*Corresponding author:
Chisato Ashida, Department of Hematology and Rheumatology, Kindai University School of Medicine, 377-2 Oonohigashi, Osaka-Sayama, Osaka, 589-0014 JapanReceived: October 08, 2018; Published: October 24, 2018
DOI: 10.26717/BJSTR.2018.10.001932
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It is known that the causative diseases of secondary hypertrophic pachymeningitis (HP) include infections, malignant tumors, autoimmune diseases. Although myelodysplastic syndromes (MDS) are often accompanied by certain autoimmune diseases, secondary HP with MDS is rare. We here describe the patient with HP complicated by MDS and have severe resistance to the steroid therapy and successfully treated with the combination of corticosteroids and 5-Azacytidine (5-Aza). Administration of 5-Aza might be a useful therapeutic option for patients with refractory autoimmune diseases complicated with MDS.
Keywords: Autoimmune Diseases; Hypertrophic Pachymeningitis; Myelodysplastic Syndrome; Azacytidine
Abbreviations: HP: Hypertrophic Pachymeningitis; MDS: Myelodysplastic Syndromes; 5-Aza:5-Azacytidine; CRP:C-Reactive Protein; PR3-ANCA:PR3-Anti-Neutrophil Cytoplasmic Antibody; MPO-ANCA: MPO-Anti-Neutrophil Cytoplasmic Antibody; CE-MRI: Contrast Enhanced Magnetic Resonance Imaging; PSL: Prednisolone; mPSL: methyl-Prednisolone; AZA: Azacytidine
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