Diagnosis of Sjögren's syndrome.

Diagnosis of Sjögren's syndrome.
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干燥综合征的诊断。

DOI:
10.1002/art.1780280524
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发表时间:
1985
影响因子:
--
通讯作者:
J. Whitcher
J. Whitcher
中科院分区:
--
文献类型:
--
作者:
T. Daniels;J. Whitcher

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我们写这封信是为了回应埃弗斯梅尔博士的信(I),在出版之前我们没有机会看到这封信。他评论了一篇早期发表的论文 (2),该论文涉及唇唾液腺 (LSG) 活检在干燥综合征 (SS) 诊断中的作用。我们同意他的基本前提,即“为了使诊断测试在临床上适用,它不仅必须提高我们的客观性,而且还必须影响我们对患者的治疗方法的决策。” Eversmeyer 博士并不质疑 LSG 活检的客观性,但他声称该测试的结果不会影响治疗决策,并且没有针对 SS 的具体治疗方法。这些说法与我们多学科干燥综合征诊所 13 年的经验形成鲜明对比;此外,他的言论似乎只涉及继发性SS,并未考虑原发性SS。原发性 SS(不伴有结缔组织疾病的口腔和眼部疾病)至少与继发性 SS(结缔组织疾病以及其他 2 种疾病中的一种或两种)发生频率相同,并且原发性 SS 的口腔和眼部特征通常比继发性 SS 更严重 (3-5)。为了确定是否满足通常的诊断标准(客观存在构成疾病的三联征的 3 个成分中的 2 个 (2, 3)),必须进行 LSG 活检来诊断所有原发性 SS 病例,此外,还需要诊断那些在不存在干燥性角结膜炎的情况下存在明确结缔组织疾病的继发性 SS 病例。 LSG 活检结果不仅可以为原发性或继发性 SS 唾液成分提供最具疾病特异性的诊断标准的明确证据,还可以影响临床决策。例如,没有明确诊断为结缔组织病但主诉口干和/或眼干的患者仅根据这些症状和简短的临床检查就可能被诊断为原发性 SS。这样的决定(不幸的是,经常发生的事件)可能会忽视这些症状的最常见原因(单一或联合药物治疗的副作用、睑板腺炎或过敏性结膜炎),从而拒绝对患者进行可逆病症的治疗,并给他或她带来“良性”但无法治愈的慢性疾病的诊断负担。另一种决定可能是忽视患者的症状,因为没有观察到明确的体征,而且这些症状似乎是神经症引起的。在 sulzh 的案例中,原发性 SS 患者如果没有适当的治疗,将继续出现这些慢性、令人不安的症状,并且会因为不知道其原因而感到沮丧。该问题将持续存在并可能进一步发展,直到患者的牙齿和/或结膜发生不可逆转的损伤(常见事件)。事实上,原发性 SS 的诊断会影响临床决策。 Eversmeyer 正确地指出,在我们的 362 个 LSG 活检系列中,仅诊断出 SS 以外的 5 种疾病,但没有注意到 169 个 LSG 活检表明不同程度的非特异性慢性唾液腺炎和萎缩,从而排除了 SS 的唾液成分。其中一些活检来自之前根据上述症状被诊断为原发性 SS 的患者。因此,唇唾液腺活检是一种经济有效的诊断技术,因为它可以提供临床上有用的信息来确定或排除 SS 的唾液成分。虽然 SS 无法治愈,但它肯定是可以治疗的,既可以显着减轻患者的症状,又可以防止对牙齿造成不可逆转的损害……
We are writing in response to Dr. Eversmeyer’s letter (I), which we did not have an opportunity to see before pubkation. He comments on an earlier published paper (2) dealing with labial salivary gland (LSG) biopsy in the diagnosis of Sjogren’s syndrome (SS). We agree with his basic premise that “for a diagnostic test to be clinically applicable, it must not only increase our objectivity, but also influence our decision-making in our approach to the patient.” Dr. Eversmeyer does not question the objectivity of the LSG biopsy, but he asserts that results from this test do not influence therapeutic decision-making and that there is no specific treatment available for SS. These statements are in sharp contrast with 13 years’ experience in our multidisciplinary Sjogren’s syndrome clinic; moreover, his remarks appear to relate only to secondary SS and do not consider primary SS. Primary SS (oral and ocular components without connixtive tissue disease) occurs at least as frequently as secondary SS (a connective tissue disease along with either or both of the other 2 components), and the oral and ocular features of primary SS are generally more severe than those of secondary SS (3-5). To determine whether the usual diagnostic criteria-the objective presence of 2 of 3 components of the triad making up the disease (2, 3)-are fulfilled, an LSG biopsy is necessary for diagnosing all cases of primary SS and, in addition, those cases of secondary SS in which a clearly-defined connective tissue disease is present in the absence of keratoconjunctivitis sicca. As well as providing clear evidence of the most disease-specific diagnostic criterion for the salivary component of primary or secondary SS, results from an LSG biopsy influence clinical decision-making. For example, patients who do not have a clear diagnosis of connective tissue disease but complain of dry mouth and/or dry eyes may be diagnosed as having primary SS based only on these symptoms and a brief clinical examination. Such a decision (an unfortunately frequent event) could overlook the most common causes of these symptoms (side effects from single or combined drug therapy, meibomitis, or allergic conjunctivitis), thus denying the patient treatment for a reversible condition and burdening him or her with the diagnosis of a “benign” but incurable chronic disease. An alternative decision might be to disregard the patient’s symptoms because definitive signs are not observed and the symptoms may seem to be of neurotic origin. In sulzh a case, a patient with primary SS would continue to have these chronic, troubling symptoms without appropriate treatment, and would suffer the frustration of not knowing their cause. The problem would continue and likely progress until irreversible damage occurs to the patient’s teeth and/or conjunctivae (a common event). Indeed, establishing the diagnosis of primary SS affects clinical decision-making.Dr. Eversmeyer correctly points out that only 5 diseases other than SS were diagnosed in our series of 362 LSG biopsies, but fails to note the 169 LSG biopsies which indicated various degrees of nonspecific chronic sialadenitis and atrophy, thus ruling out the salivary component of SS. Some of these biopsies came from patients who had previously been given the diagnosis of primary SS based on symptoms as described above. Labial salivary gland biopsy is, therefore, a cost-effective diagnostic technique because it can provide clinically useful information to either establish or rule out the salivary component of SS. While SS is not curable, it is certainly treatable to the extent of both significantly reducing patients’ symptoms and preventing irreversible damage to their teeth and …