诊断困境:在急性肝损伤患者中区分肝结节病与DRESS综合征

Milan Gaihre , Saket Jha , Rahul Pathak , Sujan Ghimire , Prince Baranawal , Aashish Poudel , Ritika Bhatta
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引用次数: 0

摘要

如果不明原因的急性肝损伤症状包含多种重叠的病因,则可能导致诊断困难。不常见的疾病如肝结节病和DRESS综合征需要纳入鉴别诊断,但药物性肝损伤和病毒性肝炎是常见的病因。病例介绍:一名28岁女性患者,表现为瘙痒性皮疹、黄疸和右上腹疼痛一个月。实验室检查显示嗜酸性粒细胞增多(520个细胞/μL),高胆红素血症(6.20 mg/dL),肝细胞损伤(AST 401 U/L, ALT 351 U/L), ACE水平高升高(216 U/L)。影像学显示纵隔淋巴结病变。患者的肝酶和症状在皮质类固醇治疗后明显改善。由于DRESS综合征和肝结节病具有相同的临床和生化特征,因此在本病例中对两种疾病的鉴别诊断带来了挑战。虽然颞叶药物关联和嗜酸性粒细胞增多倾向于药物过敏,但ACE水平升高和淋巴结病变推动了肉芽肿疾病的发展。类固醇反应强调了这两种疾病的共同免疫介导的病理生理学。结论在评估有全身性表现的急性肝损伤时,本病例强调应同时排除药物过敏和肉芽肿性疾病。它提请注意需要改进的诊断标记,同时也认识到在这些情况下血清ACE测试和成像的效用。该病例突出了在肝病困难患者中跨学科管理的优势和皮质类固醇的有效性。本病例表明,在肝结节病和药物性肝损害相似的情况下,很难区分这两种疾病。两种病因必须考虑的情况下,不明原因的急性肝损伤的全身性症状。虽然皮质类固醇可以有效治疗免疫介导性疾病,但需要延长随访时间。跨学科的合作提高了这种复杂病例的治疗和诊断的有效性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A diagnostic dilemma: Differentiating hepatic sarcoidosis from DRESS syndrome in a patient with acute liver injury

Introduction

If unexplained acute liver injury symptoms imply several overlapping etiologies, diagnostic difficulty can ensue. Less frequent diseases like hepatic sarcoidosis and DRESS syndrome need to be included in differential diagnosis, but drug-induced liver injury and viral hepatitis are frequent etiologies.

Presentation of the case

A 28-year-old woman patient presented with pruritic rash, jaundice, and right upper quadrant pain for one month. Laboratory tests showed eosinophilia (520 cells/μL), hyperbilirubinemia (6.20 mg/dL), hepatocellular injury (AST 401 U/L, ALT 351 U/L), and highly raised ACE levels (216 U/L). Imaging revealed mediastinal lymphadenopathy. The patient's liver enzymes and symptoms significantly improved with corticosteroid treatment.

Discussion

Because DRESS syndrome and hepatic sarcoidosis share clinical and biochemical characteristics, the diagnostic challenge of differentiating between the two conditions is brought about in this case. While temporal drug association and eosinophilia predisposed towards drug hypersensitivity, the raised ACE levels and lymphadenopathy pushed in the direction of granulomatous disease. The shared immune-mediated pathophysiology of the two diseases was emphasized by the steroid response.

Conclusion

In the assessment of acute liver injury with systemic manifestations, this case emphasizes the need to rule out both drug hypersensitivity and granulomatous disease. It draws attention towards the need for improved diagnostic markers while also recognizing the utility of serum ACE testing and imaging in these situations. The case highlights the advantage of interdisciplinary management in difficult hepatology patients and the effectiveness of corticosteroids.

Key clinical message

This case demonstrates the difficulty of making a distinction between hepatic sarcoidosis and drug-induced liver damage in cases where the two diseases mimic each other. Both etiologies must be considered in cases of unexplained acute liver injury with systemic symptoms. Although corticosteroids can be an effective treatment of immune-mediated disease, prolonged follow-up is indicated. Interdisciplinary collaboration enhances the treatment of such complex cases and the validity of diagnoses.
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