SAPHO-Syndrom: Ein Überblick

Zusammenfassung

Trotz erheblicher Verbesserungen der diagnostischen und therapeutischen Möglichkeiten in den letzten Jahren gehört das SAPHO-Syndrom zu den spät diagnostizierten und häufig untertherapierten Erkrankungen. Beim Zusammentreffen der typischen Hautmanifestationen (schwere Akne oder Pustulosis palmoplantaris) mit einem osteoartikulären Befall der vorderen Thoraxwand ist die Diagnose leicht zu stellen. Weniger typische Konstellationen bzw. Lokalisationen bedürfen bildgebender Diagnostik mit Skelettszintigrafie oder (Ganzkörper-) MRT und im Zweifelsfall der Biopsie zum Ausschluss einer infektiösen oder neoplastischen Genese. Therapeutisch sind die osteitischen Beschwerden durch NSAR und gegebenenfalls Bisphosphonate gut zu beeinflussen, während alle Manifestationen des Erkrankungsspektrums meist sehr gut auf TNF-Inhibition ansprechen. Sowohl bei der Diagnosestellung als auch bei der Bewertung des Therapieerfolgs sind psychosoziale Aspekte mit erheblicher Beeinträchtigung der Lebensqualität bei Patienten mit SAPHO besonders zu beachten.

Abstract

Despite the improvements in diagnosic and therapeutic options in recent years, the SAPHO syndrome is still diagnosed late and treated suboptimally. The diagnosis is readily made when typical dermatologic manifestations like severe acne or putulosis palmoplantaris occur together with osteoarticular complaints of the anterior chest wall. In less typical constellations or localisations, the diagnosis has to be established by the use of bone scintigraphy or (whole body) MRI. In unusual cases, infections or neoplasms have to be ruled out by bone biopsy. When the pain caused by osteitis is the leading clinical symptom, NSAIDs or bisphosphonates can be good treatment options. All manifestations of SAPHO have been shown to respond to TNF inhibition. Of note, SAPHO is a spectrum of diseases with high impact on the quality of life and mental health, which has to be taken into account for diagnositc and therapeutic considerations.

Schlüsselwörter

SAPHO - CRMO - NBO

Key words

SAPHO - CRMO - NBO

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