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Modern Rheumatology Jul 2022Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis (SAPHO) syndrome is a rare inflammatory osteoarticular disorder, which encompassed many diseases, including pustulotic... (Review)
Review
Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis (SAPHO) syndrome is a rare inflammatory osteoarticular disorder, which encompassed many diseases, including pustulotic arthro-osteitis (PAO). Musculoskeletal manifestations, including osteitis, synovitis, and hyperostosis, are the hallmarks of the SAPHO syndrome and affect a variety of regions of the body. Recent survey indicated that more than 80% of cases of SAPHO syndrome in Japan were PAO, originally proposed by Sonozaki et al. in 1981, whereas severe acne was the most commonly reported skin ailment amongst participants with SAPHO syndrome in Israel. Prevalence of SAPHO syndrome remains unavailable, whereas the prevalence of palmoplantar pustulosis (PPP) was reported to be 0.12% in Japan, and 10-30% of patients with PPP had PAO. SAPHO syndrome and PAO are predominantly found in patients in the third through fifth decades of life, and a female predominance is seen in both groups. The diagnosis is typically made by a rheumatologist or dermatologist. Identification of a variety of the clinical, radiological, and laboratory features outlined, as well as diagnostic criteria, are used to make the diagnosis. Goals of treatment seek to maximize health-related quality of life, preventing structural changes and destruction, and normalizing physical function and social participation. Finally, we review the non-pharmacological and pharmacological managements.
Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Chronic Disease; Female; Humans; Hyperostosis; Male; Osteitis; Psoriasis; Quality of Life; Rare Diseases; Skin Diseases, Vesiculobullous; Synovitis
PubMed: 34967407
DOI: 10.1093/mr/roab103 -
Radiologic Clinics of North America Jul 2022Paget's disease is a metabolic bone disorder affecting the elderly and characterized by bone resorption followed by compensatory bone formation. Radiography is the... (Review)
Review
Paget's disease is a metabolic bone disorder affecting the elderly and characterized by bone resorption followed by compensatory bone formation. Radiography is the imaging modality of choice for the diagnosis whereas bone scintigraphy helps stage the extent of the disease and assess response to treatment. MRI and CT are important imaging methods in the assessment of complications and surgical planning. Osteolytic lesions of Paget's first phase present with well-defined margins on radiographs, most commonly in the femur, pelvis, and skull. Cortical thickening, trabecular coarsening, bone marrow sclerosis, and deformities of long bones are present in the mixed- and late-sclerotic phases.
Topics: Aged; Bone Marrow; Bone and Bones; Humans; Magnetic Resonance Imaging; Osteitis Deformans; Radiography
PubMed: 35672089
DOI: 10.1016/j.rcl.2022.02.005 -
SAPHO syndrome: pathogenesis, clinical presentation, imaging, comorbidities and treatment: a review.Postepy Dermatologii I Alergologii Dec 2021Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a constellation of dermatological and osteoarticular symptoms. The pathogenesis of SAPHO is... (Review)
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.
PubMed: 35125997
DOI: 10.5114/ada.2020.97394 -
Nature Reviews. Rheumatology Dec 2023Since the original description of spondyloarthritis 50 years ago, results have demonstrated similarities and differences between ankylosing spondylitis (AS) and axial... (Review)
Review
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.
Topics: Humans; Arthritis, Psoriatic; Spondylitis, Ankylosing; Osteitis; HLA-B27 Antigen; Ligaments
PubMed: 37919337
DOI: 10.1038/s41584-023-01038-9 -
Endocrinology and Metabolism (Seoul,... Oct 2022Paget's disease of the bone is a prevalent bone disease characterized by disorganized bone remodeling; however, it is comparatively uncommon in East Asian countries,...
Paget's disease of the bone is a prevalent bone disease characterized by disorganized bone remodeling; however, it is comparatively uncommon in East Asian countries, including China, Japan, and Korea. The exact cause still remains unknown. In genetically susceptible individuals, environmental triggers such as paramyxoviral infections are likely to cause the disease. Increased osteoclast activity results in increased bone resorption, which attracts osteoblasts and generates new bone matrix. Fast bone resorption and formation lead to the development of disorganized bone tissue. Increasing serum alkaline phosphatase or unique radiographic lesions may serve as the diagnostic indicators. Common symptoms include bone pain, bowing of the long bones, an enlarged skull, and hearing loss. The diagnosis is frequently confirmed by radiographic and nuclear scintigraphy of the bone. Further, bisphosphonates such as zoledronic acid and pamidronate are effective for its treatment. Moreover, biochemical monitoring is superior to the symptoms as a recurrence indicator. This article discusses the updates of Paget's disease of bone with a clinical case.
Topics: Humans; Osteitis Deformans; Diphosphonates; Pamidronate; Bone and Bones; Bone Resorption
PubMed: 36327984
DOI: 10.3803/EnM.2022.1575 -
Deutsches Arzteblatt International Oct 2021
Topics: Humans; Neoplasms; Osteitis Fibrosa Cystica
PubMed: 35020584
DOI: 10.3238/arztebl.m2021.0071 -
JAMA Sep 2021
Topics: Deafness; Famous Persons; History, 18th Century; History, 19th Century; Music; Osteitis Deformans; Otosclerosis; Syphilis
PubMed: 34546309
DOI: 10.1001/jama.2020.18134 -
Ear, Nose, & Throat Journal Mar 2022Eosinophilic chronic rhinosinusitis (ECRS) is an allergic inflammatory disease characterized by chronic inflammation of the sinus mucosa, and sometimes, osteitis. This...
OBJECTIVES
Eosinophilic chronic rhinosinusitis (ECRS) is an allergic inflammatory disease characterized by chronic inflammation of the sinus mucosa, and sometimes, osteitis. This study aimed to investigate the pattern of osteitis in ECRS and the relationship between bony thickening of the middle turbinate and recurrence of ECRS.
METHODS
A total of 246 patients with paranasal diseases were included in the study. The patients' data on bone thickening level, mucosal thickening, polyp score, clinical severity, and laboratory data were retrospectively evaluated.
RESULTS
In total, 38, 186, and 22 patients had ECRS, non-ECRS (NECRS), and odontogenic sinusitis, respectively. The Lund-Mackey (LM) score and Global Osteitis Scoring Scale (GOSS) scores in patients with ECRS were higher than those in patients with other paranasal diseases. There was a significant positive correlation between the GOSS score and ECRS clinical disease severity. Postoperative recurrence was significantly increased in patients with ECRS associated with bony thickening of the middle turbinate.
CONCLUSION
Both mucosal inflammation and osteitis were more severe in patients with ECRS than in patients with other diseases, and clinical disease severity was correlated with osteitis. Furthermore, the postoperative recurrence rate tended to increase in patients with ECRS who had bony thickening of the middle turbinate.
PubMed: 35353655
DOI: 10.1177/01455613221083793