Sustained response to tumor necrosis factor α–blocking agents in two patients with SAPHO syndrome

Wagner, Annette D.; Andresen, Jan Fredrik; Jendro, M. C.; Hülsemann, J. L.; Zeidler, Henning

doi:10.1002/art.10539

Cited by 150 publications

(78 citation statements)

References 12 publications

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“…We used methotrexate in combination with NSAID and sulfasalazine as initial treatment for preventing long term steroid exposure. Because of the conflicting data and the lack of the evidence of any infectious agents in the cultures and the biopsy of our patient, we did not use any antibiotics as the first-line therapy in our patient 14,15 .…”

Section: Discussionmentioning

confidence: 95%

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Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report

Akçaboy¹,

Bakkaloğlu-Ezgü²,

Büyükkaragöz³

et al. 2017

Turk J Pediatr

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SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is defined as a syndrome that is related to various osteoarticular manifestations and chronic dermatological conditions especially severe acne. SAPHO syndrome is a rare and unusual clinical entity in childhood and treatment choices are variable. We report an 11-year-old girl who suffered from SAPHO syndrome and successfully treated with subcutaneous methotrexate. We report our case in order to take attention to this rare clinical condition in evaluating patients and also to point out that treatment options beyond biologic agents should be the first line treatment in childhood.Key words: children, methotrexate, SAPHO syndrome, treatment.The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) represents a syndrome characterized by the variable association of osteoarticular manifestations and various chronic dermatological conditions, particularly palmoplantar pustulosis and severe acne 1 . Although there have been case series of adults who have been followed up by the diagnosis of SAPHO syndrome 2,3 , there are few cases in childhood as case presentations 4,5 . The diagnosis of the SAPHO syndrome is based on exclusion of infectious arthritis, osteomyelitis, and tumoral conditions of the bone; and the presence of at least one of four diagnostic criteria proposed by Benhamou et al 6 .Here, we present an adolescent girl who was diagnosed as SAPHO syndrome and has been followed up for two years in remission with subcutaneous methotrexate therapy. Case ReportAn 11-year-old girl admitted to our hospital with a history of pain on her left thigh, both ankles, left hip, spine and on sternum especially when taking breaths for 20-days and swelling on both ankles for two days. She was not even able to move or walk due to severe pain. She had lost 4 kg during the last 3 weeks. She did not have fever or any trauma. Family history and past medical history was unremarkable. Physical examination revealed pustuloform acne on the face, and pustular lesions on the whole body ( Fig. 1), Sternal tenderness, ankle swelling and reduced motility of the vertebral column were remarkable. In her laboratory evaluation, erythrocyte sedimentation rate was 105 mm/ hour and high sensitive CRP was 6.23 mg/dl (0-0.2 mg/gl). All other rheumatologic parameters including rheumatoid factor and autoantibodies were within normal ranges or negative. She was negative for HLA-B27. Full septic screens were negative. Cultures of the pustular lesions were negative. Conventional X-rays showed periostal reactions on the head of femur and osteitis on the bilateral sacroiliac bones. MRI showed pathologic signals and contrast uptake on L1 vertebra, sacrum, left sacroiliac bones and the adjacent soft tissues suggesting malignancy (Fig. 2, 3). Nuclear bone scans demonstrated

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Section: Discussionmentioning

confidence: 95%

“…Treatment 15 . We used methotrexate in combination with NSAID and sulfasalazine as initial treatment for preventing long term steroid exposure.…”

Section: Discussionmentioning

confidence: 99%

Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report

Akçaboy¹,

Bakkaloğlu-Ezgü²,

Büyükkaragöz³

et al. 2017

Turk J Pediatr

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“…11 Preliminary experience supports the use of TNF-inhibitors and intravenous bisphosphonates in SCCH. [4][5][6][7][8][9] Ringe and colleagues 5 administered 2 mg of intravenous ibandronate every 3 months for up to a year to three patients with longstanding refractory SCCH and evaluated clinical, laboratory, and imaging at baseline and at 1 year. There was a prompt persistent pain relief, a gradual decline in inflammatory markers, and improvement in radiographic and scintigraphic findings.…”

Section: Discussionmentioning

confidence: 99%

“…3 Over the last two decades, there have been several reports in which intravenous bisphosphonates and tumor necrosis factor (TNF)-inhibitors have shown reasonable efficacy in the treatment of this disorder. [4][5][6][7][8][9] Here, we report the clinical, laboratory, and radiologic data of a patient with treatment-refractory SCCH.…”

Section: S Ternocostoclavicular Hyperostosis (Scch) Is a Rarementioning

confidence: 99%

Treatment-Refractory Sternocostoclavicular Hyperostosis

2017

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“…Similar to rheumatoid arthritis treatment, methotrexate and cyclosporine can be selected as further therapeutics for SAPHO joint pain. Antibiotics such as macrolides and tetracyclines have been used for the treatment of SAPHO syndrome in several reports [4,5,6,7,8,9,10,11,12]. There are a few options for the treatment of PPP or PPP with PAO to date, although antibiotics have been administered to several patients with PPP and PAO, referencing SAPHO treatment.…”

Section: Introductionmentioning

confidence: 99%

Cefcapene Pivoxil Hydrochloride Is a Potentially New Treatment for Palmoplantar Pustulosis with Pustulotic Arthro-Osteitis

et al. 2015

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Pustulosis palmaris et plantaris or palmoplantar pustulosis (PPP) is a refractory pustular eruption of the palms and soles with unknown etiology. In addition to skin lesions, PPP patients may present with severe joint pain and pustulotic arthro-osteitis (PAO), especially of the sternoclavicular joint. PAO is sometimes regarded as a variant of synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome. Hence, macrolide and tetracycline antibiotics are used for the treatment of PPP with PAO. We report 3 cases of PPP with PAO that did not improve upon administration of macrolide antibiotics with NSAIDs. After administration of cefcapene pivoxil hydrochloride (CFPN-PI), a third-generation cephalosporin, the swelling and sternoclavicular joint pain were promptly reduced and dramatically improved in all 3 cases. We review the conventional antibiotic treatments used currently and propose CFPN-PI as a potentially new therapy for PPP or PPP + PAO.

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Sustained response to tumor necrosis factor α–blocking agents in two patients with SAPHO syndrome

Cited by 150 publications

References 12 publications

Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report

Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report

Treatment-Refractory Sternocostoclavicular Hyperostosis

Cefcapene Pivoxil Hydrochloride Is a Potentially New Treatment for Palmoplantar Pustulosis with Pustulotic Arthro-Osteitis

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