Pulmonale Beteiligung bei idiopathischen inflammatorischen Myopathien

Zusammenfassung

Idiopathische inflammatorische Myopathien (IIM) sind seltene Systemerkrankungen, bei denen es je nach Entität häufig zu einer pulmonalen Beteiligung kommt, v.a. in Form einer interstitiellen Lungenerkrankung (ILD=interstitial lung disease). Es liegen nur wenige prospektive Daten über diese sehr heterogene Erkrankungsgruppe in Kombination mit einer ILD vor. Die Diagnose ist eng verknüpft mit dem Diagnoseleitfaden für interstitielle Lungenerkrankungen jeglicher Ätiologie und sollte in einem interdisziplinären Zentrum mit entsprechender Expertise gestellt werden. Zur Diagnostik gehören Myositis-spezifische Antikörper (MSA) und Myositis-assoziierte Antikörper (MAA), deren Nachweis mit einem Phänotyp und einem prognostischen Verlauf assoziiert sein kann. V.a. Anti-tRNA-Synthetase-Antikörper (Anti-ARS) und gegen das Melanom-Differenzierungsantigen-5-gerichtete Antikörper (MDA5-AK) spielen bei der IIM mit ILD eine entscheidende Rolle für die Prognose und das Therapieansprechen. Die am häufigsten vorkommenden ILD-Muster sind NSIP (nicht spezifische interstitielle Pneumonie) und OP (organisierende Pneumonie) oder ein Mischbild aus beiden Mustern. Die Grundbausteine der Therapie sind der initiale Einsatz von systemischen Steroiden und die frühzeitige Ergänzung von immunsuppressiven Substanzen, für deren Einsatz es hauptsächlich retrospektive Daten gibt. Neue prospektive Studien geben Anlass zur Hoffnung auf die Etablierung von Behandlungspfaden für die Zukunft.

Abstract

Idiopathic inflammatory myopathies are rare systemic diseases with different types of pulmonary manifestations depending on the underlying aetiology; here, interstitial lung diseases (ILD) are the most frequently found patterns depending on the underlying disorder. There is a lack of sufficient prospective studies on this heterogeneous group of patients, particularly in case of ILD being involved. The diagnosis is based upon guideline recommendations for ILD and requires a multidisciplinary discussion within a team with specific expertise in this field. Myositis specific antibodies and myositis associated antibodies form an essential part of the diagnostic tools and may also be associated with a certain phenotype or disease progression. Anti-t-RNA-synthetase antibodies (Anti-ARS) and anti-melanoma differentiation-associated gene 5 antibodies (MDA5) play an important clinical role for treatment the estimation of response and prognosis. The most common ILD patterns are nonspecific interstitial pneumonia (NSIP) and organising pneumonia (OP) or a mixed pattern of both. Treatment is based on systemic steroids and early initiation of other immunosuppressant drugs. Evidence for this is, however, sparse, since most of the studies having investigated treatment modalities are of retrospective nature, even though some new prospective data may be useful for the establishment of treatment pathways in the future.

Schlüsselwörter

Interstitielle Lungenerkrankungen - Myopathien - Systemerkrankungen - Interdisziplinäre Zusammenarbeit

Keywords

Interstitial lung diseases - Myopathies - Systemic diseases - Interdisciplinary cooperation

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