This review provides an overview of SAPHO (synovitis, acne, impetigo, osteohyperplasia, and osteitis), a rare autoinflammatory disease that primarily affects bones, **, and joints. We searched Medline Pubmed using keywords such as Sapho syndrome, chronic ** multifocal osteitis, osteomyelitis, and related terms. Sapho syndrome is rare, with a reported incidence of 1 in 10,000 Caucasian people. However, the actual incidence of Sapho syndrome is unknown, and the incidence of this disease may be higher. The pathogenesis of Sapho syndrome is still not fully understood. Current evidence suggests that SAPHO is the result of a complex interplay between immune dysregulation, genetic predisposition, and environmental factors.
It is unclear whether Sapho syndrome is an autoimmune or autoinflammatory disease, but current evidence suggests that it is more likely to be an autoinflammatory disease due to factors such as overactive neutrophils, decreased natural killer (NK) cells, and elevated levels of interleukin (IL)-1. And it has a good response to blocking IL-1. Osteoarticular (OA) involvement is a key clinical feature of SAPHO. It affects the anterior thoracic wall, axial bones, peripheral joints, mandibles, long bones of the extremities, and pelvis. **Disease involvement is a common target for SAPHO, with lesions observed in 60-90% of cases. Common lesions include psoriasis and acne, and less commonly, hidradenitis suppuratura and neutrophilic disease. Other clinical manifestations include systemic symptoms due to systemic inflammation, such as fever, weight loss, and fatigue. There are no specific laboratory findings for Sapho syndrome.
However, during active disease, positive acute-phase markers such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complement levels, mild leukocytosis, and thrombocytosis may increase. Diagnosis is essential for SAPHO syndrome, which lacks specific diagnostic findings and is often underrecognized. A comprehensive evaluation of the patient's history and physical examination is essential. **Options include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, conventional and synthetic disease modifiers (CDMARD and SDMARD), biological**, bisphosphonates, and antibiotics. Biological** has emerged as a viable alternative for patients with SAPHO who do not respond to conventional**.
Sapho syndrome, an abbreviation for synovitis, acne, pustular lesions, hyperostosis, and osteitis, is a rare, complex, chronic inflammatory rheumatic disease that is often **. First discovered in the 80s of the 20th century, Sapho syndrome has been associated with a variety of presentations, including acne-related arthritis and sternoclavicle hypertrophy (SCH). The syndrome shares clinical and radiological features with certain childhood disorders, such as chronic multifocal osteomyelitis (CRMO), jaw osteomyelitis, and Kohler's disease, which are considered variants. Although SAPHO syndrome is a rare disease, it has received increasing attention in clinical and research areas due to the great challenges it poses to clinicians' diagnosis and **. The different clinical manifestations and tendencies of the syndrome make it particularly difficult.