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[What rheumatologists can learn from pneumologists]
1Medizinische Klinik und Poliklinik V, Campus Großhadern, Klinikum der Universität München, Marchioninistr. 15, 81377, München, Deutschland. Gabriela.leuschner@med.uni-muenchen.de.
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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