(顺序自身免疫?罕见的MDA5型皮肌炎及结节病[j]。

IF 0.9 4区 医学 Q4 RHEUMATOLOGY
Phillip Kremer, Nikolas Ruffer, Tim Oqueka, Stefan Steurer, Daniel Köhler, Ina Kötter, Martin Krusche
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引用次数: 0

摘要

背景:抗mda5阳性皮肌炎是一种罕见的特发性炎性肌病亚型,常伴有快速进展的间质性肺疾病和严重的皮肤溃疡。它与高死亡率有关。同时发生的结节病与多系统累及肌炎只被描述非常罕见的文献迄今为止。病例报告:我们报告了一名患有3年抗mda - 5阳性皮肌炎病史的39岁男性,他出现呼吸困难和高钙血症的恶化,并伴有急性肾损伤,在连续减少免疫抑制治疗后,他的一般情况明显恶化。支气管镜检查和淋巴结活检最终证实结节病的诊断。结论:本病例报告说明了在罕见疾病病程中出现新症状时不断重新评估诊断的重要性。在出现明显的淋巴结病变,新的高钙血症和不典型皮肌炎的肺实质改变时,也应考虑结节病的鉴别诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Sequential autoimmunity? The rare case of MDA5 dermatomyositis and sarcoidosis].

Background: Anti-MDA5 positive dermatomyositis is a rare subtype of idiopathic inflammatory myopathies often accompanied by rapidly progressive interstitial lung disease and severe cutaneous ulcerations. It is associated with a high mortality. The simultaneous occurrence of sarcoidosis with multisystemic involvement of myositis has only been described very rarely in the literature to date.

Case report: We report the case of a 39-year-old man with a 3-year history of anti-MDA‑5 positive dermatomyositis who developed a worsening of dyspnea and hypercalcemia with acute kidney injury and a marked deterioration in his general condition after successive reductions in immunosuppressive therapy. A bronchoscopy and lymph node biopsy ultimately confirmed the diagnosis of sarcoidosis.

Conclusion: This case report illustrates the importance of continuously re-evaluating the diagnosis when new symptoms occur during the course of a rare disease. In the presence of pronounced lymphadenopathy, new hypercalcemia and lung parenchymal changes atypical for dermatomyositis, the differential diagnosis of sarcoidosis should also be considered.

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来源期刊
Zeitschrift fur Rheumatologie
Zeitschrift fur Rheumatologie 医学-风湿病学
CiteScore
2.20
自引率
20.00%
发文量
150
审稿时长
6-12 weeks
期刊介绍: Die Zeitschrift für Rheumatologie ist ein international angesehenes Publikationsorgan und dient der Fortbildung von niedergelassenen und in der Klinik tätigen Rheumatologen. Die Zeitschrift widmet sich allen Aspekten der klinischen Rheumatologie, der Therapie rheumatischer Erkrankungen sowie der rheumatologischen Grundlagenforschung. Umfassende Übersichtsarbeiten zu einem aktuellen Schwerpunktthema sind das Kernstück jeder Ausgabe. Im Mittelpunkt steht dabei gesichertes Wissen zu Diagnostik und Therapie mit hoher Relevanz für die tägliche Arbeit – der Leser erhält konkrete Handlungsempfehlungen. Frei eingereichte Originalien ermöglichen die Präsentation wichtiger klinischer Studien und dienen dem wissenschaftlichen Austausch.
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