Coexistence of lupus nephritis and sickle cell trait, an electron microscopic assessment of renal glomerular damage: Case report of a rare association

Abstract

Because of similarities of the musculoskeletal, central nervous system, and renal manifestations in both diseases, diagnosing systemic lupus erythematosus (SLE) in sickle cell disease (SCD) patients can be difficult to establish. Although Sickle Cell Trait (SCT) is still considered a benign form of SCD, its impact on kidney injury and other renal manifestations is well recognized in the literature. In this case report, we look at the challenges that develop when diagnosing patients with a concurrence of both diseases and the importance of early recognition and treatment of lupus nephritis in SCT patients. We present a case of a male patient with sickle-cell trait who was admitted to our hospital complaining of low grade fever and pancytopenia for investigations proven to be SLE. A renal biopsy on electron microscopy assessment with different staining modalities as well as immune fluorescence revealed mixed pathological changes. We emphasize the importance in considering the presence of a coexisting autoimmune disease in a patient with sickle hemoglobinopathies even in the milder forms like SCT which may display an atypical and/or multisystem presentation. Also, the impact of the two conditions on the renal pathological changes should be expected to be more damaging even at early onset of SLE flare, and hence, an urge to start with more intensified immunosuppressive medications.

Coevality of Systemic Lupus Erythematosus With Sickle Cell Trait: A Not So Uncommon Entity

“…With the overlapping clinical features and reporting of ANA positivity in patients with sickle cell disease (SCD), diagnosing lupus is indeed very challenging to the clinicians at large [3,5]. However, very few cases of lupus are reported with the SCT variant of hemoglobinopathies [4]. Patients with SCD have an abnormal alternate pathway of the complement system and this may make them prone to the development of autoimmune diseases [3,6].…”

“…Sickle hemoglobinopathies, a group of commonly encountered genetic disorders that include sickle cell trait, sickle cell disease, and sickle Beta−Thalassemia, are prevalent in this eastern part of India [1,2]. Due to the multisystem involvement and manifestations, the diagnosis of lupus in patients with hemoglobinopathies may often get delayed [3,4]. We report a case of known sickle cell trait whose clinical features and subsequent presentations lead to the diagnosis of lupus.…”