A statistical symptomatic evaluation on SAPHO syndrome from 56 cases of confirmed diagnosis and 352 cases of non-SAPHO involvement.

OBJECTIVE: To report a statistical evaluation of symptomatology based on 56 cases of SAPHO syndrome and 352 non-SAPHO involvement cases, to propose a symptomatic scoring system in consideration of early warning for SAPHO syndrome. METHODS: A cohort comprising 56 subjects diagnosed with SAPHO syndrome was reported, as well as 352 non-SAPHO involvement cases, including their chief complaints, skin manifestations, radiological findings, and laboratory tests. We systematically reviewed previous published five representative huge cohorts from different countries to conclude several specific features of SAPHO by comparing with our case series. The score of each specific index is based on respective incidence and comparison of two cohorts was performed. RESULT: In terms of complaint rates, all subjects of two cohorts suffered from osseous pain, which appeared in the anterior chest wall, spine, and limb which were calculated. In respect to dermatological lesions, SAPHO patients suffered from severe acne, and other patients (82.14%) accompanied with palmoplantar pustulosis. Having received radiological examinations, most SAPHO subjects rather than non-SAPHO involvement cases showed abnormal osteoarticular lesions under CT scanning and more detailed information under whole-body bone scintigraphy. Differences also emerged in elevation of inflammation values and rheumatic markers like HLA-B27. Based on our cases and huge cohorts documented, the early warning standard is set to be 5 scores. CONCLUSIONS: SAPHO syndrome case series with 56 subjects were reported and an accumulative scoring system for the early reminder on SAPHO syndrome was proposed. The threshold of this system is set to be 5 points. Key Points • Fifty-six patients diagnosed by SAPHO syndrome with detailed symptoms and radiological findings were reported. • Comparison was made between the 56 SAPHO patients and 352 non-SAPHO involvement cases. • An accumulative scoring system for the early reminder on SAPHO syndrome was proposed and the threshold of this system is set to be five points.

Comparison of 68Ga-DOTA-FAPI-04 and 18F-FDG PET/CT in Patients With SAPHO Syndrome.

PATIENTS AND METHODS: 68Ga-DOTA-FAPI-04 PET/CT was performed on a cohort of 21 patients with known SAPHO syndrome. All patients underwent 68Ga-DOTA-FAPI-04 and 18F-FDG PET/CT on 2 consecutive days. The positive rates of the PET/CT scans at the sites of the osteoarticular symptom, the uptake values, and agreement with clinical osteoarticular symptom were compared. RESULTS: A total of 38 sites of involvement were detected. 18F-FDG PET/CT revealed 28 lesions. In contrast, 68Ga-DOTA-FAPI-04 PET/CT detected not only all lesions shown on 18F-FDG PET/CT but additional 10 lesions. 68Ga-DOTA-FAPI-04 scan also demonstrated significantly higher uptake and target-to-background ratio than 18F-FDG studies in the skeletal involvements. The agreement between 68Ga-DOTA-FAPI-04-positive lesions and current osteoarticular lesions was substantial (κ = 0.79, P < 0.001), whereas 18F-FDG had low to moderate agreement with clinical symptoms (κ = 0.52, P < 0.001). CONCLUSIONS: 68Ga-DOTA-FAPI-04 has potential as a promising imaging agent for the evaluation of SAPHO syndrome.

Synovitis, acne, pustulosis, hyperostosis, osteitis syndrome with invisible organizing pneumonia: A case report.

We report the case of a 59-year-old female patient, presenting with pustular rash on both hands and pain in the lumbosacral part and left lower limb. A magnetic resonance imaging examination of the left leg was undertaken and the result showed that a malignant lesion with bone destruction of the left femoral shaft could not be excluded. Subsequently, bone tumor was excluded by pathological examination. Lung computed tomography scan showed patchy consolidation and cord shadow in the middle left lung. Subsequently, lung cancer was excluded by pathological examination, and the histopathological changes of lung were consistent with those of organized pneumonia. Blood tests revealed elevated C-reactive protein and erythrocyte sedimentation rate. Antinuclear antibody, rheumatoid factor, and human leukocyte antigen-B27 were unremarkable. Whole body bone scintigraphy via technetium 99m-methyl diphosphonate showed increased radionuclide uptake in the left middle femur. Based on her clinical manifestations, imaging results and bone scintigraphy, the patient was diagnosed as having synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome. Loxoprofen and Tripterygium wilfordii Hook F led to impressive clinical and radiologic improvement.

Whole-body Bone Scan in the Diagnosis and Treatment of SAPHO Syndrome.

Synovitis, acne, pustulosis, hyperostosis, and osteomyelitis (SAPHO) syndrome is rare disease of unknown pathogenesis. Its diagnosis and treatment are difficult. There is no standardised treatment for SAPHO syndrome, and the results of empirical therapy are unpredictable. The whole-body bone scan may help evaluate the efficacy of SAPHO syndrome treatment. The case of a 52-year Chinese male, affected by SAPHO syndrome, is presented and discussed in this report. After the patient received methotrexate, prednisone and non-steroidal anti-inflammatory drugs (NSAIDs) for six months, his clinical symptoms and whole body bone scan signs improved. Key Words: SAPHO syndrome, Whole-body bone scintigraphy, Treatment efficacy.

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

ABSTRACT: 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.

68Ga-DOTA-FAPI-04 PET/CT Imaging in a Case of SAPHO Syndrome.

ABSTRACT: A 66-year-old woman presented with anterior chest wall and knee joints pain and multiple skin lesions. 99mTc-MDP bone scintigraphy showed increased bone density in the sternal angle with abnormal bone metabolism. The patient was enrolled in a 68Ga-DOTA-FAPI-04 PET/CT tumor clinical trial, and it showed increased tracer uptake in the sternum and right knee joint. Subsequently, ultrasound of the right knee joint confirmed the existence of synovitis, and no malignant components were found in sternal biopsy. The patient's final diagnosis was synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. The case confirmed that 68Ga-DOTA-FAPI-04 imaging is helpful for evaluating the involved sites of SAPHO syndrome.