Synovitis-acne-pustulosis-hyperostosis Osteomyelitis Research Articles

Pustular Psoriasis and Associated Musculoskeletal Disorders.

Pustular psoriasis (PsO) is an uncommon variant of PsO that may present in a generalized or localized fashion with or without musculoskeletal or systemic inflammatory involvement. Generalized pustular PsO (GPP) presents as a widespread acute or subacute pustular eruption that may be familial and is often associated with severe flares and systemic inflammation. The palmoplantar pustulosis variant is localized to palms and soles, whereas acrodermatitis continua of Hallopeau is localized to the nail apparatus. Patients with pustular PsO may have overlapping plaque PsO and may develop psoriatic arthritis (PsA). Pustulosis is also a feature of both synovitis, acne, pustulosis, hyperostosis, osteomyelitis (SAPHO) syndrome and chronic nonbacterial osteomyelitis. At the 2020 Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) annual meeting, members were given an overview of the cutaneous features of pustular PsO, SAPHO, and recent insights into the genetics of GPP, leading to new targeted drug therapies and the development of validated endpoints.

SAPHO syndrome with pathological fractures of vertebral bodies: a case report

BackgroundIt’s difficult to diagnose and treat synovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome due to its rare and unknown pathogenesis. There is no effective treatment for SAPHO syndrome and the consequences of empirical treatment are unpredictable. This study reports a case of a young female diagnosed as SAPHO syndrome with pathological fractures of vertebral bodies.Case presentationA 29-year-old female complained of the right sternoclavicular joint and back pain accompanied limited activities and cutaneous lesions. Laboratory assays revealed abnormal inflammatory factors. Multiple imaging studies illustrated bone lesions and pathological fractures of vertebral bodies. A diagnosis of SAPHO syndrome was made. The patient was treated with Compound Troxerutin and Poreine Cerebroside Injection, non-steroidal anti-inflammatory drugs (NSAIDs), bisphosphonates, corticosteroids and the thoracolumbar brace. The patient was followed up for 6 months and showed improved results.ConclusionsThe case supports that multiple image inspections and laboratory tests contribute to diagnose SAPHO syndrome, and combination therapies of Compound Troxerutin and Poreine Cerebroside Injection, NSAIDs, bisphosphonates, corticosteroids and the thoracolumbar brace in the treatment of SAPHO syndrome with pathological fractures of vertebral bodies are crucial to regain health.

Imaging manifestations and its clinical significance in patients with synovitis acne pustulosis hyperostosis osteomyelitis syndrome

Objective To describe the clinical and imaging manifestations of patients with synovitis acne pustulosis hyperostosis osteomyelitis (SAPHO) syndrome,and to analyze the diagnostic importance of different clinical and imaging manifestations for SAPHO syndrome. Methods Seventeen patients (7 males and 10 females) with SAPHO syndrome were recruited in this study.Age ranged from 36 to 67 years with a mean age of (48 ± 8) years. All patients fulfilled the diagnostic criteria of Benhamou. Serum HLA B27 antigen records were reviewed for all patients. Imaging data of the abnormal bone sites were collected by conventional radiograph in all patients,CT in 13 patients as well as MR in 3 patients.Average time to take for a definite diagnosis of the syndrome was 3.7 years (ranged from O.5 to 13 years).Results Serum HLA B27 antigen was positive in all patients. Both skin and bone abnornalities were found in all patients.Ten patients had skin palmoplantar pustulosis and two patients had acne. Involving sites of bone and joints include sacroiliac joints,anterior chest and limbs.Sacroiliac joints were asymmetrically involved with imaging features in all patients.Eight patients exhibited anterior chest wall involvement. Five patients had osteomyelitis at limbs. For all images of 17 patients,CT was superior to conventional radiography in detecting abnormal changes of bone erosion and soft tissue swelling.MR imaging was able to depict edema changes that was not detectable by CT and radiography.Conclusion SAPHO syndrome is a rare disease,but for patients with skin and bone-joint abnormalities,especially with skin palmoplantar pustulosis,acne as well as with imaging features at the sacroiliac joint and anterior chest wall,SAPHO syndrome should be taken into a diagnostic consideration. Key words: Acquired hyperostosis syndrome; Magnetic resonance imaging; Tomography,X-ray computed

