Spinal and sacroiliac involvement in SAPHO syndrome: A single center study of a cohort of 354 patients

Therapeutic Advances in Musculoskeletal

et al. 2020

Self Cite

105

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a spectrum of heterogeneous diseases characterized by osteoarticular and dermatological manifestations. Osteitis and hyperostosis are core clinical manifestations in SAPHO syndrome, typically affecting multiple areas and possibly progressing to irreversible osteoarticular damage. Most patients with SAPHO have cutaneous involvement, mainly manifested as palmoplantar pustulosis and severe acne. Systemic manifestations are uncommon but occasionally reported. Epidemiological studies suggest the annual prevalence of SAPHO syndrome varies from 0.00144 in 100,000 in Japanese individuals to fewer than 1 in 10,000 in White individuals. The precise etiopathogenesis of SAPHO remains unclear, but it is generally considered an autoinflammatory syndrome that may be related to various etiologies, such as immune dysfunction, infection and genetic predisposition. Owing to the relapsing–remitting disease course, the goal of management is to improve clinical symptoms and prevent disease progression. Various treatments, including nonsteroidal anti-inflammatory drugs, conventional disease-modifying antirheumatic drugs, bisphosphonates, biologics, and antibiotics, are promising options for alleviating the disease.

Section: Clinical Manifestationsmentioning

confidence: 99%

Section: Clinical Manifestationsmentioning

confidence: 99%

Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update

Liu

Tang

Therapeutic Advances in Musculoskeletal

et al. 2020

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105

“…We recruited patients who had at least one follow-up between July, 2019 and Jan 2020 in our SAPHO syndrome cohort study. 9 Participation in the study was voluntarily, and written informed consent was obtained from each patient. Questionnaires were distributed digitally on March 3 rd , 2020, and were collected on March 5 th , 2020.…”

Section: Participantsmentioning

confidence: 99%

Concerns about disease management and psychological stress in SAPHO patients during the COVID-19 epidemic

Zhang

et al. 2020

Preprint

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Objectives The coronavirus disease 2019 (COVID-19) epidemic brings potentially impact on the care of patients with rheumatic diseases, including SAPHO syndrome. We aimed to investigate the disease status, concerns about management, and psychological stress in SAPHO patients during the COVID-19 epidemic. Method A structured questionnaire was distributed online to patients with SAPHO syndrome enrolled in a Chinese cohort study on March 3rd, 2020. Patients were ask about the current treatments, disease status, and concerns about disease management during the epidemic. Psychologic stress (scored from 0 to 10 points) and psychological problems were reported by the patients. Results A total of 157 patients (mean age 38.4 ± 12.3 years, 66.9% females) were included in the study. None of the patients were diagnosed with COVID-19. Sixty-five (41.4%) patients worried about their disease conditions during the epidemic with concerns including medication shortage (73.8%), delay of consultation (46.2%), and disease aggravation (61.5%). Sixty-seven (42.7%) patients had medication withdrawal or dose reduction due to lack of drugs, irregular daily schedule or subjective reasons. The most common psychological problems reported was little interest or pleasure in doing things (66.2%). Patients with progressive disease condition were more distressed and disturbed by the epidemic. Patients with nail involvement felt more worried about their disease conditions than patients without (59.6% vs 31.0%, p =0. 001). Conclusions The COVID-19 epidemic imposes a negative impact on the disease management and psychological stress in SAPHO patients. Patients' access to specialty care and medication well as mental stress is of great concern.

“…This rare disease has an annual prevalence of 0.00144/100,000 to 1/10,000 [ 2 , 3 ], and it imposes a heavy burden on the health and quality of life of affected patients [ 4 ]. SAPHO syndrome can occur at any age, but predominantly affects female patients [ 5 – 7 ]. SAPHO aetiology remains elusive and may be associated with genetic, infectious, and autoimmune factors [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…Osteoarticular disorder is the characteristic feature of SAPHO syndrome and typically involves the anterior chest wall. The peripheral bones are affected in one-third of patients [ 3 , 5 ]. Mandibular lesions occur in 2–10% of SAPHO patients and are generally located on the posterior mandibular body and ramus [ 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

Mandibular involvement in SAPHO syndrome: a retrospective study

Wang

et al. 2020

Orphanet J Rare Dis

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Background Mandible osteomyelitis can occur in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, a rare chronic inflammatory disease; however, few studies have explored its characteristics and management. Methods We reviewed the medical records of consecutive SAPHO patients with mandible involvement diagnosed in Peking Union Medical College Hospital from September 2014 to July 2019. Demographic, clinical, laboratory, and imaging data were collected at baseline. Prescription data and follow-up magnetic resonance imaging (MRI) and cone beam computed tomography (CBCT) images were collected from the hospital information system. An electronic questionnaire was distributed to all patients to obtain their latest symptoms. Results A total of 26 SAPHO patients with mandibular involvement were involved, all of whom responded to the questionnaire (38.5% male; median age, 28 years; median follow-up duration, 2.1 years). Ten patients (38.5%) had undergone an oral procedure 1 month before the onset of mandibular symptoms. All 14 of the patients who underwent a surgical intervention relapsed within a median duration of 2 months (range 0.25–4.0 months), and 24 patients (92.3%) achieved improvement with conservative treatment. Following bisphosphonate treatment, remission of bone marrow oedema and osteolysis was observed on MRI and CBCT, and 5 patients receiving bisphosphonates with follow-up CBCT after remission did not relapse in 5.4 months (mean 6.0, range 3.2–9.9 months). Conclusion Mandibular involvement of SAPHO syndrome predominantly occurs in young women. Dental procedures are a possible risk factor. Conservative treatment, especially intravenous bisphosphonates, can lead to oral improvement.