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AB0990 Peri-arteritis as a novel paraneoplastic presentation of myelodysplastic syndrome: a case series and review of the literature
  1. N Droz,
  2. C Gota
  1. Rheumatology, Cleveland Clinic Foundation, Cleveland, United States


Background Myelodysplastic syndromes (MDS) are characterized by ineffective and dysplastic hematopoiesis resulting in peripheral blood cytopenias with varying risk for progression to acute myelogenous leukemia (AML)1.

Autoimmune manifestations have been well described in association with MDS2. Peri-arteritis has rarely been reported.

Objectives Here, we present a novel association of MDS with peri-arteritis.

Methods Three patients within our institution and one in the literature were identified. Patients were diagnosed with MDS according to 2008 World Health Organization classification with concomitant occurrence of peri-arteritis on imaging3.

Results See Table 1.

Table 1

Conclusions Autoimmune manifestations associated with MDS occur in 7–25% of patients. The most commonly reported conditions include vasculitis, inflammatory arthritis and connective tissue disease. There is a paucity of literature describing peri-arteritis associated with MDS2. Review of the literature revealed only one other case of peri-arteritis associated with MDS outside of those identified at our facility4.

With this case series, we report peri-arteritis as a novel presentation of MDS. Peri-vascular inflammation may mimic idiopathic retroperitoneal fibrosis, however, persistent cytopenias, lack of response to immunosuppressive therapy and persistence of elevated inflammatory markers should prompt the physician to evaluate for an underlying diagnosis of MDS.


  1. Ades L, Itzykson R, Fenaux P. Myelodysplastic Syndromes. Lancet. 2014;383(9936):2239–52.

  2. Mekinian A, Grignano E, Braun T et al. Systemic inflammatory and autoimmune manifestations associated with MDS. Rheumatology.2016;55(2):291–300.

  3. Swerdlow S, Campo E, Harris N et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2008.

  4. Inoue M, Kano T, Ozaki T et al. Rheumatic manifestations and an epipharyngeal mass accompanied by MDS. Mod Rheumatol. 2015;25(4):653–6.


Disclosure of Interest None declared

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