What autoimmune syndromes have been reported to occur in patients with a myelodysplastic syndrome (MDS)?
MDS is characterized by ineffective hematopoiesis, abnormal cell morphology, and a propensity to develop acute leukemia. Autoimmunity and inflammation can occur in up to 25% of patients. The etiology is unclear but is possibly related to the accelerated apoptosis of maturing hematopoietic cells in MDS causing an increase in the cytokine production (e.g., interleukin-6, tumor necrosis factor-α, interferon-γ). In a recent case series of MDS patients who had one or more of these systemic and autoimmune conditions, the frequency of symptoms/diseases occurring in this patient series included:
- • Constitutional symptoms (35%) including noninfectious fever.
- • Systemic vasculitides (32%): polyarteritis nodosa, giant cell arteritis.
- • Connective tissue diseases (25%): relapsing polychondritis, systemic lupus erythematosus.
- • Inflammatory polyarthritis (23%): polymyalgia rheumatica, rheumatoid arthritis, remitting seronegative symmetrical synovitis with pitting edema syndrome.
- • Neutrophilic disease (10%): Sweet’s syndrome, pyoderma gangrenosum.
- • Other syndromes: unclassifiable, autoimmune hemolytic anemia, pleuropericarditis, bronchiolitis obliterans, uveitis, myositis, neuropathy, glomerulonephritis, positive serologies (antinuclear antibody, antineutrophil cytoplasmic antibody, rheumatoid factor, antiphospholipid antibodies, cryoglobulins).
Many of these patients improve considerably with GCs, which are often used in combination with synthetic disease-modifying antirheumatic drugs (e.g., hydroxychloroquine, methotrexate) and occasionally biologics in order to decrease the GC dose without relapse.
