Inflammatory Arthritis in Patients With Myelodysplastic Syndromes

Mékinian, A.; Braun, Thorsten; Decaux, Olivier; Falgarone, Géraldine; Toussirot, Éric; Raffray, L.; Omouri, Mohamed; Gombert, Bruno; Wazières, B. de; Buchdaul, Anne-Laure; Ziza, Jean-Marc; Launay, David; Denis, Guillaume; Madaule, S.; Rose, Christian; Grignano, Éric; Fenaux, Pierre; Fain, Olivier

doi:10.1097/md.0000000000000011

Cited by 46 publications

(14 citation statements)

References 34 publications

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“…One third of the patients had their AIC diagnosed at the same time as their MDS while another third of the patients were diagnosed before or after MDS diagnosis, respectively [8]. This finding is similar to previous reports that noted short latency between the onset of myeloid and rheumatological disorders [5,9]. …”

Section: Introductionsupporting

confidence: 82%

Autoimmunity and Inflammation in Myelodysplastic Syndromes

Wolach

Stone²

2016

Acta Haematol

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Autoimmune and inflammatory conditions (AICs) are encountered in up to 25% of patients with myelodysplastic syndromes (MDS). A wide range of AICs have been reported in association with MDS and can range from limited clinical manifestations to systemic diseases affecting multiple organs. Vasculitides, connective tissue diseases, and inflammatory arthritis are frequently reported in different studies; noninfectious fever and constitutional symptoms at presentation are common. Associations between AICs and specific MDS characteristics vary by study, but the available data suggest that AICs cluster more often in younger patients with higher-risk MDS. In general, AICs do not seem to confer worse survival, although certain AICs may be associated with adverse outcome (e.g. vasculitis) or progression of MDS (Sweet's syndrome). Nonetheless, these complications may have a significant impact on quality of life and affect the timing and type of MDS-directed therapy. The mainstay of management of these complications in the short term relies on immunosuppressive drugs. Increasing evidence suggests that hypomethylating agents may be effective in treating these complications and reduce steroid dependence. While the pathogenesis of AICs is incompletely understood, growing appreciation of cellular immune deregulation, cytokine hypersecretion, and the genetic heterogeneity underlying MDS may improve our understanding of common pathways linking MDS, inflammation, and autoimmunity.

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Section: Introductionsupporting

confidence: 82%

Autoimmunity and Inflammation in Myelodysplastic Syndromes

Wolach

Stone²

2016

Acta Haematol

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“…Complete/ partial remission (CR/PR) of AID after Azacitidine were defined by complete/partial disappearance of clinical or biological signs with stable dose or decrease/discontinuation of steroids or IST, as previously defined [2]. Hematological response to Azacitidine was classified according to IWG 2006 criteria [18].…”

Section: Methodsmentioning

confidence: 99%

“…Most common AID associated with MDS/CMML include relapsing polychondritis, vasculitis, non-erosive and seronegative arthritis [2,3] and Sweet's syndrome [4]. While AID associated with MDS/CMML usually respond to corticosteroids [2], many patients remain steroid dependent or resistant, requiring additional IST, a situation which increases the risk of severe cytopenias and infections in the context of MDS/CMML [1].…”

Section: Introductionmentioning

confidence: 99%

Efficacy of Azacitidine in autoimmune and inflammatory disorders associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

et al. 2016

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This retrospective study describes efficacy of Azacitidine on autoimmune disorders (AID) associated with MDS/CMML in 22 patients. Response of AID to Azacitidine was observed in 19 patients (86%). Reduction or discontinuation of steroids and/or immunosuppressive therapy (IST) was possible in 16 cases (73%). Hematologic response was seen in 55% of the patients. MDS/CMML and AID evolution was concordant in 13 cases (59%): both favorable (n=11), both unfavorable (n=2), but AID improved while MDS/CMML worsened (n=8) and vice versa (n=1). Azacitidine frequently seems effective in controlling steroid-dependent AID associated with MDS/CMML, but prospective studies are necessary to confirm those findings.

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“…Although this is a common disease in the elderly, the observations suggested that those associations were not fortuitous. 4 Interestingly, a recent literature review reported that arthritis preceded MDS in 55% of cases, and that both the pathologies were concomitantly diagnosed in 27% of cases, 4 which suggests that the existence of inflammation precedes the appearance of MDS. Similarly, MDS and inflammatory bowel disease (IBD) are frequently diagnosed simultaneously.…”

Section: The Inflammatory and Autoimmune Nature Of Mdsmentioning

confidence: 99%

Deregulation of innate immune and inflammatory signaling in myelodysplastic syndromes

et al. 2015

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Myelodysplastic syndromes (MDSs) are a group of heterogeneous clonal hematologic malignancies that are characterized by defective bone marrow (BM) hematopoiesis and by the occurrence of intramedullary apoptosis. During the past decade, the identification of key genetic and epigenetic alterations in patients has improved our understanding of the pathophysiology of this disease. However, the specific molecular mechanisms leading to the pathogenesis of MDS have largely remained obscure. Recently, essential evidence supporting the direct role of innate immune abnormalities in MDS has been obtained, including the identification of multiple key regulators that are overexpressed or constitutively activated in BM hematopoietic stem and progenitor cells. Mounting experimental results indicate that the dysregulation of these molecules leads to abnormal hematopoiesis, unbalanced cell death and proliferation in patients' BM, and has an important role in the pathogenesis of MDS. Furthermore, there is compelling evidence that the deregulation of innate immune and inflammatory signaling also affects other cells from the immune system and the BM microenvironment, which establish aberrant associations with hematopoietic precursors and contribute to the MDS phenotype. Therefore, the deregulation of innate immune and inflammatory signaling should be considered as one of the driving forces in the pathogenesis of MDS. In this article, we review and update the advances in this field, summarizing the results from the most recent studies and discussing their clinical implications.

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Inflammatory Arthritis in Patients With Myelodysplastic Syndromes

Abstract: Supplemental Digital Content is available in the text.

Cited by 46 publications

References 34 publications

Autoimmunity and Inflammation in Myelodysplastic Syndromes

Autoimmunity and Inflammation in Myelodysplastic Syndromes

Efficacy of Azacitidine in autoimmune and inflammatory disorders associated with myelodysplastic syndromes and chronic myelomonocytic leukemia

Deregulation of innate immune and inflammatory signaling in myelodysplastic syndromes

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