Skeletal changes during the course of pustulosis palmoplantaris--report of two cases

We report two cases of coincidence palmoplantar pustulosis and joint changes. Two patients fulfilled criteria for arthroosteitis pustulosa: pustular eruptions of the palms and soles and pain of sterno-clavicular joint. They also fulfills criteria for SAPHO syndrome: osteoarticular manifestations of palmoplantar pustulosis. Clinical examination and scintigraphy confirmed the diagnoses. Treatment was started with antibiotics and nonsteroid anti-inflammatory drugs. Oral corticosteroids therapy with methotrexate was given to one patient.     

Synovitis,acne, pustulosis,hyperostosis, and osteitis syndrome with cranial bone involvement: Case report and literature review

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.     

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.     

Síndrome de sinovitis,acné, pustulosis,hiperostosis, osteítis y tratamiento con antagonistas del factor de necrosis tumoral

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis) encompasses a group of osteoarticular manifestations, especially hyperostosis in the joints and bones of the anterior chest wall and skin in the form of severe acne or hidradenitis. In the treatment of this syndrome, whose pathogenesis is unknown, many drugs have been used such as nonsteroidal anti-inflammatory agents and antibiotics. Corticosteroids, methotrexate, sulfasalazine and intravenous bisphosphonates have been used mainly in refractory cases. The development of biological treatments, indicated in certain rheumatic diseases, has provided a new therapeutic option, especially in patients without response. Anti-tumor necrosis factor-alfa agents, specifically infliximab and etanercept, have been used in isolated cases and small series. In general, the results have been satisfactory and these drugs can be an effective alternative to conventional treatments.     

Successful treatment of SAPHO syndrome with zoledronic acid

The SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a chronic, relapsing, inflammatory condition with skin and osteoarticular manifestations. Its etiology remains unclear, and various treatment regimens with steroids and nonsteroidal antiinflammatory drugs frequently fail to control the disease, while exposing patients to the side effects of these drugs. Because the SAPHO syndrome manifests as a destructive inflammatory bone disease, use of bisphosphonates that possess antiosteoclastic and probably antiinflammatory properties has been suggested to be helpful. To our knowledge, this is the first reported case of successful treatment with zoledronic acid of SAPHO syndrome that was resistant to conventional treatment.     

SAPHO syndrome treated with pamidronate: an open-label study of 10 patients

BACKGROUND: In recent years the SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) has been encountered more frequently. However, clinical evidence indicating superiority of a specific therapeutic modality is still absent. Pamidronate, a second-generation bisphosphonate, has a pronounced effect on bone metabolism by suppressing bone resorption. We report our clinical experience with intravenous pamidronate in SAPHO syndrome. METHODS: Between the years 1999 and 2003 we treated 10 patients with the SAPHO syndrome who did not respond to NSAIDs, oral corticosteroids, colchicine, methotrexate, sulphasalazine or infliximab. All patients were treated with 60 mg pamidronate, given intravenously within an hour. In cases of no response a subsequent dose was given within a month and if there was a partial response an additional infusion was given after 4 months. The primary endpoint was the disappearance of recurrent bouts of bone pain, osteitis or hyperostosis, or recurrent synovitis. Reduction of the frequency of attacks by 50% was regarded as a partial response. RESULTS: Seven of the patients were females and three were males. The age at diagnosis ranged from 26 to 68 yr. All patients had axial or peripheral arthritis and cutaneous involvement; three had severe acne, eight had pustulosis and two had concomitant psoriasis vulgaris. Hyperostosis of the anterior chest wall involving either sternocostal or sternoclavicular joints, as seen on technetium 99 bone scintigraphy, was detected in all patients. Complete remission was observed following therapy in six patients, three others partially responded and only one patient had no response. Two patients needed four cycles of pamidronate infusion, one patient needed three, six needed two infusions and one patient remitted following a single pamidronate infusion. In all but one patient pamidronate was effective in preventing recurrent bouts of pustulosis. CONCLUSION: Pamidronate seems to be a very effective mode of therapy for patients with the SAPHO syndrome, by promoting remission in all components of the disorder, such as bone, joint and skin involvement, and ceases the bouts that characterize this disorder.     

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...     

Crohn's disease and the SAPHO syndrome during treatment with infliximab: a case report and review of literature

We report the case of a 40 year-old Turkish woman with severe ileal Crohn's disease associated with palmoplantar pustulosis and aseptic spondylitis, also known as the SAPHO syndrome. However, in the present case, the syndrome developed three months after starting infliximab (Remicade) therapy, which is generally used to treat the SAPHO syndrome. This case-report discusses the unclear relationship between Crohn's disease, the appearance of a SAPHO syndrome and treatment with infliximab.     

Infliximab can induce a prolonged clinical remission and a decrease in thyroid hormonal requirements in a patient with SAPHO syndrome and hypothyroidism

SAPHO syndrome is a rare entity that compromises the skeletal system (arthritis–osteitis) and is associated with various dermatological conditions such as palmoplantaris pustulosis (PPP) and acne. We present the case of a 39-year-old man with invalidating arthritis derived from a SAPHO syndrome and hypothyroidism (after radioiodine treatment for a Graves’ disease). Due to the severity and refractoriness of his disease, we decided to use infliximab. He showed a prompt and prolonged response of his joint and cutaneous manifestations after three doses of a tumor necrosis factor alpha (TNF-α) blocker. Interestingly, he also decreased his levothyroxine requirements after TNF-α blockade therapy.     

