Clinical heterogeneity of SAPHO syndrome: Challenge of diagnosis

Li, Chen; Cao, Yihan; Zhang, Wen

doi:10.1080/14397595.2017.1416733

Cited by 17 publications

(16 citation statements)

References 25 publications

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“…8,9 Most patients with SAPHO have cutaneous involvement, mainly manifested as palmoplantar pustulosis (PPP) and severe acne (SA), which are shown in Figure 1. 34 PPP, which is recognized as a special type of psoriasis, is characterized by chronic, recurrent, sterile, small pustules and vesicles. 39 Among the 354 Chinese patients in the cohort, 94.6% reported skin involvement, of whom 91.9% had PPP, 14.3% had SA, and 15.8% had psoriasis vulgaris.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update

Liu

Tang

Cao

et al. 2020

Therapeutic Advances in Musculoskeletal

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105

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Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a spectrum of heterogeneous diseases characterized by osteoarticular and dermatological manifestations. Osteitis and hyperostosis are core clinical manifestations in SAPHO syndrome, typically affecting multiple areas and possibly progressing to irreversible osteoarticular damage. Most patients with SAPHO have cutaneous involvement, mainly manifested as palmoplantar pustulosis and severe acne. Systemic manifestations are uncommon but occasionally reported. Epidemiological studies suggest the annual prevalence of SAPHO syndrome varies from 0.00144 in 100,000 in Japanese individuals to fewer than 1 in 10,000 in White individuals. The precise etiopathogenesis of SAPHO remains unclear, but it is generally considered an autoinflammatory syndrome that may be related to various etiologies, such as immune dysfunction, infection and genetic predisposition. Owing to the relapsing–remitting disease course, the goal of management is to improve clinical symptoms and prevent disease progression. Various treatments, including nonsteroidal anti-inflammatory drugs, conventional disease-modifying antirheumatic drugs, bisphosphonates, biologics, and antibiotics, are promising options for alleviating the disease.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update

Liu

Tang

Cao

et al. 2020

Therapeutic Advances in Musculoskeletal

Self Cite

105

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show abstract

“…Eine axiale Manifestation weisen etwa 32–52 % der Patienten auf [ 15 , 16 ], einen Befall peripherer Knochen und Gelenke 65–83 % [ 13 , 18 ]. Eine kutane Beteiligung beim SAPHO Syndrom ist häufig, meist in Form einer palmaren-plantaren Pustulose (ppP) und schweren Akne [ 19 ]. In einer monozentrischen chinesischen Kohortenstudie [ 13 ] von 354 SAPHO-Betroffenen aus dem Jahr 2019 konnte bei 94 % eine Hautbeteiligung (davon in 92 % eine ppP, die restlichen in Form einer schweren Akne, einer Psoriasis vulgaris und in wenigen Fällen einer psoriatischen Nagelbeteiligung) nachgewiesen werden.…”

Section: Klinische Manifestationen Beim Sapho-syndromunclassified

SAPHO-Syndrom

Klemm

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2021

Z Rheumatol

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ZusammenfassungBeim SAPHO-Syndrom handelt es sich nicht um eine Entität, sondern um einen inhomogenen, nosologisch wie pathogenetisch heterogenen Symptomenkomplex. Klinisch imponieren subakute, rezidivierende und/oder chronische Krankheitsprozesse mit charakteristisch gemeinsamer Haut-Knochen-Assoziation („ski[n]bo[ne]-disease“). Die chronisch rekurrierende multifokale Osteomyelitis (CRMO) ist die häufigste „SAPHO“-Erkrankung im Jugend- und Adoleszentenalter. Im Erwachsenenalter ist die Spondarthritis hyperostotica pustulo-psoriatica mit der Trias palmoplantare Pustulose, sternokostoklavikuläre Hyperostose und ossifizierenden Wirbelsäulenmanifestationen am häufigsten und generell als „SAPHO“ bekannt. Zusätzlich gibt es jedoch abortive Krankheitsformen: das entzündliche Anterior-chest-wall-Syndrom, das sternoklavikuläre Hyperostosesyndrom, die Akne-CRMO und die Akne-Spondarthritis. Insgesamt heilen die SAPHO-Krankheitsfälle meist mit relativ günstiger Prognose aus, es gibt aber auch ungünstige Verläufe mit funktionellen Einschränkungen. Neben der typischen Klinik dienen Bildgebung (Röntgen, Szintigraphie, Magnetresonanztomographie) und/oder histologische Knochenbiopsieanalyse der Diagnosestellung. Die Therapie sollte interdisziplinär erfolgen. Eine Antibiose ist obsolet. Der vorliegende Artikel vermittelt einen Überblick über das SAPHO-Syndrom und eine klinisch-rheumatologische wie bildgebende Differenzierung sowie nosologische Klassifizierung von 35 Fällen bei Erstvorstellung.

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“…The pathogenesis of SAPHO syndrome is not defined, but likely includes a combination of genetic, infectious and immunological components leading to the activation of innate and cell-mediate immune system [4]. Therefore, it is classified as an autoinflammatory disorder [5].…”

Section: Introductionmentioning

confidence: 99%

SAPHO syndrome: the supposed trigger by isotretinoin, the efficacy of adalimumab and the specter of depressive disorder: a case report

et al. 2020

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Background SAPHO (synovitis, acne, pustolosis, hyperostosis and osteitis) syndrome is a rare autoinflammatory chronic disorder, presenting with non-infectious osteitis, sterile joint inflammation and skin manifestations including palmoplantar pustolosis and severe acne. It could be often misdiagnosed for its heterogeneous clinical presentation. Treatment is challenging and, due to the rarity of this syndrome, no randomized controlled clinical trials have been conducted. Empirical treatments, including non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, antibiotics and bisphosphonates and disease-modifying anti-rheumatic drugs (DMARDs) could be quite effective. Anti-tumor necrosis factor-alpha (anti-TNF-α) agents and interleukin-1 (IL-1) antagonists have shown promising results in refractory patients. Isotretinoin, commonly used for severe acne, has been rarely described as possible trigger of osteo-articular manifestations, in particular sacroiliitis. Case presentation The case of a boy, affected by acne fulminans and depression, who presented with sacroiliitis after a 10-week treatment with isotretinoin is presented. After SAPHO diagnosis, NSAIDs therapy was started but the onset of bilateral gluteal hidradenitis suppurativa required the switch to a TNF-α antagonist (Adalimumab) with the achievement of a good control of the disease. Despite specific therapy with sertraline, the patient continued to complains severe depression. Conclusions Our case reports a temporal association between the onset of osteo-articular symptoms and the introduction of isotretinoin, as previously described. However, this timeline is not sufficient to establish a causal role of this drug into the pathogenesis of sacroiliitis. At this regard, further studies are required. The occurrence of hidradenitis suppurativa during SAPHO course supported the introduction of TNF-α blockers with a favourable result, as reported in a few cases in literature. The association between SAPHO syndrome and depressive mood disorders is already reported. Our patient experienced severe depression whose trend seems to be independent from the course of the main disease. Currently, it is not clarified if depression could be considered reactive to the underling disease or if it forms an integral part of the autoinflammatory disorder.

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Clinical heterogeneity of SAPHO syndrome: Challenge of diagnosis

Cited by 17 publications

References 25 publications

Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update

Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update

SAPHO-Syndrom

SAPHO syndrome: the supposed trigger by isotretinoin, the efficacy of adalimumab and the specter of depressive disorder: a case report

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