Hamsho Suaad, Alannouf Isam, Ashour Anas A
Faculty of Medicine, Damascus University, Damascus, Syria.
Department of Internal Medicine, Hamad Medical Corporation, Doha, Qatar.
Int Med Case Rep J. 2022 Sep 2;15:473-477. doi: 10.2147/IMCRJ.S365004. eCollection 2022.
Felty syndrome (FS) is a disorder that develops after a long history of erosive rheumatoid arthritis and presents with splenomegaly and neutropenia. In addition to joint deformities, FS causes a variety of extra-articular manifestations such as vasculitis, skin lesions, and lymphadenopathy. FS is also reported to cause non-cirrhotic portal hypertension which may result in variceal bleeding. FS is usually treated by disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate. Herein, we report a case of rapidly deteriorating FS and a severe relapsing neutropenia only a few weeks after discontinuation of methotrexate and other available DMARDs. The patient presented with a fever and a multi-drug resistant gangrenous ulcer consistent with ecthyma gangrenosum. The patient was also found to have hepatosplenomegaly and portal hypertension. The case was managed with antibiotics and symptomatic treatments only as DMARDs were either unavailable or not affordable by the patient. However, the patient's condition did not improve. This case highlights that DMARDs are considered an essential part of preventing infections due to FS neutropenia. Patients with FS should continue DMARDs for life to avoid the relapse of their condition.
费尔蒂综合征（FS）是一种在长期侵蚀性类风湿关节炎病史后出现的疾病，表现为脾肿大和中性粒细胞减少。除关节畸形外，FS还会引起多种关节外表现，如血管炎、皮肤病变和淋巴结病。据报道，FS还会导致非肝硬化性门静脉高压，这可能会导致静脉曲张出血。FS通常用甲氨蝶呤等改善病情的抗风湿药物（DMARDs）治疗。在此，我们报告一例FS迅速恶化且在停用甲氨蝶呤和其他可用的DMARDs仅几周后出现严重复发性中性粒细胞减少的病例。患者出现发热和与坏疽性脓皮病一致的耐多药坏疽性溃疡。还发现该患者有肝脾肿大和门静脉高压。由于患者无法获得或负担不起DMARDs，该病例仅用抗生素和对症治疗。然而，患者的病情并未改善。该病例突出表明，DMARDs被认为是预防FS中性粒细胞减少所致感染的重要组成部分。FS患者应终身持续使用DMARDs以避免病情复发。