SAPHO-Syndrom

The SAPHO syndrome, an acronym for synovitis, acne, pustulosis, hyperostosis and osteitis, is a rare disease which affects bones, joints and the skin. The main osteoarticular features are hyperostosis and osteitis. Osteoarticular symptoms predominantly occur on the anterior chest wall but the spine and the peripheral skeleton can also be involved. The most important skin affections are palmoplantar pustulosis and severe acne. The etiology of this syndrome remains unclear but infectious, immunological and genetic factors are involved. The diagnostic features of SAPHO syndrome are clinical and radiological. The most important diagnostic procedure is Tc-99 m bone scintigraphy but conventional x-rays as well as computed tomography (CT) and magnetic resonance imaging (MRI) can also contribute to the final diagnosis. Bone histology and positron emission tomography CT (PET-CT) may help to differentiate SAPHO syndrome from malignancies and infectious osteomyelitis. Nonsteroidal anti-inflammatory drugs (NSAIDs) are the cornerstone of treatment. The results obtained using antibiotics and disease-modifying antirheumatic drugs (DMARDs), such as sulfasalazine and methotrexate are inconsistent. Bisphosphonates and anti-tumor necrosis factor (anti-TNF) drugs have shown promising results in small studies but further research is still necessary.     

À propos d’un cas de SAPHO de localisation laryngée

Introduction

Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) is a syndrome that combines dermatological, articular and osseous inflammatory manifestations. Bilateral laryngeal immobility relative to cricoarytenoid joint origin is very uncommon. This article presents a case of bilateral cricoarytenoid joint ankylosis in a SAPHO syndrome context.

SAPHO Syndrome: Current Developments and Approaches to Clinical Treatment

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare autoimmune disease which, due to its clinical presentation and symptoms, is often misdiagnosed and unrecognized. Its main features are prominent inflammatory cutaneous and articular manifestations. Treatments with immunosuppressive drugs have been used for the management of SAPHO with variable results. To date, the use of anti-TNF-α agents has proved to be an effective alternative to conventional treatment for unresponsive or refractory SAPHO cases. TNF-α is a pro-inflammatory cytokine and pivotal regulator of other cytokines, including IL-1 β, IL-6, and IL-8, involved in inflammation, acute-phase response induction, and chemotaxis. IL-1 inhibition strategies with anakinra have shown efficacy as first and second lines of treatment. In this review, we will describe the main characteristics of biological drugs currently used for SAPHO syndrome. We also describe some of the promising therapeutic effects of ustekinumab, an antibody against the p40 subunit of IL-12 and IL-23, after failure of multiple drugs including anti-TNF-α and anakinra. We discuss the use and impact of the new anti-IL-1 antagonists involved in the IL-17 blockade, in particular for the most difficult-to-treat SAPHO cases.     

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.     

Hyperostosis and multifocal osteitis: a purely rheumatological subset of the SAPHO syndrome

SAPHO has recently been proposed as an acronym to identify a syndrome characterized by synovitis, acne, pustulosis, hyperostosis and osteomyelitis. Several authors have, however, found that this rare condition may even occur without cutaneous manifestations. The work reported here presents the case of a 43-year-old male with skeletal involvement alone. An in-depth study of the patient revealed the arthro-osteitic pattern typical of the SAPHO syndrome. In agreement with previous reports, these data confirm that the present clinical picture of hyperostosis and osteitis, even without cutaneous involvement, is nonetheless related to the SAPHO syndrome.     

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.     

Synovitis-acne-pustulosis-hyperostosis-osteitis Syndrome with Bilateral Pleural Effusion

Pleural effusion is a rare manifestation in synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome, which is characterized by the presence of osteoarticular lesions and dermatological involvement. We herein report a 71-year-old man with pleural effusion resulting from SAPHO syndrome. He was successfully treated using corticosteroids and has experienced no recurrence for one year. We should consider SAPHO syndrome when encountering cases of anterior chest pain and pleural fluid.     

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     

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.     

SAPHO syndrome or psoriatic arthritis? A familial case study.

OBJECTIVE: To discuss the relationships between SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome and the group of spondylarthropathies. METHODS: Few reports of familial SAPHO have been published. We describe three children, two sisters and one brother, whose clinical and radiological presentation was in accordance with SAPHO syndrome. RESULTS: Two children developed psoriasis, and one child palmoplantar pustulosis. Both sacroiliac and sternoclavicular joints were involved in these three cases. Some features in our observations are also common to psoriatic arthritis. No association was found with HLA antigens, but a history of trauma preceding the onset of symptoms was present in all three children. CONCLUSIONS: We can consider that SAPHO is nosologically related to spondylarthropathies. Psoriatic arthritis could be the missing link between SAPHO and spondylarthropathies. It is likely that both genetic and environmental factors are involved.     

The SAPHO syndrome--are microbes involved?

The syndrome of synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) includes a rare group of chronic, relapsing, inflammatory osteoarticular disorders that is conventionally associated with manifestations in the skin. Diagnostic dilemmas can arise due to incomplete manifestations or confusion generated through mimicking of other conditions, such as osteomyelitis. The aetiology of this syndrome remains unclear, but probably involves genetic, immunological and infectious mechanisms. The possible pathogenetic role of infectious agents in genetically predisposed individuals, resulting in a 'reactive osteitis', has been suggested because microbes such as Propionibacterium acnes have been recovered from bone biopsy samples. However, this hypothesis has not been demonstrated as yet. Current knowledge with regard to treatment of this syndrome is based on results reported from small case studies and, thus, is still empiric. The use of antibiotics, instituted based on the isolation of Propionibacterium acnes, has been reported to show conflicting results. Promising results for potential future application have recently been reported for treatment of SAPHO with bisphosphonates and antagonists of tumour necrosis factor-α. This review aims to evaluate the existing knowledge on the SAPHO syndrome and to provide information on symptoms, diagnosis and treatment options for this disease.