Subject(s)
Acquired Hyperostosis Syndrome , Polyarteritis Nodosa , Thalidomide , Humans , Thalidomide/therapeutic use , Thalidomide/analogs & derivatives , Polyarteritis Nodosa/drug therapy , Polyarteritis Nodosa/complications , Acquired Hyperostosis Syndrome/drug therapy , Acquired Hyperostosis Syndrome/complications , Female , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Treatment Outcome , Middle AgedABSTRACT
Synovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome is characterised by aseptic osteitis and is often complicated by pustular dermatitis, such as palmoplantar pustulosis or acne. Although bone lesions are most found in the anterior thoracic region or spine, femoral lesions are not well documented in the literature. There is no established treatment for this condition, and few reports have described its long-term course. Here, we describe two cases of SAPHO syndrome involving the femur and discuss their long-term follow-up. A 40-year-old man (Case 1) presented with right thigh pain. Fifteen years after the initial diagnosis, the pain could be controlled with minomycin, salazosulfapyridine, and methotrexate. X-rays of the femur showed gradual cortical thickening. Although there were waves of pain, it gradually improved with the adjustment of drugs 25 years following the initial diagnosis. A 35-year-old man (Case 2) with right thigh pain was prescribed salazosulfapyridine and methotrexate; however, these were ineffective. Alendronate and guselkumab also proved ineffective. Ultimately, infliximab was started 9 years following disease onset, and pain became manageable. X-rays of the femur showed cortical thickening. SAPHO syndrome can be managed with drug therapies, such as nonsteroidal anti-inflammatory drugs, methotrexate, and conventional synthetic disease-modifying antirheumatic drugs; however, there are occasional treatment-resistant cases.
Subject(s)
Acquired Hyperostosis Syndrome , Femur , Humans , Male , Adult , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/drug therapy , Femur/pathology , Femur/diagnostic imaging , Treatment Outcome , Methotrexate/therapeutic use , Methotrexate/administration & dosageSubject(s)
Acquired Hyperostosis Syndrome , Pulmonary Arterial Hypertension , Humans , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/drug therapy , Pulmonary Arterial Hypertension/etiology , Pulmonary Arterial Hypertension/diagnosis , Pulmonary Arterial Hypertension/drug therapy , Pulmonary Arterial Hypertension/physiopathology , Treatment Outcome , Female , Pulmonary Artery/diagnostic imaging , Antihypertensive Agents/therapeutic use , Arterial Pressure/drug effects , Middle AgedABSTRACT
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is an uncommon clinical syndrome with the signs of skin problems and osteoarthropathy as its main features. The pathogenesis of SAPHO syndrome has not been fully elucidated, and multiple complications may be present, including thrombosis. A 39-year-old male patient was diagnosed with SAPHO syndrome, complicated by multiple venous thrombosis of the left lower limb. We conducted a brief review of the current available literature on thrombosis in patients with SAPHO syndrome and speculated that the presence of lower extremity thrombosis in this patient with SAPHO syndrome may be related to physiological structure or antiphospholipid syndrome. Whether positive lupus anticoagulant has an effect on thrombosis in patients with SAPHO syndrome remains to be investigated.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Antiphospholipid Syndrome , Osteitis , Synovitis , Venous Thrombosis , Male , Humans , Adult , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/drug therapy , Synovitis/complications , Venous Thrombosis/diagnostic imaging , Venous Thrombosis/drug therapy , Venous Thrombosis/etiology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Antiphospholipid Syndrome/drug therapy , Acne Vulgaris/complicationsABSTRACT
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) is a rare chronic inflammatory disease that develops in adults. We present a case of SAPHO syndrome in a 37-year-old male presenting with gradually worsening back and neck pain for a 7-year period. The episodes were preceded by a history of pustular skin eruptions, which first appeared on the upper trunk and then involved his face and were pustular and scarring. The purpose of presenting this case report from Iraq is to raise awareness about this rare condition, which is frequently misdiagnosed and under-recognized.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Osteitis , Synovitis , Male , Adult , Humans , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/drug therapy , Synovitis/diagnosis , Back Pain/diagnosis , Back Pain/etiology , Skin , Acne Vulgaris/diagnosisABSTRACT
