In patients with myelodysplastic syndromes (MDS), an increased risk for certain rheumatologic disorders has been reported, according to findings from a retrospective cohort study published in the journal Rheumatology (Oxford).

A link between certain rheumatologic diseases and hematologic disorders has been identified, with several studies having demonstrated an association between various rheumatologic disorders and MDS. In fact, among individuals with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), an increased risk for MDS has been observed, which is frequently attributed to the use of immunosuppressive agents in these individuals. The exact incidence rates of MDS and the specific risk factors related to the development of MDS, however, remain to be elucidated.

The researchers performed a study among patients who had been diagnosed with a rheumatologic disorder at a tertiary care hospital between May 2009 and July 2022, identifying those individuals who were later diagnosed with MDS. Each patient in the study was age- and sex-matched with 5 controls who were selected from the cohort of patients with the following rheumatologic disorders:

  • RA
  • SLE
  • Behçet’s disease (BD)
  • Sjögren’s syndrome
  • Ankylosing spondylitis (AS)
  • Systemic vasculitis except BD
  • Antiphospholipid syndrome
  • Polymyalgia rheumatica
  • Adult-onset Still’s disease
  • Idiopathic inflammatory myositis
  • Palindromic rheumatism
  • Relapsing polychondritis
  • Psoriatic arthritis
  • Crystal arthropathy
  • Systemic sclerosis
  • Immunoglobin G4 (IgG4)-related disease

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Of the 64 patients evaluated, 35 were women. The median participant age was 57.0 years (range, 41.0-69.0 years). Study results revealed that during the follow-up of 55,841 patient-years (PYs) in individuals with rheumatologic disorders, 64 patients received a diagnosis of MDS, which yielded an incidence rate of 1.15 per 1000 PYs. Among these individuals, the following diagnoses were noted:

  • RA: n=22; MDS incidence rate: 1.38 per 1000 PYs
  • BD: n=10; MDS incidence rate: 1.53 per 1000 PYs
  • SLE: n=10; MDS incidence rate: 1.54 per 1000 PYs
  • AS: n=6; MDS incidence rate: 2.66 per 1000 PYs

Following adjustment for age, participants with SLE exhibited a significantly higher incidence rate of MDS compared with the entire study population with rheumatologic disorders (adjusted hazard ratio [HR], 2.61; 95% CI, 1.19-36.06; P =.01). Although individuals with BD, AS, and vasculitis also had higher incidence rates of MDS compared with the overall population, following age adjustment, the HRs were not statistically significant.

The onset of MDS was reported at a younger age among patients with AS, BD, and SLE than in those with RA. Refractory cytopenia with multilineage dysplasia was the most frequently occurring phenotype, which was observed in 35.9% of patients with MDS. Overall, 54.7% of participants had karyotypes with a favorable prognosis.

In patients with AS, RA, and SLE, lower hemoglobin levels were seen at the time of diagnosis of rheumatologic disease compared with the matched controls. In addition, those with MDS who had BDS or SLE reported higher levels of glucocorticoid use than control patients.

“SLE is a significant risk factor for MDS among patients with rheumatologic diseases,” the researchers noted. “A lower hemoglobin level at the time of diagnosis of rheumatologic disease was associated with the future development of MDS,” they concluded.


Kim Y-E, Ahn SM, Oh JS, et al. Incidence of and risk factors for myelodysplastic syndromes in patients with rheumatologic diseases. Rheumatology (Oxford). Published online July 27, 2023. doi:10.1093/rheumatology/kead374