Li Jian, Huang Xiao-Ming, Fang Wei-Gang, Zeng Xue-Jun
Department of Internal Medicine, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, PR China.
J Clin Rheumatol. 2006 Jun;12(3):114-7. doi: 10.1097/01.rhu.0000221794.24431.36.
Although the association of Pneumocystis carinii pneumonia (PCP) with connective tissue disease (CTD) has been noted for a long time, there are few series reported.
The objective of this study was to describe clinical features and prognosis of PCP infections in patients with CTD in China.
We retrospectively reviewed the characteristics, clinical features, and prognosis of PCP in patients with CTD in a single hospital.
A total of 7 cases were reviewed (systemic lupus erythematosus n = 2, microscopic polyangiitis n = 2, dermatomyositis n = 2, polymyositis n = 1). Eighty-six percent of patients developed PCP within 3 months of the diagnosis of CTD. All patients were receiving daily glucocorticoid therapy and cytotoxic drugs before the diagnosis of PCP. Most patients had fever, progressive dyspnea, and dry cough at onset of PCP. The mean duration of symptoms before PCP diagnosis was 7 days. Absolute lymphocyte counts ranged from 126 to 528/microL. The CD4 lymphocyte counts of all patients were 87 +/- 78/microL. One patient was diagnosed by induced sputum; 6 patients were diagnosed by bronchoalveolar lavage fluid. Complicating fungal infection was found in 4 of 7 patients at the time of diagnosis of PCP. All patients were treated by trimethoprim-sulfamethoxazole and corticosteroids. Six (86%) patients died. The mean duration of the time from diagnosis to death was 14 +/- 4 days.
Our results suggest that PCP is an uncommon and fatal opportunistic infection in patients with CTD. When patients with CTD who are receiving immunosuppressive therapy have low lymphocyte counts and/or CD4 lymphocyte counts less than 250/microL develop fever, dry cough, dyspnea, and chest radiography shows diffuse interstitial infiltrate, the diagnosis of PCP should be highly suspected. Induced sputum or BAL must be quickly performed to confirm diagnosis. Further study is needed as to whether earlier treatment will improve prognoses or whether patients with CTD with low CD4 counts should receive PCP prophylaxis.
尽管卡氏肺孢子虫肺炎（PCP）与结缔组织病（CTD）的关联早已被注意到，但报道的病例系列较少。
本研究的目的是描述中国CTD患者中PCP感染的临床特征和预后。
我们回顾性分析了一家医院中CTD患者PCP的特征、临床特点和预后。
共回顾了7例患者（系统性红斑狼疮2例，显微镜下多血管炎2例，皮肌炎2例，多发性肌炎1例）。86%的患者在CTD诊断后3个月内发生PCP。所有患者在PCP诊断前均接受每日糖皮质激素治疗和细胞毒性药物治疗。大多数患者在PCP发病时出现发热、进行性呼吸困难和干咳。PCP诊断前症状的平均持续时间为7天。绝对淋巴细胞计数范围为126至528/μL。所有患者的CD4淋巴细胞计数为87±78/μL。1例患者通过诱导痰诊断；6例患者通过支气管肺泡灌洗液诊断。7例患者中有4例在PCP诊断时发现合并真菌感染。所有患者均接受甲氧苄啶-磺胺甲恶唑和糖皮质激素治疗。6例（86%）患者死亡。从诊断到死亡的平均时间为14±4天。
我们的结果表明，PCP在CTD患者中是一种罕见且致命的机会性感染。当接受免疫抑制治疗的CTD患者淋巴细胞计数低和/或CD4淋巴细胞计数低于250/μL并出现发热、干咳、呼吸困难且胸部X线显示弥漫性间质浸润时，应高度怀疑PCP诊断。必须尽快进行诱导痰或支气管肺泡灌洗以确诊。关于早期治疗是否会改善预后或CD4计数低的CTD患者是否应接受PCP预防，还需要进一步研究。
