Kodithuwakku G D E, Fonseka C L, Nanayakkara S, Herath H M M
University Medical Unit, Teaching Hospital Karapitiya, Galle, Sri Lanka.
Department of Medicine, Faculty of Medicine, University of Ruhuna, Galle, Sri Lanka.
Case Rep Rheumatol. 2020 Feb 19;2020:8327068. doi: 10.1155/2020/8327068. eCollection 2020.
Adult onset Still's disease (AOSD) is a rare inflammatory disorder with a variety of clinical presentations. Even though pneumonitis and pleurisy are known to occur in AOSD, pulmonary onset presentations are exceedingly rare. . We present a 40-year-old male, presenting with fever and bilateral alveolar shadows with pleural effusions mimicking community-acquired severe pneumonia. He was initially treated as severe pneumonia with poor response to broad spectrum antibiotics. Subsequently, he was managed as AOSD-induced pneumonitis, as he fulfilled Yamaguchi criteria. Few weeks later, he developed macular rash and arthralgia with generalized lymphadenopathy with lymph node histology, showing Kikuchi lymphadenitis. He responded well to steroids and had a complete recovery.
Non-infective causes of pneumonitis should be suspected in the setting of poorly resolving pneumonias, especially when microbiological and serological investigations does not support an infective etiology. Presence of systemic symptoms with arthralgia, rash, and disproportionately elevated ferritin level supports the diagnosis of AOSD. Kikuchi lymphadenitis is a reported association with AOSD, and there could be a causal link between the two disorders.
成人斯蒂尔病(AOSD)是一种罕见的炎症性疾病,临床表现多样。尽管已知AOSD可发生肺炎和胸膜炎,但肺部起病的表现极为罕见。我们报告一名40岁男性,表现为发热及双侧肺泡阴影伴胸腔积液,类似社区获得性重症肺炎。他最初被当作重症肺炎治疗,对广谱抗生素反应不佳。随后,由于他符合山口标准,被诊断为AOSD诱发的肺炎。几周后,他出现斑丘疹、关节痛及全身淋巴结肿大,淋巴结组织学检查显示为菊池淋巴结炎。他对类固醇治疗反应良好,完全康复。
在肺炎难以缓解的情况下,应怀疑非感染性肺炎病因,尤其是微生物学和血清学检查不支持感染性病因时。存在关节痛、皮疹等全身症状以及铁蛋白水平不成比例升高支持AOSD的诊断。菊池淋巴结炎是报道的与AOSD相关的疾病,两者之间可能存在因果关系。