Systemic lupus erythematosus associated with sickle-cell disease: a case report and literature review

Abstract

IntroductionThe occurrence of systemic lupus erythematosus has been only rarely reported in patients with sickle-cell disease.Case presentationWe describe the case of a 23-year-old North-African woman with sickle-cell disease and systemic lupus erythematosus, and discuss the pointers to the diagnosis of this combination of conditions and also present a review of literature. The diagnosis of systemic lupus erythematosus was delayed because our patient’s symptoms were initially attributed to sickle-cell disease.ConclusionsPhysicians should be alerted to the possible association of sickle-cell disease and systemic lupus erythematosus so as not to delay correct diagnosis and initiation of appropriate treatment.

Red blood cell alloimmunization is influenced by the delay between Toll-like receptor agonist injection and transfusion

“…45,46 Moreover, autoimmunity has also been described in SCD patients. [47][48][49] The strong expression of these two molecules may affect alloimmunization, especially when poly(I:C) is injected 7 days before transfusion. Specifically, the injection of TLR3 agonists combined with CD40 stimulation facilitates the secretion of pro-inflammatory cytokines by CD4 + T cells by promoting the expression of CD134.…”

“…DC were fixed and permeabilized with a commercial kit (eBioscience) for intramembranous staining with CD283-PE (Biolegend, San Diego, CA, USA), IL12-FITC and IFNγ-AF700 (BD Biosciences). The HEL protein contains an immunodominant peptide, NR16 (HEL [46][47][48][49][50][51][52][53][54][55][56][57][58][59][60][61] : NTDGSTDYGILQIN-SR). NR16-presenting DC were studied using an AW3.18 antibody.…”

Nephritis, Neuropsychiatric Lupus in Sickler Patient: A Case Report

“…Idiopathic nephrotic syndrome, focal and segmental glomerulosclerosis, membranoproliferative glomerulonephritis are known as renal complication of sickle cell anemia, our patient has lupus nephritis class 4 proved by renal biopsy, Articular involvement is the most common symptom in SLE/SCD patients the percentage is about 84% of cases in literature, less commonly sororities (36%), then glomerulonephritis class III or IV occurred in 11% of SLE /SCD reported cases. In our reported case the articular manifestation was minimal and had no serositis, Cutaneous and neurologic manifestations in patients with SLE and SCD are rare in literature except for some cases with seizures and less commonly polyneuropathy [5] but our patient has discoid lupus , and also presented with severe polyneuropathy and myositis as well and has CNS vascular disease could be due to either lupus or sickle cell disease and has brain atrophy also which most likely due to sickle disease.…”

“…The association between SLE and SCD is uncommon, SLE and SCD are chronic diseases that have several clinical and laboratory findings in common [12], around 40 cases have been reported with SLE and SCD association, which is uncommon, most of them were African women, All cases were diagnosed as lupus at young age and known to have sickle cell disease many years before the diagnosis of lupus [5], we report the described case of Saudi woman with SLE and SCD overlap, in our case the symptoms started with peripheral nervous system involvement then diagnosed with sickle cell disease and severe active SLE wit multisystem involvement (neuropsychiatric lupus and lupus nephritis, thrombocytopenia, anemia, discoid lupus, myositis) our patient was almost asymptomatic for sickle cell disease and was not investigated or admitted to hospital due to sickle crisis or anemia and she did not receive blood transfusion before. Anemia is a common presentation for lupus patients occur with different types and severity, but rarely associated with haemoglobinopathies, hematologic manifestations of SLE are variable [13], lupus patient may have leucopenia, lymphopenia, thrombocytopenia, autoimmune hemolytic anemia (AIHA), which are included in classification criteria of SLE, Thrombotic Thrombocytopenic Purpura (TTP), and myelofibrosis which are not included in SLE classification criteria [14].…”

“…The actual incidence of lupus in patients with sickle cell disease is unknown because The most cases in literature are case reports, the diagnosis of lupus in patients with known to have sickle cell disease is sometimes difficult and delayed due to similar presentations (arthralgia, anemia, central nervous system involvement, renal disease ...etc.) [5]. This is a case of Saudi woman (not known to have sickle cell disease) presented with neurologic symptoms and symptomatic anemia, after further evaluation proved to have SLE and SCD.…”