Profiles of systemic lupus erythematosus patients with co-existing sickle cell disease: a coincidence or true association?

Introduction: Systemic lupus erythematosus (SLE) and sickle cell disease (SCD) are distinct multisystemic diseases that commonly affect blacks. There are few reports of their co-existence in Western literature and a paucity of reports in Sub-Saharan Africa. Their co-existence is associated with diagnostic delay and treatment dilemmas. The aim is to describe the clinical, laboratory, and treatment profile of Nigerian lupus with sickle cell disease.

Material and methods: A 7-year retrospective descriptive study of lupus patients with sickle cell disease was performed. Medical records of eligible patients were extracted into a proforma, transferred into SPSS, and analyzed with descriptive statistics. Sociodemographic, clinical, laboratory, and treatment data were presented as frequency and percentages.

Results: Twelve SLE-SCD cases (female 11, male 1) were identified. The mean age was 28.5 years and the mean duration of illness prior to diagnosis was 9.5 years. The median follow-up period was 3.1 years and the common presentations were mucocutaneous (66%), renal, (50%) serositis (33%), and neurological (16%) in decreasing order. All had anemia and positive antinuclear antibody, 33% had pancytopenia and 75% had positive anti-dsDNA and anti-Smith. Two are on maintenance hemodialysis, one with interstitial lung disease, and one on long-term anticoagulation due to deep vein thrombosis.

Conclusions: Sickle cell disease and SLE should be considered in SCD with atypical clinical and laboratory features. We hope this report will raise diagnostic suspicion and prompt early diagnosis and treatment to prevent multiorgan damage that may ensue from such an association.