de l’os que sol cursaramb remissions i exacerbacions espontànies. Té una etiologia desconeguda. Sol presentar-se com un dolor ossisubagut, acompanyat o no de clínica sistèmica, i és necessari descartar altres causes, com la neoplàstica o la infecciosa. Les proves d’imatge donen suport al diagnòstic.Observació clínica. Es presenten dos casos de pacients ambdolor ossi insidiós a les extremitats inferiors de diversessetmanes d’evolució, amb alteració de la força i la mobilitat. Ambdós casos s’associen a anorèxia. L’analítica presenta elevació dels paràmetres inflamatoris, VSG (velocitatde sedimentació globular) i PCR (proteïna C reactiva). Lagammagrafia òssia i la ressonància magnètica nuclear permeten fer el diagnòstic d’OCNB. En ambdós casos es fatractament amb antiinflamatoris no esteroidals durant quatre setmanes, i es requereix l’addició de bifosfonats a causad’una resposta parcial.Comentaris. L’OCNB és una causa d’inflamació òssia en elsinfants en què un diagnòstic precoç i la instauració d’untractament efectiu permet evitar complicacions. És important tenir-la en compte en fer el diagnòstic diferencial deldolor ossi insidiós. Un coneixement millor en pot disminuirl’infradiagnòstic. (AU)


Introducción. La osteomielitis crónica no bacteriana (OCNB) esuna entidad poco frecuente en pediatría. Se trata de una enfermedad inflamatoria no infecciosa del hueso que suele cursar conremisiones y exacerbaciones espontáneas. Su etiología es desconocida. Suele presentarse como un dolor óseo subagudo, acompañado o no de clínica sistémica, y es necesario descartar otrascausas como la neoplásica o la infecciosa. Las pruebas de imagenapoyan el diagnóstico.Observación clínica. Se presentan dos casos de pacientes con doloróseo insidioso en extremidades inferiores de varias semanas de evolución con alteración de la fuerza y la movilidad. Ambos casosse asocian a anorexia. A nivel analítico presentan elevación de losparámetros inflamatorios, VSG (velocidad de sedimentación globular) y PCR (proteína C reactiva). La gammagrafía ósea y la resonancia magnética nuclear permiten realizar el diagnóstico de OCNB.En ambos casos se realiza tratamiento con antiinflamatorios noesteroideos durante cuatro semanas, requiriendo la adición de bifosfonatos debido a respuesta parcial.Comentarios. La OCNB es una causa de inflamación ósea en losniños en la que un diagnóstico precoz y la instauración de untratamiento efectivo permite evitar complicaciones. Es importantetenerla en cuenta al realizar el diagnóstico diferencial del doloróseo insidioso. Un mayor conocimiento puede disminuir su infradiagnóstico. (AU)


Introduction. Chronic non-bacterial osteomyelitis (CNBO) is a rarecondition in pediatrics. It is a non-infectious inflammatory diseaseof the bone that usually results in spontaneous remissions andexacerbations. Its etiology is unknown. It usually presents as subacute bone pain, with or without systemic signs and symptoms. Itis often necessary to rule out other causes such as neoplastic orinfectious. Imaging tests support the diagnosis.Clinical observation. We present two cases of patients with insidious bone pain in the lower extremities of several weeks of evolution with impaired strength and mobility. Both cases were associated with anorexia nervosa. Laboratory evaluation showed elevatedinflammatory parameters, erythrocyte sedimentation rate andC-reactive protein. Bone scintigraphy and nuclear magnetic resonance imaging allowed the diagnosis of CNBO. In both cases,treatment with nonsteroidal anti-inflammatory drugs was performed for 4 weeks, requiring the addition of bisphosphonates due topartial response.Comments. CNBO is a cause of bone inflammation in children inwhich early diagnosis and effective treatment can prevent complications. It is important to keep this entity in mind when makingthe differential diagnosis of insidious bone pain. Greater knowledgemay decrease its underdiagnosis. (AU)


