Intravenous pamidronate in the treatment of sternoclavicular hyperostosis

The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a rare clinical entity in which osteosclerosis and osteolysis are consistent radiological findings. We present a patient with SAPHO syndrome manifesting as painful sternoclavicular hyperostosis treated successfully with pamidronate. For the first time, a reduction in isotope bone scan activity was documented in parallel to a favourable clinical response. Bisphosphonates have potent antiosteoclastic and anti-inflammatory effects. By virtue of their dual effects on bone remodelling and inflammation, they seem an appropriate therapy in the management of SAPHO syndrome. This case demonstrates the efficacy of pamidronate, both clinically and radiologically, as first line therapy for patients with isolated hyperostosis.     

A retrospective study of bone scintigraphy in the follow-up of patients with synovitis,acne, pustulosis,hyperostosis, and osteitis syndrome: is it useful to repeat bone scintigraphy for disease assessment?

Clinical Rheumatology - To investigate the value of repeated bone scintigraphy in the follow-up of patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome and to...     

ARTHRO-OSTEITIS--A CLINICAL SPECTRUM

Arthro-osteitis is an uncommon condition which can be associatedwith palmoplantar pustulosis. It forms part of a group of conditionswhich include the synovitis, acne, pustulosis, hyperostosis,osteitis syndrome (SAPHO) and sternocostoclavic-ular hyperostosis.We report four cases illustrating the clinical spectrum of thiscondition which occurred in the absence of concomitant skinlesions. One patient had extensive aortic calcification a featurenot previously reported in this condition, which may representa low grade inflammatory aortitis. KEY WORDS: Sternocostoclavicular hyperostosis, SAPHO syndrome, Osteitis, Aortic calcification, Palmoplantar pustulosis     

Ilium osteitis as the main manifestation of the SAPHO syndrome: response to infliximab therapy and review of the literature

OBJECTIVE: To analyze the clinical efficacy of anti-tumor necrosis factor (TNF)-alpha therapy in the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. We describe 2 new cases with ilium osteitis as the main SAPHO syndrome feature and review reported cases treated with anti-TNF-alpha. METHODS: A literature search of SAPHO syndrome cases treated with TNF-alpha blocking therapy with special emphasis on osteoarticular and skin responses was performed. RESULTS: Eighteen cases were identified: 17 SAPHO syndrome and 1 chronic recurrent multifocal osteomyelitis, a juvenile variant of SAPHO syndrome. Sixteen were reported cases and 2 were nonreported cases seen in our arthritis unit. Sixteen patients received infliximab and 2 received etanercept, with an early, sustained clinical improvement in most cases. CONCLUSIONS: Anti-TNF-alpha therapies are effective treatment for patients with refractory SAPHO syndrome, not only for cutaneous lesions but also for persistent bone lesions such as osteitis.     

SAPHO syndrome and transient hemiparesis in a child: coincidence or new association?

We describe a case of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO syndrome) in an 8-year-old boy with an unusual presentation of acute transitory hemiparesis. SAPHO syndrome has been reported in association with inflammatory bowel diseases, chest complications, and pulmonary involvement. No patient with both SAPHO syndrome and neurologic complaints has been previously described. Further observations are needed to confirm if SAPHO syndrome and hemiparesis represent a coincidence or a new association.     

SAPHO syndrome in an adult with ulcerative colitis responsive to intravenous pamidronate: a case report and review of the literature

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome is a rare group of sterile, inflammatory osteoarticular disorders classically associated with skin lesions. It is occasionally associated with enteropathic disease such as ulcerative colitis. We present a 39-year-old patient with chronic ulcerative colitis who developed enteropathic SAPHO and responded well to pamidronate. We discuss the clinicopathological features with particular attention to bone pathology, and perform a literature review of this fascinating syndrome.     

