Internal Medicine Department, Rush University Medical Center, Chicago, IL, USA.
Nephrology Department, Rush University Medical Center, Chicago, IL, USA.
J Med Case Rep. 2022 Oct 14;16(1):370. doi: 10.1186/s13256-022-03606-1.
Collapsing glomerulopathy, characterized by marked hypertrophy and hyperplasia of the podocytes with eventual collapse of the glomerular tuft, is an important cause of end-stage renal disease. Among the many causes of collapsing glomerulopathy, autoimmune diseases, such as systemic lupus erythematosus, have been implicated. There are also rare reports of adult-onset Still's disease, an autoinflammatory condition characterized by fever, rash, and inflammatory arthritis being associated with collapsing glomerulopathy.
Herein, we present a review of three published cases, and present a new case of a 15-year-old African American female patient with collapsing glomerulopathy who was diagnosed with adult-onset Still's disease 12 years later when she presented with fevers, arthralgias, sore throat, lymphadenopathy, hepatocellular injury, and elevated serum ferritin. Her collapsing glomerulopathy was initially well controlled following induction therapy with cyclosporine and prednisone and maintenance therapy with losartan. However, after developing adult-onset Still's disease, she had multiple flare-ups despite various immunosuppressive therapies and developed worsening renal function, eventually progressing to end-stage renal disease.
Our case-based review highlights a rare but important association between adult-onset Still's disease and collapsing glomerulopathy, and postulates a possible pathophysiological link.
塌陷性肾小球病的特征是足细胞明显肥大和增生，最终导致肾小球簇塌陷，是终末期肾病的重要原因。在塌陷性肾小球病的众多病因中，自身免疫性疾病，如系统性红斑狼疮，已被牵涉其中。也有罕见的成人Still 病报告，这是一种自身炎症性疾病，其特征为发热、皮疹和炎性关节炎与塌陷性肾小球病有关。
在此，我们回顾了三例已发表的病例，并介绍了一例新的病例，一名 15 岁的非裔美国女性患者，患有塌陷性肾小球病，12 年后因发热、关节痛、咽痛、淋巴结病、肝细胞损伤和血清铁蛋白升高而被诊断为成人Still 病。她的塌陷性肾小球病在接受环孢素和泼尼松诱导治疗以及洛沙坦维持治疗后最初得到了很好的控制。然而，在患上成人Still 病后，尽管进行了多种免疫抑制治疗，她仍多次复发，肾功能逐渐恶化，最终进展为终末期肾病。
我们基于病例的回顾强调了成人Still 病和塌陷性肾小球病之间罕见但重要的关联，并推测了可能的病理生理学联系。
