Biological Treatments for SAPHO Syndrome: An Update

Firinu, Davide; Murgia, Giuseppe; Lorrai, Maria Maddalena; Barca, Maria Pina; Peralta, Maria Monica; Manconi, Paolo Emilio; Giacco, S.R. Del

doi:10.2174/1871528113666140520100402

Cited by 37 publications

(21 citation statements)

References 1 publication

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“…Etanercept 50 mg weekly was begun but discontinued because of severe injection site reactions. Treatment options for the patient at that time were to switch to another tumour necrosis factor (TNF)‐α inhibitor or ustekinumab, as involvement of the interleukin (IL)‐23/T helper (Th)17 pathway in SAPHO syndrome has been suggested . The latter was favoured because of a previously diagnosed demyelinating peripheral neuropathy.…”

Section: Case Reportmentioning

confidence: 99%

Successful treatment of SAPHO syndrome with apremilast

Adamo¹,

Nilsson²,

Krebs³

et al. 2018

Br J Dermatol

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SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a rare disease with inflammatory osteoarticular and skin involvement. The pathogenesis of SAPHO syndrome remains unclear, but evidence suggests it may be an autoinflammatory disease triggered upon exposure to infectious agents in genetically predisposed individuals. Induction of the interleukin (IL)-23/T helper 17 axis in addition to neutrophil activation seem to play a key role, and therapies targeting these immunological pathways, including tumour necrosis factor (TNF) inhibitors, ustekinumab, secukinumab and the IL-1 inhibitor anakinra, are potential treatment options that need further investigation. Here we report a case of a 24-year-old woman with SAPHO syndrome who presented at our clinic with palmoplantar pustulosis and sternoclavicular joint involvement. Previous treatments with topical steroids and keratolytics combined with nonsteroidal anti-inflammatory drugs, intravenous methylprednisolone, methotrexate and sulfasalazine had all failed to improve symptoms. Therapy with etanercept was not tolerated, and because of a previous demyelinating peripheral neuropathy, further treatment with TNF inhibitors was avoided. We initiated ustekinumab 45 mg, which improved skin manifestations but not joint pain. Dose escalation to 90 mg initially improved joint pain, but the dose had to be reduced to 45 mg again because of increased infections. During subsequent 45-mg ustekinumab treatment, joint pain exacerbated so we switched to adalimumab which caused an exacerbation of the disease, so we switched to secukinumab, which improved skin and joint symptoms significantly but was associated with a pustular hypersensitivity reaction. Finally, we began treatment with apremilast, a pan-cytokine approach, resulting in stabilization of the skin and joint symptoms without side-effects. To our knowledge, this is the first case report of apremilast as a treatment for SAPHO syndrome.

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Section: Case Reportmentioning

confidence: 99%

Successful treatment of SAPHO syndrome with apremilast

Adamo¹,

Nilsson²,

Krebs³

et al. 2018

Br J Dermatol

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“…Bisphosphonates, especially pamidronate, which act by inhibiting bone resorption and turnover, and by possible anti‐inflammatory activity, have rapid but transient activity of pain relief in some of these patients, and may lead to partial or complete sustained remission over time . Various case series and reports have stated the use of anti‐TNF‐α agents as a therapeutic option for cases of SAPHO unresponsive or refractory to conventional drugs . In cases of resistant SAPHO, the IL‐1 antagonist anakinra has also been successfully used …”

Section: Synovitis Acne Pustulosis Hyperostosis and Osteitismentioning

confidence: 99%

“…70 Various case series and reports have stated the use of anti-TNF-a agents as a therapeutic option for cases of SAPHO unresponsive or refractory to conventional drugs. 71 In cases of resistant SAPHO, the IL-1 antagonist anakinra has also been successfully used. 72…”

Section: Synovitis Acne Pustulosis Hyperostosis and Osteitismentioning

confidence: 99%

Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation

et al. 2016

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Pyoderma gangrenosum is a rare inflammatory neutrophilic dermatosis manifesting as painful ulcers with violaceous, undermined borders on the lower extremities. It may occur in the context of classic syndromes like PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) and SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis), as well as in a recently described entity named PASH (pyoderma gangrenosum, acne and suppurative hidradenitis). Pyoderma gangrenosum has recently been included within the spectrum of autoinflammatory diseases, which are characterized by recurrent episodes of sterile inflammation, without circulating autoantibodies and autoreactive T cells. In PAPA syndrome, different mutations involving the PSTPIP1 gene, via an increased binding affinity to pyrin, induce the assembly of inflammasomes. These are molecular platforms involved in the activation of caspase 1, a protease that cleaves inactive prointerleukin (pro-IL)-1β to its active isoform IL-1β. The overproduction of IL-1β triggers the release of a number of proinflammatory cytokines and chemokines, which are responsible for the recruitment and activation of neutrophils, leading to neutrophil-mediated inflammation. In SAPHO syndrome, the activation of the PSTPIP2 inflammasome has been suggested to play a role in inducing the dysfunction of the innate immune system. Patients with PASH have recently been reported to present alterations of genes involved in well-known autoinflammatory diseases, such as PSTPIP1, MEFV, NOD2 and NLRP3. Pyoderma gangrenosum and its syndromic forms can be regarded as a single clinicopathological spectrum in the context of autoinflammation.

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“…In recent case series, remarkable improvement of bone, joints, and skin inlammatory manifestations was observed with Inliximab (INFX) therapy. In resistant SAPHO cases, the IL-1 antagonist anakinra can be also tried [35,36].…”

Section: Synovitis Acne Pustulosis Hyperostosis and Osteitis (Sapmentioning

confidence: 99%

Acne-Associated Syndromes

Emiroğlu¹

2017

Acne and Acneiform Eruptions

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Introduction: Acne, a chronic inlammatory disorder of pilosebaceous unit, is characterized by comedones, pustules, papules, nodules, cysts, and scars. It afects nearly 85% of adolescents. High sebaceous gland secretion, follicular hyperproliferation, high androgen efects, propionibacterium acnes colonization, and inlammation are major pathogenic factors. Systemic disease or syndromes that are associated with acne are less commonly deined. Therefore, these syndromes may not be usually recognized easily.Research methods: Acne-associated syndromes prove the nature of these diseases and are indicative of pathogenesis of acne. Polycystic ovary (PCOS), synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO), hyperandrogenism-insulin resistance-acanthosis nigricans (HAIR-AN), pyogenic arthritis-pyoderma gangrenosum-acne (PAPA), pyoderma gangrenosum-acne vulgaris-hidradenitis suppurativa-ankylosing spondylitis (PASS), pyoderma gangrenosum-acne conglobate-hidradenitis suppurativa (PASH), seborrhea-acne-hirsutism-androgenic alopecia (SAHA), and Apert syndromes are wellknown acne-associated syndromes. Endocrine disorders (insulin resistance, obesity, hyperandrogenism, etc.) can be commonly seen in these syndromes, and there are too many unknown factors that must be investigated in the formation of these syndromes.Conclusion-key results: If we are aware of the component of these syndromes, we will recognize those easily during dermatological examination. Knowledge of clinical manifestations and molecular mechanisms of these syndromes will help us to understand acne pathogenesis. When acne pathogenesis is explained clearly, new treatment modalities will be developed.

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Biological Treatments for SAPHO Syndrome: An Update

Cited by 37 publications

References 1 publication

Successful treatment of SAPHO syndrome with apremilast

Successful treatment of SAPHO syndrome with apremilast

Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation

Acne-Associated Syndromes

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