The spectrum of renal dysfunctions in sickle cell disease: Aetiopathogenesis and management of sickle cell nephropathy and non-sickle cell nephropathies in patients with sickle cell disease

Abstract

Renal dysfunction (RD) is a common complication of sickle cell disease (SCD). Literature regarding RD in SCD is dominated by 'sickle cell nephropathy' (SCN), with little reference to 'non-sickle cell nephropathies' (NSCNs) that also affect SCD patients. This overview aims to present a comprehensive but concise narrative review of aetiopathogenesis and management of both SCN and NSCNs in SCD patients. Literature was searched using terms relevant to RD in SCD. Only articles that examined aetiology, pathogenesis, and/or management of acute and chronic RD in SCD were selected. In addition to SCN, SCD patients incur a range of acute and chronic NSCNs due to separate or combined effects of rhabdomyolysis, infection, hyperuri-cemia, analgesics, autoimmune disorders, and amyloidosis. Hence, RD in SCD patients may be caused by SCN or NSCN or both. Despite differences in aetiopathogenesis and treatment, SCN and NSCNs share similar clinical features. Thus, NSCNs can be mis-diagnosed as SCN, while SCN can mask and delay diagnosis of NSCN if the two disorders coexist. While SCN remains the prototype RD in SCD, a myriad of NSCNs also cause RD in patients with SCD. Although SCN and NSCNs share similar clinical features, the two conditions are managed differently. Therefore, SCD patients presenting with RD should always be broadly investigated for both SCN and NSCNs. This strategy will ensure accurate diagnosis and differentiation between SCN and NSCNs, which is essential for providing 'cause-specific' management of RD in patients with SCD.