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惠普尔病与关节炎。

Whipple disease and arthritis.

作者信息

Puéchal X

机构信息

Service de Rhumatologie, Centre Hospitalier du Mans, Le Mans, France.

出版信息

Curr Opin Rheumatol. 2001 Jan;13(1):74-9. doi: 10.1097/00002281-200101000-00012.

Abstract

Whipple disease is a chronic, multisystem, curable, bacterial infection that usually affects middle-aged men and has a wide range of clinical manifestations. The most common symptoms are weight loss and diarrhea, preceded in three quarters of cases by arthritis for a mean of 6 years. In most patients, periodic acid-Schiff staining of proximal small bowel biopsy specimens reveals inclusions within the macrophages, corresponding to bacterial structures. However, patients with various manifestations of the disease may have no gastrointestinal symptoms and negative jejunum biopsy results. Before the onset of gastrointestinal symptoms, a strong index of clinical suspicion is the key to diagnosis. The classic setting is long-term, unexplained, seronegative oligoarthritis or polyarthritis with a palindromic or relapsing course, although chronic destructive polyarthritis and spondyloarthropathy have been repeatedly reported. Identification of the Whipple bacterium, Tropheryma whippelii, has led to the development of polymerase chain reaction as a diagnostic tool in patients in the early stages of the disease or with atypical Whipple disease. This technique can be used to detect the bacterium in many tissues and fluids, including synovial tissue and fluid. The recent cultivation of the Whipple bacillus should lead to the development of serologic tests, further facilitating diagnosis. These recent major advances may show that the infection is more frequent than previously suspected and may expand the clinical spectrum of the disease. It may also allow earlier diagnosis, thereby preventing the development of the severe systemic and sometimes fatal forms of the disease.

摘要

惠普尔病是一种慢性、多系统、可治愈的细菌感染性疾病,通常影响中年男性,临床表现广泛。最常见的症状是体重减轻和腹泻,四分之三的病例在出现这些症状之前平均有6年的关节炎病史。在大多数患者中,近端小肠活检标本的过碘酸希夫染色显示巨噬细胞内有包涵体,与细菌结构相对应。然而,患有该疾病各种表现的患者可能没有胃肠道症状,空肠活检结果也可能为阴性。在胃肠道症状出现之前,高度的临床怀疑指数是诊断的关键。典型的情况是长期、原因不明的血清阴性寡关节炎或多关节炎,病程呈回纹型或复发型,尽管慢性破坏性多关节炎和脊柱关节病也屡有报道。惠普尔菌(Tropheryma whippelii)的鉴定促使聚合酶链反应作为一种诊断工具应用于疾病早期或非典型惠普尔病患者。该技术可用于在许多组织和液体中检测该细菌,包括滑膜组织和滑液。最近惠普尔杆菌的培养应该会促使血清学检测的发展,进一步便于诊断。这些最近的重大进展可能表明该感染比以前怀疑的更为常见,并且可能扩大该疾病的临床谱。这也可能使早期诊断成为可能,从而预防该疾病严重的全身性甚至有时致命形式的发展。

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