New insights into synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome

In 1987, synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome was proposed as an umbrella term for a group of diseases with similar musculoskeletal manifestations, in particular hyperostosis of anterior chest wall, synovitis, and multifocal aseptic osteomyelitis, observed in association with dermatologic conditions such as palmoplantar pustulosis, severe acne, and hidradenitis suppurativa. Despite recent advances in our understanding of the epidemiologic, pathophysiologic, and immunogenetic mechanisms involved in SAPHO syndrome, etiopathogenesis remains poorly understood. Propionibacterium acnes, the microorganism associated with acne, has been recovered on bone biopsy in some patients, but the possible pathogenetic role of an infectious agent in a genetically predisposed individual, resulting in exaggerated inflammatory response as “reactive osteitis,“ is a largely unproven hypothesis. The newly available whole-body MRI will assist early diagnosis by detecting multifocal osteitis lesions, some of them asymptomatic, in axial (anterior chest wall, spine, and jaws) and nonaxial sites. Moreover, outcomes are vastly improved by treatment with bisphosphonates and tumor necrosis factor-α antagonists.     

Síndrome SAPHO en la infancia. Presentación de un caso clínico

The acronym of SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) combines a cluster of cutaneous and musculoskeletal manifestations, such as hyperostosis of bones of the anterior chest wall associated with acne fulminans and hidradenitis suppurativa. There are no validated diagnostic criteria in children. Nonsteroidal anti-inflammatory drugs are not always sufficient, and the use of corticosteroids, disease-modifying agents, tumor necrosis factor-α inhibitors or bisphosphonates may be necessary.We present the case of a child with polyarticular involvement, osteoarthritis of the sternoclavicular joint with severe inflammatory disorders and acne conglobata, with an excellent response to intravenous pamidronate.     

SAPHO: rare or just not recognized?

OBJECTIVE: The SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome describes an association between musculoskeletal disorders, in particular hyperostosis involving the bones and joints of the anterior chest wall, and various dermatologic conditions. It has been reported in Europe and Japan, but no Australian series have been published. We describe the clinical, laboratory, and radiographic features of a group of patients with the SAPHO syndrome and compare this with the literature. METHODS: We performed a retrospective review of patients seen in our department between 1990 and 1998 who met the proposed diagnostic criteria for SAPHO. Information regarding age, sex, disease duration, skeletal site(s) of disease, presence of skin disease, previous treatment, and response to treatment was collected. Laboratory tests were reviewed, as was all available radiology and bone scintigraphy. RESULTS: Six women with a mean age of 40 years fulfilled the criteria for SAPHO. The skeletal manifestations were similar to those reported in the literature, with hyperostosis of the anterior chest wall being the central feature. Cervical spine and pubic bone were other sites of involvement, whereas sacroiliitis and peripheral joint synovitis were not seen. Skin disease was less frequent in our population than has been reported in other series. Nonsteroidal anti-inflammatory drugs were frequently prescribed as first-line treatment but had limited efficacy. Intravenous pamidronate was administered to two patients, resulting in complete resolution of pain in one patient and 50% reduction in pain in the other. CONCLUSIONS: The SAPHO syndrome may be underrecognized as the skin manifestations in our patients were mild or absent. Although optimal treatment for these patients remains unclear, it is important to make the diagnosis of SAPHO to avoid unnecessary investigations and treatment.     

Pustulotic arthro-ostitis (SAPHO syndrome)

The sterile arthro-osteitis, especially in anterior chest wall, and also in spine and peripheral joints were observed in patients with pustulosis palmaris et plamtaris. These conditions were named pustulotic arthro-osteitis. Furthermore, the SAPHO syndrome, which stands for synovitis, acne, pustulosis, hyperostosis and osteitis, has been proposed in order to describe more varieties of bone, joints and skin lesions.     

SAPHO syndrome.

SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome put together osteoarticular lesions described separately under numerous denominations, such as multifocal osteomyelitis, pustulotic arthroosteitis, acne rheumatism. The association of sterile inflammatory bone lesions and neutrophilic skin eruptions is the cornerstone of this new syndrome, which also has links with spondyloarthropathies and plain psoriasis.     