Fluctuating mixed-type hearing loss associated with synovitis–acne–pustulosis–hyperostosis–osteomyelitis (SAPHO) syndrome

Synovitis, acne, palmoplantar pustulosis (PPP), hyperostosis, and osteitis (SAPHO) are the characteristic features of SAPHO syndrome. A 53-year-old woman had been treated for PPP for 2 years. She complained of hearing loss in the right ear, and otitis externa was diagnosed. The pure-tone audiogram (PTA) indicated mild hearing loss in the right ear, and her hearing continued to deteriorate despite recovery from inflammation. Her tympanogram was of the As-type, and acoustic reflex was absent in the right ear. A computed tomography (CT) scan revealed bilateral normal ossicles and cochleas. Bone scintigraphy revealed tracer uptake in the bilateral sternoclavicular joints, glenohumeral joints, and the capital humerus. She was hospitalized for arthralgia, and the pain was controlled with steroid therapy. Her right hearing deteriorated soon after the tapering of the steroid; her hearing recovered after cyclosporine therapy was initiated. The first tonsil provocation test showed increased blood cells in the urine, and the second test showed exacerbation of pustulosis. Despite immunosuppressant therapy, the arthritis attacks and hearing loss persisted; therefore, tonsillectomy was performed, which improved PPP. However, her hearing remained unchanged after the operation. We considered that irreversible changes might have already developed in the ossicular joints, and ossicular reconstruction was performed. Thereafter, her hearing and earache improved.

A case of SAPHO (synovitis-acne-pustulosis-hyperostosis-osteomyelitis) syndrome in which [18F]fluorodeoxyglucose positron emission tomography was useful for differentiating from multiple metastatic bone tumors

We report the case of a 50-year-old Japanese woman with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteomyelitis) syndrome. Radiographs showed osteosclerosis of the cervical and lumbar vertebrae, as well as osteosclerosis and osteolysis of the right femoral neck, resembling multiple metastatic bony lesions. Arriving at a diagnosis required hematological and imaging tests. Whole-body bone scintigraphy identified diffuse uptake from the lower cervical vertebrae to the lumbar vertebrae and marked uptake in the right femoral neck. However, with [(18)F]fluorodeoxyglucose positron emission tomography ([(18)F]FDG-PET) scanning, abnormal [(18)F]FDG uptake was not detected in cervical and lumbar spine, or in the femoral neck. Bone biopsy showed signs of chronic nonspecific inflammation, rather than tumor or infection. Based on these findings, the patient was diagnosed with SAPHO syndrome unaccompanied by skin lesions, and administration of non-steroidal anti-inflammatory drugs provided pain relief.

Chronic osteomyelitis of clavicle as primary manifestation of SAPHO syndrome in adolescents: report of four cases and long-term evolution

Four cases of chronic osteomyelitis of clavicle as primary manifestation of synovitis, acne, pustulosis, hyperostosis, osteomyelitis (SAPHO) are reported in adolescents. In all cases a typical radiographical evolution had been observed with progressive slow migration of sclerotic area from medial to lateral side of clavicle. Long-term evolution was alternation of remission and exacerbation but none of the patients healed.

Classification of clinical subsets in psoriatic arthritis.

The classification of PsA is controversial. Two recent papers have suggested new subgroup classifications, however both have served only to expand the controversy. In this study the clinical and radiologic features of 100 patients with PsA are recorded in order to examine possible clinical or radiologic features which may be associated with functional disability and to examine the usefulness of recent reclassification proposals in our patient population. Results indicate that an asymmetric oligoarthritis (AO) pattern of disease is most common occurring in 43%. A symmetric polyarthritis (SP) was present in 33%, and this subgroup had the highest number of erosions and deformities, and a higher proportion of patients with grade III/IV ARA functional disability. While the DIP joints were involved in 46%, predominant DIP joint disease occurred in 16%. As the DIP group had the shortest duration of arthritis, it may be that this group represents recent onset PsA which will evolve into one of the other subgroups. Sacroiliitis was a feature in 14% with predominant spondylitis (SPON) in 4%. Arthritis mutilans (AM) was rare occurring in only 2% of this population, perhaps representing patients with end-stage SP disease. Finally, 2% of patients had features of the synovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome. A classification comprising just three subgroups (AO, SP, SPON) is proposed as being of most use clinically though a long-term prospective study is needed for verification.