[Autoinflammatory syndromes and AA amyloidosis]

Autoinflammatory syndromes (AIS) are characterized by uniform attacks often with febrile episodes, exanthema, abdominal pain, muscle and joint pain. Patients show markedly elevated levels of the inflammatory serum parameters C‑reactive protein (CRP) and systemic amyloid A (SAA) during an attack. The origin of the family of the patient and the duration of the attacks are helpful to find the appropriate diagnosis. Molecular genetic tests are used to confirm the clinical diagnosis of an AIS. Colchicine can prevent attacks of familial Mediterranean fever but not the other forms of AIS. In refractory cases anakinra or canakinumab can be used to control the inflammatory exacerbations. Systemic AA amyloidosis can develop secondary to any insufficiently treated chronic inflammatory disease. Renal involvement is the predominant initial organ dysfunction, which can be detected early on by the evaluation of proteinuria. If AA amyloidosis can be diagnosed early and successfully treated, the renal function and the function of other organs can be preserved for many years. In patients with advanced AA amyloidosis renal failure with the subsequent necessity for dialysis can often no longer be prevented. These patients should be treated to prevent involvement of the stomach, intestines and heart.