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[系统性红斑狼疮患儿卡氏肺孢子虫肺炎的临床特征]

[Clinical characteristics of Pneumocystis carinii pneumonia in children with systemic lupus erythematosus].

作者信息

Tang Xiao-yan, Li Ji, Dong Fen, Song Hong-mei

机构信息

Department of Pediatrics, Peking Union Medical College Hospital, Beijing 100730, China.

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出版信息

Zhonghua Er Ke Za Zhi. 2013 Dec;51(12):920-4.

Abstract

OBJECTIVE

To identify the risk factors which will indicate the Pneumocystis carinii (Pc) infection in children with systemic lupus erythematosus (SLE) and investigate the clinical features and to elevate the level to find out the high-risk patients and make early diagnosis and treatment.

METHOD

The characteristics, clinical features, laboratory examinations, treatment and prognosis of Pneumocystis carinii pneumonia (PCP) in children with SLE under 18 years of age treated in our hospital between January 2000 and January 2013 were prospectively reviewed. A comparison was made with the 26 cases of SLE children without PCP who were matched for gender, age and course, and a literature review was made.

RESULTS

(1) Five cases were enrolled, 3 were male and 2 female. Their age range was 13-17 (14.0 ± 1.6) years. All the children had kidney involvement. The courses were from 3 months to 4.5 years. All patients were receiving daily glucocorticoid therapy and immunosuppressive drugs before the diagnosis of PCP.Four patients were in the inactive phase of SLE (SLEDAI 2-4 points), and the fifth case was in active phase (SLEDAI 8, low complement 2 points, anti-dsDNA antibody positive 2 points, urine-protein 4 points). (2) Besides the clinical manifestations of SLE, most patients had progressive dyspnea, fever and dry cough at onset of PCP. Two children accepted mechanical ventilation because of respiratory failure. The mean duration of the symptoms to diagnosis was 10-30 (17.6 ± 7.8) days. Lactose dehydrogenase (LDH) was elevated more or less, median was (700 ± 263) U/L. Lymphocyte count were (0.3-1.4)×10(9)/L (median 0.5×10(9)/L), and three children had CD4 T lymphocyte count <0.3×10(9)/L. Arterial blood gas analyses showed severe hypoxemia. Chest radiographs showed in all cases diffuse interstitial infiltration. Pc was positive in the sputum. All patients were treated with trimethoprim-sulfamethoxazole and corticosteroids.

CONCLUSION

When SLE children are treated with corticosteroids and immunosuppressive drugs, low lymphocyte count is the risk factor for Pc infection.It is essential to monitor lymphocyte count.We should pay more attention to fever, dry cough and hypoxemia. Chest radiologic examination may help diagnose the PCP in SLE children.It may be helpful for SLE children whose CD4T lymphocyte was below 0.3×10(9)/L to take trimethoprim-sulfamethoxazole for PCP prophylaxis.

摘要

目的

确定系统性红斑狼疮(SLE)患儿发生卡氏肺孢子虫(Pc)感染的危险因素,探讨其临床特征,提高对高危患者的识别水平,以便早期诊断和治疗。

方法

对2000年1月至2013年1月在我院治疗的18岁以下SLE合并卡氏肺孢子虫肺炎(PCP)患儿的特点、临床特征、实验室检查、治疗及预后进行前瞻性回顾。与26例性别、年龄及病程相匹配的未患PCP的SLE患儿进行比较,并进行文献复习。

结果

(1)纳入5例,男3例,女2例。年龄范围为13 - 17(14.0±1.6)岁。所有患儿均有肾脏受累。病程为3个月至4.5年。所有患者在诊断PCP前均接受每日糖皮质激素治疗及免疫抑制剂治疗。4例处于SLE非活动期(SLEDAI 2 - 4分),第5例处于活动期(SLEDAI 8分,低补体2分,抗双链DNA抗体阳性2分,尿蛋白4分)。(2)除SLE临床表现外,多数患者PCP起病时有进行性呼吸困难、发热及干咳。2例患儿因呼吸衰竭接受机械通气。症状至诊断的平均时间为10 - 30(17.6±7.8)天。乳酸脱氢酶(LDH)或多或少升高,中位数为(700±263)U/L。淋巴细胞计数为(0.3 - 1.4)×10⁹/L(中位数0.5×10⁹/L),3例患儿CD4⁺T淋巴细胞计数<0.3×10⁹/L。动脉血气分析显示严重低氧血症。胸部X线片均显示弥漫性间质浸润。痰中Pc阳性。所有患者均接受甲氧苄啶 - 磺胺甲恶唑及糖皮质激素治疗。

结论

SLE患儿在接受糖皮质激素及免疫抑制剂治疗时,淋巴细胞计数低是Pc感染的危险因素。监测淋巴细胞计数至关重要。应更加关注发热、干咳及低氧血症。胸部影像学检查有助于诊断SLE患儿的PCP。对于CD4⁺T淋巴细胞低于0.3×10⁹/L的SLE患儿,服用甲氧苄啶 - 磺胺甲恶唑预防PCP可能有益。

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