Sternoclavicular pustulotic osteitis (SAPHO). 20-year interval between skin and bone lesions

In 2 cases of palmoplantar pustulosis, a 20-year interval was observed between the skin lesion and the bone involvement. In one case, the bone lesion came first. In the other a skin lesion was seen for 3 weeks without subsequent relapse. The 2 cases underline the multifaceted presentation of the condition for which we have coined the name synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome.     

SAPHO: has the time come for tailored therapy?

Clinical Rheumatology - SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a heterogeneous condition combining osteoarticular and cutaneous manifestations. Conventional...     

Osteitis as a symptom of SAPHO syndrome

SAPHO syndrome is an acronym given to a spectrum of diseases with the following features: synovitis, acne, pustulosis, hyperostosis and osteitis. The main problem of the differential diagnosis in 3 cases presented in our paper was the aseptic osteitis.     

Orofacial manifestations of SAPHO syndrome: a systematic review of case reports

Clinical Rheumatology - SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a poorly known disease with cutaneous and osteo-articular manifestations requiring a...     

Successful treatment of SAPHO syndrome with severe spinal disorder using entercept: a case study

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare disease. Presently, there is no treatment guideline for this illness. Several studies suggested entercept, a novel biological agent against tumor necrosis factor-alpha, is effective in treating SAPHO syndrome. We report a case in which the clinical conditions of a middle-aged female patient diagnosed with SAPHO syndrome, with noted spinal disorder, improved significantly after receiving entercept treatment. The patient remained stable after 3-month follow-up.     

The effectiveness of treatments for patients with SAPHO syndrome: a follow-up study of 24 cases from a single center and review of literature

Clinical Rheumatology - To evaluate the effectiveness of current treatments for SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) by delineating the therapeutic choices and...     

SAPHO syndrome presenting as widespread bony metastatic disease of unknown origin

SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome represents an inflammatory spectrum ranging from an osteitis through to a spondyloarthropathy which may or may not be associated with dermatological manifestations. We present the case of a 52-year-old female who presented with non-specific back pain. Radiological imaging showed mixed lytic and sclerotic lesions of her lumbar spine and pelvis consistent with bony metastatic disease. She underwent CT-guided biopsy that confirmed histological findings in keeping with non-bacterial osteitis/chronic recurrent multifocal osteomyelitis. This case report and review of the literature highlights the importance of the SAPHO syndrome and its treatment.     

Ostéomyélite sclérosante diffuse de la mandibule et SAPHO

Diffuse sclerosing osteomyelitis of the mandible may be the presenting manifestation of synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome. We report an additional case of such a presentation.     

In SAPHO syndrome anti-TNF-alpha therapy may induce persistent amelioration of osteoarticular complaints, but may exacerbate cutaneous manifestations

OBJECTIVES: SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a rare disease combining skin, bone and joint manifestations. In recent years new therapeutic strategies have been tried, among them TNF-alpha-blocking agents. We report our experience with infliximab in four cases of SAPHO syndrome refractory to conventional therapies. METHODS: Between 2002 and 2005, four cases of SAPHO syndrome (two females and two males; mean age 49.7 yr) responding poorly to conventional drugs were treated with infliximab. The dose was 5 mg/kg, according to the protocol used in spondyloarthropathies, with infusions at 0, 2 and 6 weeks followed by 6 weeks intervals. No active cutaneous manifestations were present at the time of starting therapy. RESULTS: Complete remission of osteoarticular involvement was achieved after the second or third infusion, and the positive response was maintained for up to 12 months. A patient relapsed after discontinuation of infliximab, because of infectious complication. Palmoplantaris pustulosis relapsed in two patients after three and six infusions, respectively; there was slight improvement after discontinuation of anti-TNF-alpha drugs. CONCLUSIONS: Infliximab seems to be a very effective therapy for osteoarticular complaints of SAPHO syndrome. Cutaneous involvement responded less favourably, palmoplantaris pustulosis relapse being a possible complication.