Jain Harsh, Roy Debaditya, Mavidi Sunil, Haldar Subhankar, Mondal Sumantro, Bhattacharya Paramita, Ghosh Alakendu
Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India.
Genetics Service Unit, National Institute of Biomedical Genomics, Kalyani, India.
Mediterr J Rheumatol. 2024 Sep 30;35(3):490-493. doi: 10.31138/mjr.271223.eos. eCollection 2024 Sep.
We report the case of a 67-year-old male with a two-year history of inflammatory polyarthritis, fatigue, and low back pain. He also had a history of biopsy proven neutrophilic dermatosis in the past. On admission and examination, he had pallor. Laboratory evaluation showed macrocytic anaemia, elevated erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). MRI of Sacroiliac joints showed presence of bilateral sacroiliitis. Bone marrow examination showed the presence of cytoplasmic vacuolisation in myeloid and erythroid precursor cells. Genetic analysis confirmed a diagnosis of VEXAS syndrome. He improved with prednisolone and Sulfasalazine with no further relapse on follow up. This case report highlights the importance of considering VEXAS syndrome in older adults with presentation of spondyloarthritis and macrocytic anaemia. Early diagnosis and treatment with corticosteroids and steroid-sparing agents can lead to significant improvement in symptoms and are important for a good outcome.
我们报告了一例67岁男性病例，该患者有两年的炎性多关节炎、疲劳和腰痛病史。他过去还有活检证实的嗜中性皮肤病病史。入院检查时，他面色苍白。实验室检查显示大细胞性贫血、红细胞沉降率（ESR）升高和C反应蛋白（CRP）升高。骶髂关节MRI显示双侧骶髂关节炎。骨髓检查显示髓系和红系前体细胞存在细胞质空泡化。基因分析确诊为VEXAS综合征。他使用泼尼松龙和柳氮磺胺吡啶后病情好转，随访期间未再复发。本病例报告强调了在出现脊柱关节炎和大细胞性贫血的老年人中考虑VEXAS综合征的重要性。早期使用皮质类固醇和类固醇节省剂进行诊断和治疗可显著改善症状，对取得良好预后很重要。
