Systemic Amyloidosis with Renal Failure: A Challenging Diagnosis of SAPHO Syndrome

Abstract

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare, unrecognized and chronic inflammatory disorder characterized by distinct cutaneous and osteoarticular manifestations. Renal complications are uncommon. We describe the unusual case of a patient with severe refractory and chronic hidradenitis suppurativa (HS) that progressed to chronic renal failure due to secondary amyloidosis, presenting with mandibular pain and renal failure. The challenging diagnosis of SAPHO syndrome was made. The purpose of this case report is to emphasize the need for vigilance, timely recognition and multidisciplinary treatment. The fundamental management of AA amyloidosis and SAPHO syndrome requires an individualized approach with control of the underlying inflammatory disease.LEARNING POINTSSystemic amyloidosis is a serious but rare complication of chronic inflammatory disorders such as hidradenitis suppurativa and should be periodically considered.SAPHO syndrome should be suspected in patients with cutaneous and osteoarticular manifestations, and is a challenging diagnosis with exclusion of neoplastic, autoimmune and infectious diseases.The cornerstone of management of AA amyloidosis is vigilance, timely recognition and sometimes aggressive immunosuppressive treatment.