Arthritis & Rheumatism, Volume 60,
October 2009 Abstract Supplement

The 2009 ACR/ARHP Annual Scientific Meeting
Philadelphia October 16-21, 2009.


A Study of Myelodysplastic Syndrome Patients and Rheumatologic Phenomena

Burns,  Christopher M., Albert,  Daniel A.

Purpose:

Patients with myelodysplastic syndrome (MDS) frequently have associated rheumatic diseases, including rheumatoid arthritis and polymyalgia rheumatica. In some (and perhaps all) patients, the cytopenias in MDS are immune-mediated. It is theorized that immune activation in the bone marrow in MDS may lead to systemic autoimmunity and rheumatic manifestations. Additionally, with the advent of stimulating factor therapy to reduce transfusion requirements, treatment-related autoimmune conditions are becoming more common in MDS. We reviewed the rheumatologic findings in all the MDS patients in our institution over a 2 year period.

Method:

We conducted an IDX (238.75 myelodysplastic syndrome) based search of the records from Dartmouth Hitchcock Medical Center from 2006 to 2008 to identify patients with MDS, and then reviewed each chart for rheumatologic phenomena

Results:

We found 7 patients with bone marrow biopsy-proven MDS. All patients were older, 58 to 87 years of age, and 5 of 7 have died. Two patients had necrotizing vasculitis one with a lung nodule and the other with mononeuritis multiplex. One male patient had palmar fasciitis with exudative pleural effusions (non malignant) similar to ovarian carcinoma. One patient had inflammatory arthritis negative for crystal, malignancy or infection. One patient had steroid responsive extremity pain similar to hypertropic osteoarthropathy with no evidence of periarthritis. One patient had nonmalignant mediastinitis with exudative pleural effusions. One patient had typical ANCA+ Wegener's Granulomatosis.

Conclusion:

MDS patients had a wide range of rheumatic findings and every patient had some immune-mediated phenomena, but only one patient had a typical rheumatic disease. In some cases, treatment of the rheumatic disease ameliorated the MDS. Immune activation within the bone marrow milieu is a feature of MDS. A subset of patients responds to immunosuppressive agents with reduced transfusion requirements and slower progression to acute myelocytic leukemia. Multiple components of the immune system are involved, including cytolytic CD8 cells directed at tumor neoantigens expressed on hematopoietic cells with the trisomy 8 mutation, cytokines such as TNF-a, and NK cells. It is postulated that local immune activation induces systemic autoimmunity and rheumatic manifestations. We conclude that a host of rheumatic conditions, both classic and atypical, can occur in the setting of MDS or its treatment. Rheumatologists should be vigilant for MDS when diagnosing rheumatic conditions, particularly atypical presentations or in the setting of unexplained cytopenias. We propose an MDS-related Autoimmune Disease Registry as a data-base for these cases with the hope that patterns will emerge to help elucidate the pathogenesis of both MDS and rheumatic diseases.

To cite this abstract, please use the following information:
Burns, Christopher M., Albert, Daniel A.; A Study of Myelodysplastic Syndrome Patients and Rheumatologic Phenomena [abstract]. Arthritis Rheum 2009;60 Suppl 10 :794
DOI: 10.1002/art.25874

Abstract Supplement

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2009 ACR/ARHP