快樂小藥師
本部落格為介紹醫藥相關知識部落格,並沒有販賣藥品。此外文章部分內容可能會引起您的不安或是心理不適,在按下繼續閱讀前,請先自我評估,謝謝(原創文章如需轉載請先告知 左右水平廣告輪播-語法
  • // 如果你希望圖片繼續多一點,那就自己再複製增加

 以下內容選自維基百科

 

Still's disease is a form of juvenile idiopathic arthritis, characterized by high spiking fevers and transient rashes, named after the English physician Sir George Frederic Still (1861-1941). The disease was first discovered in children, but now it is also known to occur, less commonly, in adults in whom it is referred to as adult-onset Still's disease.

There are several theories about the cause of Still's disease. It has been suggested it may be caused by a microbacterial infection or that it is an autoimmune disorder. However, the cause of Still's disease remains unknown

 Symptoms

Patients with Still's disease usually have systemic symptoms. Usual symptoms include:

  • waves of high fevers that rise to 40 °C (104 °F) which may be accompanied by extreme fatigue
  • A faint transient non-itching salmon-colored skin rash can also be observed.
  • Flu like pain throughout the body,
  • muscle pain

Other signs and symptoms include:

Diagnosis

Because the disease presents in many different ways, diagnosis is difficult. It is often a laborious process consisting of the elimination of other diseases. It is diagnosed on a basis of clinical features of the illness, with the results of a number of common tests combined. Persistent arthritis (lasting at least 6 weeks) is frequently a symptom. A rash is frequently visible on the body, and the patients usually suffer from high, spiking fevers. Blood tests frequently indicate elevated white blood cell counts, suggesting there is serious inflammation. Also, low red blood cell counts (anemia) are common. Typically, elevated blood tests indicate in high sedimentation rates, an indicator of inflammation. Other indicators of inflammation may also be elevated, including CRP (c-reactive protein) and ferritin levels. However, the classic blood tests for rheumatoid arthritis and systemic lupus erythematosus are usually negative.

 Prognosis

The fever and most of the other symptoms tend to run their course within several months. However, the arthritis can become a long-term problem as a chronic illness persisting into adulthood. In addition, since the disease can present as an acute illness in adulthood, many adult patients with chronic illness from Still's Disease never show signs or symptoms of the disease in childhood, but both initially present with the disease in adulthood and may have persistence of all symptoms through some period of adulthood. The fever and systemic symptoms in these patients may go in remission, leaving arthritis symptoms. Conversely, all signs and symptoms, including arthritis and systemic signs and symptoms, may either remit completely during adulthood, present intermittently in a "flare" type pattern or, less commonly, continue indefinitely during adulthood.

以下內容選自Harrison's Principles of Internal Medicine - 16th Ed. (2005)

A number of patients in these series had temporal arteritis, adult Still's disease, drug-related fever, and factitious fever. In recent series, ~25 to 30% of cases of FUO have remained undiagnosed. The general term noninfectious inflammatory diseases applies to systemic rheumatologic or vasculitic diseases such as polymyalgia rheumatica, lupus, and adult Still's disease as well as to granulomatous diseases such as sarcoidosis and Crohn's and granulomatous hepatitis.

 

TABLE 18-3  Ten Leading Causes of Classic FUO among Adults at Community Hospitals in the United States
Cause
% of Total
Lymphoma
 16
Collagen vascular disease
 16
Abscess
 13
Undiagnosed cause
  9
Solid tumor
  8
Thrombosis or hematoma
  7
Granulomatous disease, nonmycobacterial
  5
Endocarditis
  5
Mycobacterial disease
  5
Viral disease
  5
Remaining causes
 11
 
100
Source: Adapted from Kazanjian, 1992.

11_case_fig3.jpg

g_1593577077.jpg

補一點中文的解說:

成人型史笛兒氏症(adult-onset Still's disease,簡稱為AOSD)是一種可侵犯多處器官的發炎性疾病,也是成人不明原因發燒的重要病因,其臨床特徵是持續性高燒、易於消褪的皮膚紅疹、關節痛或關節炎、喉嚨痛、淋巴結腫大、肝脾腫大、或其他內臟器官侵犯。

 實驗室檢查異常主要包括周邊血液白血球增加、肝功能異常、急性反應物質增高、及高鐵血素血症等。由於此病無獨特的臨床表徵,而且實驗室檢查結果除了高鐵色素血症外,其餘較不具專一性,使得此病之確定診斷較為困難且常延誤。患者發病時,因持續發燒常被初步診斷為感染而使用不必要的抗生素,也常被視為不明熱而做多項侵入性檢查,但仍無法確立診斷。

喉嚨痛是AOSD一項臨床特徵,咽喉檢查多為正常,我們利用影像檢查首次發現其病變在於頸部環型甲狀軟骨(crico-thyroid cartilage)處發炎。目前AOSD的免疫致病機轉仍不清楚,過去學者與我們的研究結果顯示活動性AOSD患者,其血清介白質-6、介白質-8、介白質-18及腫瘤壞死因子-α濃度增加,而且在病變組織之基因表現量也增高,顯示細胞激素在此病扮演重要的致病角色。利用細胞流速儀分析AOSD患者周邊血液輔助性T細胞之類型,結果顯示活動性AOSD患者的周邊血液是以第1型(Th1)為主,而且介白質-18Th1類型的形成相關。在發炎過程中,要終止活化T細胞的過度增生,須有適當且無傷害性的調節機轉,細胞凋亡就扮演重要的角色。利用Annexin-V/PI雙染色及流速細胞儀分析AOSD患者淋巴細胞的細胞凋亡變化,結果顯示AOSD患者的周邊血液淋巴細胞發生自發性及刺激性細胞凋亡的比率明顯增加,這暗示AOSD患者可能藉由淋巴細胞之細胞凋亡來減少過量的活化,以降低免疫發炎反應。

 

 

    全站熱搜

    快樂小藥師 發表在 痞客邦 留言(0) 人氣()


    留言列表

    發表留言

    您尚未登入,將以訪客身份留言。亦可以上方服務帳號登入留言

    請輸入暱稱 ( 最多顯示 6 個中文字元 )

    請輸入標題 ( 最多顯示 9 個中文字元 )

    請輸入內容 ( 最多 140 個中文字元 )

    請輸入左方認證碼:

    看不懂,換張圖

    請輸入驗證碼