Prevalence and Clinical Characteristics of Rheumatoid Arthritis in an Inner City Population with Sickle Cell Disease

Abstract

Objectives Rheumatoid arthritis (RA) has been rarely reported in association with sickle cell disease (SCD). Our study aimed to estimate the prevalence of RA in SCD population and to describe the clinical characteristics of RA associated with SCD. Methods Retrospective chart review of SCD and RA patients followed at 2 large urban hospitals. Seven RA/SCD patients were identified and compared to age and sex matched cohort of SCD only and of RA only group. All patients were Black. Results There were 739 SCD cases, seven (0.94%) met ACR criteria for RA (SCD-RA), 411 cases were RA only group. Mean age was significantly higher in SCD-RA compared to the entire population of SCD and RA (41.7 ± 3.9 (± SEM) vs. 33.26 ± 0.47, vs. 61.39 ± 0.79, p<0.01). SCD-RA patients had lower hemoglobin (g/dl) when compared to the age and sex matched SCD or RA only patients (7.4 ± 0.49 vs. 8.3 ± 0.60 vs. 11 ± 0.59, p <0.01) respectively. There were no significant differences in laboratory and treatment approach between SCD-RA and RA only groups, except for the radiographic evidence of periarticular osteopenia and greater difficulty in the activities of daily living (ADL) among SCD-RA cohort, compared to the age and sex matched RA cohort (p=0.01) Conclusion In contrast to older reports, the prevalence of RA among SCD patients in our study (0.94%) was similar to that reported in the general population (0.5–1%) and was to be associated with difficulty in ADL and periarticular osteopenia. Since RA manifests at an older age, our reported prevalence is likely explainable by improved survival of SCD patients due to enhanced medical care and the advent of hydroxyurea as a major therapeutic breakthrough for SCD.

Rheumatoid Arthritis in Sickle-Cell Population: Pathophysiologic Insights, Clinical Evaluation and Management

“…Michel et al performed a retrospective study analyzing connective tissue disease in patients with SCD. There were 15 patients identified with RA coexisting with SCD, with 93% having rheumatoid factor positivity, 88% having anti-citrullinated peptide antibodies and 80% had erosive joint disease [ 25 ].…”

“…The SCD-RA patients were also older than the SCD-only population but younger than RA-only patient population and had been diagnosed with RA at a younger age (about 5 years earlier) than RA-only patients. SCD-RA patients had more difficulty with ADL [ 25 ]. We hypothesize that improved survival of SCD patients resulting from medical care, preventive measures such vaccinations and the introduction of hydroxyurea, have made SCD population susceptible to the development of RA that generally occurs at a more advanced age [ 26 , 27 ].…”

“…The underlying mechanism is largely unknown at this time however, we can propose that ischemia damages SCD patient’s synovium leading to the release of synovial antigen into the circulation thus inducing autoantibody formation. This theory could explain why SCD patients were diagnosed with RA at a younger age (5 years earlier) and tended to be seropositive for RF and anti-CCP than RA only patients [ 25 ].…”

Rheumatoid arthritis and sickle cell disease: A potential association

“…Musculoskeletal manifestations of sickle cell disease (SCD) may delay the diagnosis of inflammatory forms of arthritis, but in the case of persistent joint involvement, RA should be considered as a diagnosis, given the early and irreversible dysfunction related to the disease. Besides the case reports in literature (Table I), two important retrospective studies have demonstrated that a longer life expectancy for SCD patients correlates with a greater increase in the prevalence of RA (Michel et al 1 , 2 . We describe the coexistence of RA with SCD in six Afro‐descendant women (Table II) to emphasise the importance of early diagnosis and specific treatment, given that bone erosions become evident within less than three months of disease duration, particularly in untreated patients.…”

“…The management of RA in SCD patients is a dilemma because of the potential risks and benefits of interventions in terms of infection and immunosuppression. The permanent inflammatory state, as a consequence of the vaso‐occlusive crisis and haemolysis, might be a factor in the severity of the first years of RA 2 …”