SAPHO is an acronym derived from capital letters of Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO). SAPHO syndrome is an umbrella term covering a constellation of bone lesions and skin manifestations. A 40-year-old male complained about his jaw and back pain, swelling of multiple joints and weight loss accompanied by physical deterioration and acne type skin lesions. Laboratory tests revealed abnormal elevation of inflammatory markers. Imaging studies illustrated multiple osteolytic bone lesions and paraosseal infiltrates. According to the set of criteria diagnosis of SAPHO syndrome was stated. The patient was treated with glucocorticoids and non-steroidal anti-inflammatory drugs (NSAIDs), but only high dose dexamethasone and prednisone were effective. Daily subcutaneous administration of anakinra at the dose of 100 mg was initiated due to limited response to more classical therapies. Because of planned mandibular osteosynthesis initiation of denosumab was preferred before bisphosphonates. Therapeutic response was confirmed by FDG-PET/MR after 5 months of anakinra and denosumab therapy, showing decreased accumulation of FDG in periosteal and paraosseal infiltrates. Inflammatory markers significantly decreased, bone pain deferred but skin manifestation receded only partially. Therefore the response was evaluated as partial remission.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Osteomyelitis , Male , Humans , Adult , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/drug therapy , Acquired Hyperostosis Syndrome/diagnosis , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Denosumab/therapeutic use , Fluorodeoxyglucose F18/therapeutic use , Osteomyelitis/drug therapy , Osteomyelitis/complications , Osteomyelitis/microbiology , Acne Vulgaris/complications , Acne Vulgaris/diagnosisABSTRACT
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare autoimmune inflammatory disease characterized by osteoarticular and dermatological manifestations. The most common osteoarticular manifestations involve the anterior chest wall, axial skeleton, and long bones. Cranial bone involvement is less reported in SAPHO syndrome. We herein present three cases of SAPHO syndrome with cranial bone involvement, and review the previous literature on similar manifestations. It was revealed that SAPHO syndrome could lead to cranial bone involvement, which could involve the dura mater, leading to hypertrophic pachymeningitis, but the outcome is usually good. Janus kinase inhibitors may be a potential treatment option.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Hyperostosis , Osteitis , Synovitis , Humans , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/drug therapy , Osteitis/diagnostic imaging , Osteitis/drug therapy , Hyperostosis/diagnostic imaging , Hyperostosis/drug therapy , Acne Vulgaris/diagnosis , Acne Vulgaris/drug therapy , Rare DiseasesABSTRACT
Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic inflammatory disease. The main clinical manifestation of SAPHO syndrome is an osteoarthropathy with cutaneous involvement. Relapsing polychondritis (RP) characterized by chronic inflammation and cartilage degeneration is a rare systematic autoimmune disease. Here we report a RP case in a SAPHO syndrome patient, in which auricularitis happened 10 years after the diagnosed as SAPHO syndrome. Tofacitinib treatment can alleviate the symptoms.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Osteitis , Polychondritis, Relapsing , Synovitis , Humans , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/drug therapy , Polychondritis, Relapsing/complications , Polychondritis, Relapsing/diagnosis , Polychondritis, Relapsing/drug therapy , Synovitis/diagnosis , Acne Vulgaris/diagnosisABSTRACT
SAPHO syndrome is an autoinflammatory disease with a variety of clinical manifestations, which may be accompanied by other systemic inflammatory diseases in addition to the typical manifestations of common synovitis, acne, pustulosis, hyperostosis, and osteitis. Here, we report the first case of SAPHO syndrome combined with Takayasu arteritis.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Hyperostosis , Osteitis , Synovitis , Takayasu Arteritis , Humans , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/drug therapy , Takayasu Arteritis/diagnosis , Takayasu Arteritis/diagnostic imagingABSTRACT
We report a case of isolated lesions of the thoracic spine attributed to synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. A 55-year-old woman who suffered from 6 months of back pain had vertebral osteomyelitis on magnetic resonance imaging (MRI). There were no laboratory findings suggestive of infection, malignancy, or autoimmune disease. Radiography, computed tomography (CT), and MRI of the thoracic spine showed mixed lesions of sclerosis and erosion, whereas bone scintigraphy did not show accumulation at any site except the thoracic spine. No lesions in the anterior chest wall or sacroiliac joints were apparent from CT and MRI. No lesions other than at the thoracic spine were observed. As the isolated lesions of the thoracic spine were considered not to have resulted from infection, malignancy, or autoimmune disease, the patient was referred to our department for differential diagnosis. Given that isolated sterile hyperostosis/osteitis among adults is included in the modified diagnostic criteria for SAPHO syndrome, we suspected that the mixed lesions of sclerosis and erosion of the thoracic spine in this case may reflect SAPHO syndrome with chronic non-bacterial osteitis (CNO) of the thoracic spine. Treatment with non-steroidal anti-inflammatory drugs (NSAIDs) was initiated and led to alleviation of her back pain, although the thoracic spine lesions remained on the 6-month MRI. Based on the CNO of the thoracic spine and the rapid response to NSAIDs, the final diagnosis was SAPHO syndrome with isolated lesions of the thoracic spine.