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Sklerodermie

Scleroderma

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Zusammenfassung

Die Sklerodermie (Synonyme: systemische Sklerose, systemische Sklerodermie) ist eine Systemerkrankung, die neben der Haut auch innere Organe wie die Lunge, den Gastrointestinaltrakt, die Niere und das Herz befällt. Pathogenetisch ist zwischen einer unkontrollierten Bindegewebsvermehrung (Fibrose) und einer Vaskulopathie zu unterscheiden. Dies führt klinisch neben den Organfibrosen auch zu Gefäßmanifestationen. Hierzu zählen Fingerkuppenulzera, die pulmonalarterielle Hypertonie und die akute Nierenkrise. Von der systemischen Sklerose sind lokalisierte Sklerodermieformen wie die Morphea abzugrenzen, die ohne Organkomplikationen verlaufen. Aufgrund ihrer klinischen Heterogenität, ihrer hohen Morbidität und Mortalität stellt die systemischen Sklerose für den klinischen Alltag eine große diagnostische und therapeutische Herausforderung dar. Dieser Übersichtsartikel fasst den aktuellen Stand zur Klassifikation und Epidemiologie, Pathogenese, den wichtigsten klinischen Manifestationen wie interstitielle Fibrose, pulmonalarterielle Hypertonie, akute Nierenkrise und periphere Vaskulopathie zusammen und gibt einen Überblick aktueller und zukünftiger Therapiemöglichkeiten.

Abstract

Scleroderma (synonyms: systemic sclerosis, systemic scleroderma) is a systemic disease which affects the skin as well as internal organs such as the lungs, gastrointestinal tract, kidneys, and the heart. Pathogenetically a distinction should be made between uncontrolled formation of extracellular matrix proteins (fibrosis) and vasculopathy. In addition to organ fibrosis, this leads to a clinical picture of vascular manifestations. These include fingertip ulcers, pulmonary arterial hypertension, and acute renal crisis. Localized forms of scleroderma, such as morphea, which do not involve organ complications, should be differentiated from systemic sclerosis. Due to its clinical heterogeneity and high rate of morbidity and mortality, systemic sclerosis poses an enormous diagnostic and therapeutic challenge in everyday clinical practice. This review article summarizes the current status of classification and epidemiology, pathogenesis, and the most important clinical manifestations such as interstitial fibrosis, pulmonary arterial hypertension, acute renal crisis, and peripheral vasculopathy and provides an overview of current and future treatment options.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin:

Dr. Distler has consultancy relationships and/or has received research funding from Actelion, Pfizer, Encysive, FibroGen, Ergonex, NicOx, BMS, and Biovitrum in the area of potential treatments of scleroderma and its complications. He has received lecture honoraria from Actelion, Pfizer, Encysive, and Ergonex.

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Authors and Affiliations

  1. Rheumaklinik und Institut für physikalische Medizin, UniversitätsSpital Zürich, Gloriastrasse 25, 8091, Zürich, Schweiz

    O. Distler & S. Gay

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  1. O. Distler
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  2. S. Gay
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Correspondence to O. Distler.

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Distler, O., Gay, S. Sklerodermie. Internist 51, 30–38 (2010). https://doi.org/10.1007/s00108-009-2405-z

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