Amyloidose in der Pneumologie

Abstract

Amyloidosis is a rare disorder within the field of pneumology, however, it is estimated that there are a relevant number of unreported cases. In routine clinical practice, the disease is seldom considered in the diagnostic work-up of unclear respiratory symptoms and radiological findings. For amyloidosis to be considered as a differential diagnosis, the clinician must be aware of the broad variety of its clinical manifestations. In cases where amyloidosis is suspected, it is important to obtain an early tissue biopsy. If the diagnosis can be confirmed by Congo red staining, further subtyping of the amyloid protein is necessary in order to identify a treatable cause of the disease. The amyloidoses are classified according to the type of amyloid protein, with clinical subclassifications distinguishing hereditary from acquired forms and localised from generalised manifestations. Apart from causal therapy of the primary disease, the treatment of generalised amyloidosis includes specific systemic therapy. The majority of localised forms are treated with specific local interventions showing good long-term results. This review outlines the pathophysiology, classification, diagnostic pathways and therapeutic modalities in amyloidosis. Furthermore, typical manifestations of amyloidosis of the lung and the specific treatment options are discussed.