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Sklerodermie

Scleroderma

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Die Gastroenterologie Aims and scope

Zusammenfassung

Die gastrointestinale Beteiligung bei der systemischen Sklerodermie (SSc) wird klinisch durch Ösophagusfunktionsstörungen mit Dysfunktion der tubulären Peristaltik und Schwächung der gastroösophagealen Barriere mit erhöhtem Reflux und ihren Komplikationen bestimmt. Die gastrointestinale Beteiligung ist prognoserelevant und für einen signifikanten Anteil der Todesursachen verantwortlich. Die Pathophysiologie besteht aus vaskulären Veränderungen, Fibrose und Immunveränderungen, die wahrscheinlich in sequenzieller Folge ablaufen und die Speiseröhre schädigen. Beschwerden, die mit Ösophagusfunktionsstörungen korrelieren, sind Dysphagie, Reflux, Aufstoßen, Dyspepsie, Sodbrennen bzw. retrosternale Schmerzen. Zusätzlich können Gewichtsverlust, Übelkeit, Erbrechen, Husten bzw. Heiserkeit vorliegen. Die Diagnostik beinhaltet die Ösophagogastroduodenoskopie, die hochauflösende Ösophagusmanometrie und die kombinierte 24-h-pH-Metrie-Impedanzmessung. Die Therapie umfasst die Behandlung der Grunderkrankung und eine konsequente leitliniengerechte Refluxtherapie.

Abstract

Gastrointestinal involvement in systemic sclerosis (SSc) is clinically determined by esophageal functional disorders with dysfunction of esophageal peristalsis and decrease of gastroesophageal barrier function with increased gastroesophageal reflux and its complications. Gastrointestinal involvement in SSc is relevant for the prognosis and is responsible for a significant number of deaths. The pathophysiology includes vascular alterations, fibrosis and immune alterations that most likely occur in sequential order leading to esophageal damage. Symptoms that correlate with esophageal dysfunction are dysphagia, reflux, belching, dyspepsia, pyrosis and retrosternal pain. Weight loss, nausea, vomiting, cough and hoarseness can be additional symptoms. The diagnostics include esophagogastroduodenoscopy, high-resolution esophageal manometry and combined 24 h pH impedance monitoring. Therapy comprises the treatment of the underlying disease and consistent guideline-conform treatment of reflux.

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Martina Müller-Schilling, Regensburg

Stefan Zeuzem, Frankfurt am Main

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Frieling, T. Sklerodermie. Gastroenterologie 17, 296–307 (2022). https://doi.org/10.1007/s11377-022-00646-3

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