초고도로 증가된 페리틴 수치를 보이는 원인불명열 환자 1예
A Case of Extremely Elevated Ferritin Levels in a Patient with Fever of Unknown Etiology
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초록
원인불명열의 원인질환 중 류마티스질환이 가장 흔하며, 그 중성인형스틸병은 매우 드문 원인 미상의 전신염증성질환이다. 발열,발진 및 관절염, 백혈구 증가 등의 매우 다양한 증상 및 징후를 보이지만 질병특이적인 양상은 없다. 종양, 감염, 염증성 질환 등을배제해야 진단이 가능하기 때문에 진단 및 치료 시기가 지연될 때가 많다. 성인형스틸병 환자에서 혈청 페리틴 수치의 증가가 흔하게 관찰되므로 혈청 페리틴 검사는 성인형스틸병을 진단하는데 중요한 검사이다. 또한 당화페리틴 비율의 감소가 성인형스틸병 환자에서 특징적으로 관찰되므로 두 검사를 동시에 실시하면 성인형스틸병의 감별진단에 도움을 받을 수 있다. 저자들은 높은 혈청페리틴 수치와 당화페리틴 비율 감소 소견을 보인 AOSD증례 1례를관찰하였기에 이를 보고하는 바이다.
Abstract
Fever of unknown etiology that occurs along with highly elevated serum ferritin concentrations ( >500 ng/mL) is mostly observed in hematologic malignancies and rheumatic diseases such as systemic lupus erythematosus, temporal arteritis, and adult-onset Still’s disease (AOSD), among which AOSD is a type of systemic inflammatory disorder with unknown pathophysiology and has very low incidence. AOSD presents with various nonspecific symptoms and signs such as high spiking fever, joint pain, skin rash, and increased leukocytes. Because AOSD is diagnosed after excluding the possibility of other conditions such as neoplasms, infections, and inflammations, diagnosis and treatment are generally delayed. Patients with AOSD often have high serum ferritin levels than those with other conditions, although the underlying mechanism for this is not clearly understood. In addition, decreased proportion of glycosylated ferritin are observed in most patients with AOSD. Therefore a combination of high serum ferritin and a decreased proportion of glycosylated ferritin seems to be important for the differential diagnosis of AOSD that thus may allow early diagnosis of AOSD. Here we report a case of AOSD diagnosed via extremely high serum ferritin levels and decreased glycosylated ferritin proportion.
참고문헌 (24)
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