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

Abstract

The coexistence of systemic lupus erythematosus (SLE) with sickle cell trait is quite sparingly reported in literature. Here, we narrate the case of a 17-year-old girl from Eastern India with sickle cell trait who presented with acute lupus pneumonitis. The challenges to the final diagnosis of SLE with sickle cell trait were because of the often lesser degree of clinical suspicion at the outset. In this report, we discuss this not so uncommon combination of conditions and review related literature. This girl, who was a known case of sickle cell trait, presented with fever, cough, shortness of breath with subsequent rashes, oral ulceration, high erythrocyte sedimentation rate (ESR) and proteinuria. After ruling out infective causes, she was found to be antinuclear antibody (ANA) positive and with stage 4 lupus nephritis. Emphasis should be given to the presence of autoimmune conditions in patients with sickle hemoglobinopathies, including sickle cell trait wherein atypical or systemic involvement may occur. Such association holds more importance as sickle hemoglobinopathies is one of the major hemoglobinopathies reported in this part of the country.

Coexistence of Sickle Cell Thalassaemia with Overlapping Syndrome: A Case Report of Systemic Lupus Erythematosus and Autoimmune Hepatitis

“…2,9 While the exact mechanisms responsible for these interactions are not fully understood, it is hypothesised that individuals with these interactions may possess an aberrant alternate pathway within their complement system, potentially heightening their vulnerability to autoimmune disease development. 4,9 Another hypothesis posits that individuals afflicted with this condition may have an immune system that does not function optimally. This immune dysfunction may be attributed to factors such as diminished spleen function, disruptions within the complement pathway, and compromised processes of opsonisation and phagocytosis, contributing to the pathogenesis.…”

“…• susceptible to infections, and potentially prone to autoimmune disorders. 4,9 Patients with SCD may also experience hepatic crises due to red blood cell sickling, leading to hepatomegaly, elevated liver enzyme levels (serum transaminases), or cholestasis. 10 Liver issues in these patients can result from various factors, including hepatic sequestration, viral hepatitis from blood transfusions, haemosiderosis (iron accumulation in the liver), gallstones, or unrelated medical conditions.…”

“…1,2 SCD encompasses several other variants, including sickle cell trait, sickle cell anaemia, and sickle cell Hb-C disease, each with distinct characteristics. 3,4 Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterised by autoantibodies binding to tissues, forming immune complexes, and causing damage across various organs, leading to chronic inflammation. 5 The disease is more prevalent in females, with a female-to-male ratio of 9:1.…”

Autoimmune disease and sickle cell anaemia: ‘Intersecting pathways and differential diagnosis’

“…The association of SLE and SCD has been thought to be rare overall; however, several reports document an association 17,18 . For example, a large review of the literature reported on 45 cases of SLE in SCD 19 .…”

“…2,15,16 The association of SLE and SCD has been thought to be rare overall; however, several reports document an association. 17,18 For example, a large review of the literature reported on 45 cases of SLE in SCD. 19 One large retrospective study (>350 patients) on SCD patients showed that in a single centre, five cases of SLE were identified.…”