Zusammenfassung
Hintergrund
Eine pulmonale Beteiligung bei Patienten mit autoimmuner Grunderkrankung stellt eine große Herausforderung in Bezug auf Diagnostik und Therapie an den behandelnden Arzt. Aufgrund des damit vergesellschafteten erhöhten Mortalitätsrisikos ist eine frühzeitige Diagnose entscheidend.
Fragestellung
Betrachtet werden Inzidenz und Mortalität von interstitiellen Lungenerkrankungen (CTD-ILD) und pulmonaler Hypertonie (PH) bei Patienten mit Rheuma, Klinik, diagnostisches Vorgehen, Prognose und Therapieempfehlungen.
Material und Methode
Es erfolgte eine Analyse bemerkenswerter Publikationen und Darstellung von Leitlinien.
Ergebnisse
CTD-ILD und PH sind v. a. bei Patienten mit systemischer Sklerose (SSc) häufige Komorbiditäten mit signifikant erhöhtem Mortalitätsrisiko. Besonders bei primär fibrotischer und nichtinflammatorischer CTD-ILD, wie sie v. a. bei Patienten mit rheumatoider Arthritis vorkommt, ist eine immunsuppressive Therapie nur bedingt wirksam. Bei einem Teil der Patienten bleibt aktuell nur eine Lungentransplantation als definitive Therapie.
Schlussfolgerung
Die Diagnostik und Therapie einer CTD-ILD und PH bei Patienten mit autoimmuner Grunderkrankung erfordern ein interdisziplinäres Vorgehen. Die Effektivität einer antifibrotischen Therapie muss in der Zukunft evaluiert werden.
Abstract
Background
Pulmonary involvement in patients with underlying autoimmune diseases poses a major diagnostic and therapeutic challenge to the treating physician. Due to the associated increased mortality risk, early diagnosis is crucial.
Objective
The incidence and mortality rate of connective tissue disease-related interstitial lung diseases (CTD-ILD) and pulmonary hypertension (PH) were evaluated in patients with rheumatic disease including clinical aspects, diagnostic procedure, prognosis and treatment recommendations.
Material and methods
An analysis of remarkable publications was carried out and guidelines are presented.
Results
The CTD-ILD and PH are frequent comorbidities with significantly increased mortality risk, especially in patients with systemic sclerosis (SSc). In primary fibrotic and non-inflammatory CTD-ILD, as occurs especially in patients with rheumatoid arthritis, immunosuppressive therapy is only partially effective. Currently, in some eligible patients only lung transplantation remains as a definitive therapy.
Conclusion
The diagnostics and treatment of CTD-ILD and PH in patients with an underlying autoimmune disease requires an interdisciplinary approach. The effectiveness of antifibrotic treatment needs to be evaluated in the future.
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G. Leuschner erhielt Reisekostenübernahme für Konferenzen von Boehringer Ingelheim, Intermune und Novartis. C. Neurohr ist als Referent tätig und erhält Beraterhonorare von Actelion, AstraZeneca, Boehringer Ingelheim, GlaxoSmithKline, Novartis und Roche.
Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.
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C. Fiehn, Baden-Baden
H.-M. Lorenz, Heidelberg
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Leuschner, G., Neurohr, C. Was der Rheumatologe vom Pneumologen lernen kann. Z Rheumatol 77, 477–483 (2018). https://doi.org/10.1007/s00393-018-0507-5
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DOI: https://doi.org/10.1007/s00393-018-0507-5
Schlüsselwörter
- Interstitielle Lungenerkrankung
- Kollagenose-assoziierte interstitielle Lungenerkrankung
- Usual interstitial pneumonia
- Pulmonale Hypertonie
- Interdisziplinarität