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一名75岁女性的肺纤维化和显微镜下多血管炎

Pulmonary fibrosis and microscopic polyangiitis in a 75-year-old woman.

作者信息

Koutsoviti Sofia, Elezoglou Antonia, Katsimpari Chaido, Sofianos Ioannis, Raftakis Ioannis, Theotikos Evangelos, Samaras Charilaos, Myriokefalitakis Ioannis

机构信息

Rheumatology Department of Asklepieion Voulas General Hospital, Athens, Greece.

1 Internal Medicine Department of Asklepieion Voulas General Hospital, Athens, Greece.

出版信息

Mediterr J Rheumatol. 2019 Mar 28;30(1):44-47. doi: 10.31138/mjr.30.1.44. eCollection 2019 Mar.

Abstract

We present a case of a 75-year-old woman who admitted in the internal medicine department for a recent onset of persisting moderate daily fever and fatigue that started 30 days prior to her hospitalization. Her past medical history is remarkable for mild pulmonary fibrosis, megaloblastic anaemia, and hypergammaglobulinaemia of no obvious causes. On presentation, she was febrile (38°C) and had high ESR and CRP levels, but most of her laboratory tests were within normal levels and had no signs of arthritis or rash. She was hospitalized for suspected lower urinary tract infection and started on antibiotics. During hospitalization, her renal function deteriorated together with microscopic haematuria, proteinuria and granular urine casts in urine analysis and her inflammation markers raised further. A renal biopsy revealed glomerulonephritis with pauci-immune crescents, and serology tests were positive for anti-MPO p-ANCA, both suggesting a diagnosis of microscopic polyangiitis (MPA). While high-dose methylprednisolone pulses and cyclophosphamide were introduced intravenously, there was no remission, but respiratory failure occurred that led to patient's intubation and transfer to the ICU. She died a few days later due to septic shock. Asymptomatic pulmonary fibrosis can precede microscopic polyangiitis for several years and is associated with a poor prognosis.

摘要

我们报告一例75岁女性患者,因近期持续中度发热和乏力入院,症状始于住院前30天。她既往有轻度肺纤维化、巨幼细胞贫血和原因不明的高丙种球蛋白血症病史。入院时,她发热(38°C),血沉和C反应蛋白水平升高,但大多数实验室检查结果在正常范围内,且无关节炎或皮疹迹象。她因疑似下尿路感染住院并开始使用抗生素。住院期间,她的肾功能恶化,同时尿液分析显示镜下血尿、蛋白尿和颗粒管型,炎症指标进一步升高。肾活检显示为寡免疫性新月体性肾小球肾炎,血清学检查抗髓过氧化物酶(MPO)-抗中性粒细胞胞浆抗体(p-ANCA)阳性,均提示诊断为显微镜下多血管炎(MPA)。虽然静脉注射了大剂量甲泼尼龙冲击和环磷酰胺,但病情未缓解,反而出现呼吸衰竭,导致患者插管并转入重症监护病房(ICU)。几天后,她因感染性休克死亡。无症状性肺纤维化可在显微镜下多血管炎之前数年出现,且预后不良。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cd1d/7045917/946228dad776/MJR-30-1-44-g001.jpg

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