Background: Amyloid fibrils are protein polymers. Functional amyloids play a beneficial role in a variety of physiologic processes. Amyloidosis results from the accumulation of pathogenic amyloids in a variety of tissues. Case Presentation: The authors present a case of a 60-year-old male with a pseudotumoral silicosis, a history of intermittent fever, and renal insufficiency with intermittent proteinuria (maximum of 3 g/dl). The diagnosis of amyloidosis was made from multiple biopsies. During the investigation, it was never possible to characterize the subtype of amyloidosis [the patient had findings either in favor of AL amyloidosis and AA amyloidosis (AA)]. A definite diagnosis was only obtained from an autopsy, and AA was found to be the subtype. Conclusion: At the time of this publication and to the best of authors’ knowledge, AA has never been described to be associated with pseudotumoral form of pulmonary silicosis. We believe that an inflammatory response associated with pulmonary silicosis was the trigger to the development of AA.

Keywords: Amyloidosis AA, amyloidosis AL, systemic amyloidosis, pseudotumoral silicosis