Coexisting systemic lupus erythematosus and sickle cell disease: Case report and literature review

Abstract

r e v b r a s r e u m a t o l . 2 0 1 5;5 5(1):68-74 REVISTA BRASILEIRA DE REUMATOLOGIA w w w . r e u m a t o l o g i a . c o m . b r Sickle cell disease Systemic lupus erythematosus Hemoglobinopathies a b s t r a c t Objective: To report a case of coexisting systemic lupus erythematosus (SLE) and sickle cell disease (SCD) with a review of the literature on the topic.Methodology: Case report and literature review of the association between SLE and SCD through scientific articles in health sciences databases, such as LILACS, MEDLINE/Pubmed and Scielo, until May 2012. Descriptors used: 1. Sickle cell anemia; 2. Sickle cell disease; 3. Systemic lupus erythematosus; 4. Hemoglobinopathies. Results: The authors describe an association between SLE and SS hemoglobinopathy in an eight-year-old female patient presentingarticular, hematologic and neuropsychiatric manifestations during clinical evolution. Forty-five cases of association between SLE and SCD are described in literature, mostly adults (62.2%), women (78%) and with the SS phenotype in 78% of the cases, and diverse clinical manifestations. Compared with our patient, articular, hematologic and neuropsychiatric manifestations were present in 76%, 36% and 27% of the cases, respectively.Conclusion: SLE and SCD are chronic diseases that have several clinical and laboratory findings in common, meaning difficult diagnosis and difficulty in finding the correct treatment.Although the association between these diseases is not common, it is described in literature, so it is imperative that physicians who treat such diseases be alert to this possibility. Coexistência de lúpus eritematoso sistêmico e doença falciforme: relato de caso e revisão da literaturaPalavras-chave: Anemia falciforme Doença falciforme r e s u m o Objetivo: relatar um caso de coexistência de lúpus eritematoso sistêmico (LES) e doença falciforme (DF) com revisão da literatura sobre o tema. Metodologia: relato de caso e pesquisa da associação entre LES e DF na literatura, através de artigos científicos nas bases de dados de ciências da saúde, como LILACS, MEDLINE/Pubmed r e v b r a s r e u m a t o l . 2 0 1 5;5 5(1):68-74 69 Lúpus eritematoso sistêmico Hemoglobinopatias e Scielo, até maio de 2012.Descritores utilizados: 1. anemia falciforme; 2. doença falciforme; 3. lúpus eritematoso sistêmico; 4. hemoglobinopatias. Resultados: os autores descrevem a associação de LES e hemoglobinopatia SS em paciente do sexo feminino, oito anos, apresentando manifestaç ões articulares, hematológicas e neuropsiquiátricas durante a sua evolução clínica. Na literatura são descritos 45 casos de associação entre LES e DF, sendo a maioria em mulheres (78%) adultas (62,2%), apresentando fenótipo SS em 78% dos casos e com manifestaç ões clínicas variadas. Comparando com a nossa paciente, manifestaç ões articulares, hematológicas e neuropsiquiátricas, estiveram presentes em 76%, 36% e 27% dos casos, respectivamente. Conclusões: LES e DF são doenças crônicas que apresentam diversos achados clínicos e laboratoriais em comum, implica...

Nephritis, Neuropsychiatric Lupus in Sickler Patient: A Case Report

“…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].…”

Sickle cell disease and systematic lupus erythematous association in a 14-year-old adolescent female

“…SLE is defined as a rare, chronic autoimmune multi-organ manifestations. 7,8 Incidence of SLE in paediatric population is 0.3-0.9 per 100.000 children and prevalence 3.3-8.8 per 100.000 children. 9 SCD and SLE rarely coincide in literature due to the limited number of cases and the overlapping of symptoms of both diseases.…”

“…Females contributed to 78 per cent of cases, while only 22 per cent were male. 8 Majority affecting child bearing age, with a mean of 23-year-old. 10 The combination of both diseases reduces the life span of patients.…”

“…Encapsulated bacteria that lead to repeated infections may be a relationship between the autoimmune disease and haemoglobin S. [12][13][14] Articular manifestations like joint pain are common in both diseases. 8 SCD patients complain of joint pain and are most often diagnosed as vaso-occlusive crisis consequently detain the diagnosis of SLE. 8 In our patient, back pain was the main complaint which was dull, aching and not radiating.…”

“…8 SCD patients complain of joint pain and are most often diagnosed as vaso-occlusive crisis consequently detain the diagnosis of SLE. 8 In our patient, back pain was the main complaint which was dull, aching and not radiating. She was diagnosed with SLE after SCD diagnosis by six years.…”