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肝结节病的治疗。

Management of Hepatic Sarcoidosis.

机构信息

Department of Internal Medicine II, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.

Department of Medicine B, University Medical Center, Münster, Germany.

出版信息

J Gastrointestin Liver Dis. 2022 Sep 15;31(3):323-330. doi: 10.15403/jgld-4122.

Abstract

BACKGROUND AND AIMS

Liver involvement in sarcoidosis may occur in up to 60% of all patients. As many patients experience only minor symptoms, a high number of undiagnosed cases must be assumed. In order to successfully identify patients with hepatic sarcoidosis, a throughout characterization of these patients and their course of disease is necessary.

METHODS

We collected 40 patients from four German centers to evaluate current treatment standards and course of disease. All of our patients underwent liver biopsy with histologically proven granulomatous hepatitis.

RESULTS

Detailed characterization of our patients showed an overall benign course of disease. Treatment was very diverse with glucocorticoids for 1 year in 55% (22/40), 5-10 years in 18% (7/40), and permanently in 18% (7/40). Other treatments included disease-modifying anti-rheumatic drugs (DMARDs), the conventional non-biological type in 53% of all patients (of these 81% received azathioprine, 46% metotrexate, 10% hydroxychloroquine, 10% mycophenolate mofetil and 10% cyclophosphamide and biologicals in 8%. Despite these very diverse treatments, patients generally showed slow progression of the disease. Two patients died. None of our patients received a liver transplantation.

CONCLUSIONS

Patients received diverse treatments and generally showed slow progression of the disease. Based on our experience, we proposed a diagnostic work up and surveillance strategy as a basis for future, prospective register studies.

摘要

背景与目的

结节病累及肝脏的发生率可达所有患者的 60%。由于许多患者仅有轻微的症状,故推测存在大量未确诊的病例。为了成功识别肝结节病患者,需要对这些患者及其疾病过程进行全面的描述。

方法

我们从德国的 4 个中心收集了 40 名患者,以评估目前的治疗标准和疾病进程。所有患者均接受了肝活检,病理证实为肉芽肿性肝炎。

结果

对我们患者的详细特征描述显示,疾病总体呈良性进程。治疗方案非常多样,55%(22/40)的患者接受糖皮质激素治疗 1 年,18%(7/40)的患者接受糖皮质激素治疗 5-10 年,18%(7/40)的患者接受糖皮质激素长期治疗。其他治疗包括改善病情的抗风湿药物(DMARDs),所有患者中有 53%(21/40)接受了传统的非生物性 DMARDs,其中 81%(17/21)接受了硫唑嘌呤、46%(10/21)接受了甲氨蝶呤、10%(2/21)接受了羟氯喹、10%(2/21)接受了霉酚酸酯、10%(2/21)接受了环磷酰胺,还有 8%(2/21)接受了生物制剂。尽管这些治疗方案非常多样,但患者的疾病总体呈缓慢进展。有 2 名患者死亡。我们的患者均未接受肝移植。

结论

患者接受了多样的治疗,疾病总体呈缓慢进展。基于我们的经验,我们提出了一种诊断方法和监测策略,作为未来前瞻性登记研究的基础。

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