Assuntos
Humanos , Feminino , Pré-Escolar , Criança , Osteomielite/diagnóstico por imagem , Osteomielite/terapia , Dor Crônica , Osteíte/diagnóstico por imagem , Osteíte/diagnóstico , Osteíte/terapia , Anti-Inflamatórios não Esteroides/administração & dosagem , Anti-Inflamatórios não Esteroides/uso terapêutico , Difosfonatos/uso terapêutico
18.
Clin Implant Dent Relat Res ; 25(6): 1091-1102, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37574767

RESUMO

OBJECTIVES: To investigate the effects of zygomatic implant placement on the maxillary sinus using radiographic and clinical indicators. METHODS: Patients with an atrophic maxilla who underwent zygomatic implant placement were included. The thickness and morphology of the Schneiderian membrane (SM), infundibular obstruction, and posterior bone wall of the maxillary sinus were analyzed. The generalized estimating equation and chi-square tests were performed to compare the measurements. RESULTS: Fifty patients with 100 maxillary sinuses were included. In total, 148 zygomatic implants and 105 regular implants were placed in the maxilla. Overall, the mean pre- and postoperative SM thickness was 2.79 ± 3.26 mm and 3.97 ± 5.45 mm, respectively (p = 0.063). In sinuses with two zygomatic implants, the SM thickness increased significantly from 2.12 ± 2.14 mm preoperatively to 4.07 ± 6.14 mm postoperatively (p = 0.026). The number of sinuses with type IV morphology (fully radiopaque) increased from zero preoperatively to six (13%) postoperatively. Sinuses with a single zygomatic implant showed no difference in the pre- and postoperative SM thickness. Postoperatively, six sinuses had infundibulum obstructions. Postoperative osteitis of the bilateral sinuses was found in two patients. CONCLUSIONS: We have proposed a new imaging evaluation method and system for evaluating the maxillary sinus response. Preoperative infundibulum obstruction combined with mucosal thickening and double zygomatic implant placement are more likely to induce postoperative maxillary sinus mucositis and osteitis.


Assuntos
Implantes Dentários , Osteíte , Humanos , Seio Maxilar/diagnóstico por imagem , Seio Maxilar/cirurgia , Implantes Dentários/efeitos adversos , Seguimentos , Osteíte/induzido quimicamente , Osteíte/cirurgia , Mucosa Nasal/cirurgia , Maxila/cirurgia , Zigoma/diagnóstico por imagem , Zigoma/cirurgia , Implantação Dentária Endóssea/métodos
19.
Pediatr Rheumatol Online J ; 21(1): 89, 2023 Aug 24.
Artigo em Inglês | MEDLINE | ID: mdl-37620906

RESUMO

BACKGROUND: Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by chronic vasculitis involving small to medium sized arteries, granulomatous inflammation of the upper and lower respiratory tracts, pauci-immune necrotizing glomerulonephritis, as well as vasculitis of other organs. Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory syndrome characterized by sterile bone inflammation. CASE PRESENTATION: We report a case of CRMO that was doing well on non-steroidal anti-inflammatory drugs (NSAID for 6 years and then developed ANCA positive limited GPA presenting with pyoderma gangrenosum, persistent bilateral otalgia with serous otitis, otorrhea, then sensorineural hearing loss. CONCLUSION: This is the first report of limited GPA initially presenting as pyoderma gangrenosum in a patient with underlying CRMO. It is unclear how the pathology of an autoimmune and an autoinflammatory condition can overlap.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Osteíte , Osteomielite , Pioderma Gangrenoso , Criança , Humanos , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Osteomielite/complicações , Osteomielite/diagnóstico , Anti-Inflamatórios não Esteroides
20.
Orphanet J Rare Dis ; 18(1): 206, 2023 07 21.
Artigo em Inglês | MEDLINE | ID: mdl-37480122

RESUMO

BACKGROUND: Chronic 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. METHODS: A 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. RESULTS: 36 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. CONCLUSIONS: Following 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.


Assuntos
Síndrome de Hiperostose Adquirida , Hiperostose , Osteíte , Osteomielite , Criança , Adulto , Humanos , Osteíte/diagnóstico , Osteíte/tratamento farmacológico , Pamidronato/uso terapêutico , Osteomielite/diagnóstico , Osteomielite/tratamento farmacológico , Osteomielite/patologia , Hiperostose/tratamento farmacológico , Inflamação
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...