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Purpose: Systemic amyloidosis represents a yet poorly characterized complex and multifaceted rare multisystem disease with high mortality. Clinical presentation, clinical course, and treatment vary within and across subtypes. Established biomarkers are predominantly non-specific. Diagnosis and management of these patients require specifically tailored interdisciplinary structures. Well-characterized patient cohorts are needed to optimize diagnostic tools and treatment options. Method: The prospective cohort study AmyKoS was established as a monocentric observational study for patients with suspected and proven systemic and localized amyloidosis at the Interdisciplinary Amyloidosis Centre of Northern Bavaria in 2018. We here describe the study rationale and design based on the underlying clinical structures. The primary aim is to establish a large in-detail characterized patient population with appropriate biomaterial resources. The primary endpoint is overall survival; secondary endpoints are disease-associated complications, prevalence and incidence of ICD-10-coded psychological disorders and characterization of organ involvement and response. Results/conclusions: AmyKoS serves as an umbrella structure for clinical and translational research. It differs at least partly from other amyloidosis cohort studies by its cross-subtype approach and is unique regarding the inclusion of patients under suspicion regardless of confirmation of amyloidosis. Therefore, AmyKoS simultaneously generates a negative control group for the evaluation of diagnostic markers and methods and allows to test their generalizability to all subtypes. As a special feature, the multi-step AMY-NEEDS substudy systematically evaluates the established structures with the aim of improving patient-orientated care and the interfaces with referring physicians. The characteristics of the AmyKoS cohort are congruent with the findings of large clinical trials. [ABSTRACT FROM AUTHOR]