上海交通大学学报(医学版) ›› 2024, Vol. 44 ›› Issue (2): 278-286.doi: 10.3969/j.issn.1674-8115.2024.02.015
• 病例报告 • 上一篇
收稿日期:
2023-07-23
接受日期:
2023-12-26
出版日期:
2024-02-28
发布日期:
2024-03-25
通讯作者:
魏强华
E-mail:chenqiongjone@163.com;weiqh1122@sina.com
作者简介:
陈 琼(1987—),女,主治医师,硕士;电子信箱:chenqiongjone@163.com。
基金资助:
CHEN Qiong1(), FANG Jie2, WEI Qianghua1()
Received:
2023-07-23
Accepted:
2023-12-26
Online:
2024-02-28
Published:
2024-03-25
Contact:
WEI Qianghua
E-mail:chenqiongjone@163.com;weiqh1122@sina.com
Supported by:
摘要:
该文对1例系统性红斑狼疮(systemic lupus erythematosus,SLE)合并纯红细胞再生障碍性贫血(pure red cell aplasia,PRCA)病例进行报道,并复习1974—2021年国内外文献报道的51例SLE合并PRCA患者的资料,对该52例患者(51+1例)的流行病学特征、临床特征、实验室指标、治疗以及预后进行统计分析。结果显示,52例患者多见于亚洲育龄期女性。诊断为SLE和诊断为PRCA时,患者的中位年龄分别为31.5岁和36.0岁。先诊断为SLE的患者,诊断为SLE与诊断为PRCA的间隔时间明显长于先诊断为PRCA的患者(P=0.042)。52例患者临床表现多为乏力、关节痛、雷诺现象和皮疹,并可合并自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)、胸腺瘤、甲状腺功能减退、重症肌无力(myasthenia gravis,MG)。实验室指标中抗核抗体(antinuclear antibody,ANA)阳性、抗双链DNA(double stranded DNA,dsDNA)抗体阳性、尿蛋白阳性和低补体水平的人数比例均较高。52例患者中,有51例的治疗方案包含糖皮质激素,占比98.08%,其次依次为输血治疗、环孢素A、环磷酰胺、高剂量静脉注射免疫球蛋白等。在报道了预后的50例病例中,预后好转44例,无效和死亡各3例。该文旨在提高临床医师对SLE合并PRCA的认识。
中图分类号:
陈琼, 方杰, 魏强华. 系统性红斑狼疮合并纯红细胞再生障碍性贫血1例报道及文献复习[J]. 上海交通大学学报(医学版), 2024, 44(2): 278-286.
CHEN Qiong, FANG Jie, WEI Qianghua. Systemic lupus erythematosus complicated with pure red cell aplasia: a case report and literature review[J]. Journal of Shanghai Jiao Tong University (Medical Science), 2024, 44(2): 278-286.
图1 病情变化和治疗过程图Note: CsA—cyclosporine A; Pred—prednisone; MPS—methylprednisolone; Hb—hemoglobin; Ret—reticulocyte.
Fig 1 Process of disease change and treatment
First author | Published year | Origin of the case | Case sample/n | Gender | Age/year | Reference |
---|---|---|---|---|---|---|
ZHANG L | 2005 | China | 1 | F | 6 | [ |
ZHANG G C | 1991 | China | 1 | M | 44 | [ |
XIE Q B | 2002 | China | 2 | M/F | 54/56 | [ |
CHEN N N | 2006 | China | 1 | F | 26 | [ |
LIU G Q | 2005 | China | 2 | F/F | 41/31 | [ |
HUANG R W | 1998 | China | 1 | F | 43 | [ |
LIU W F | 1999 | China | 1 | F | 21 | [ |
LIU Y | 2017 | China | 1 | M | 19 | [ |
NI X | 2011 | China | 1 | F | 37 | [ |
MITSUNAKA H | 1998 | Japan | 1 | F | 68 | [ |
IWADATE H | 2006 | Japan | 1 | F | 43 | [ |
HABIB G S | 2002 | Iseral | 1 | F | 31 | [ |
LINARDAKI G D | 1999 | Greece | 2 | F/F | 21/28 | [ |
KOYAMA R V | 2014 | Brazil | 1 | F | 52 | [ |
CHOI B G | 2002 | Korea | 1 | F | 31 | [ |
DUCHMANN R | 1995 | Germany | 1 | F | 63 | [ |
FRANCIS D A | 1982 | UK | 1 | F | 64 | [ |
OKADA H | 1994 | Japan | 1 | M | 43 | [ |
MAVRAGANI C P | 2003 | Greece | 1 | F | 38 | [ |
KIELY P D | 1995 | India | 2 | F/F | 22/33 | [ |
ATZENI F | 2003 | Italy | 1 | F | 50 | [ |
DUARTE-SALAZAR C | 2000 | Mexico | 1 | F | 21 | [ |
CHAN A Y K | 2005 | Hongkong, China | 1 | F | 59 | [ |
IDEGUCHI H | 2007 | Japan | 1 | F | 82 | [ |
SUZUKI T | 2003 | Japan | 1 | F | 54 | [ |
GUPTA R K | 2011 | Vietnam | 1 | F | 23 | [ |
FRANZÉN P | 1987 | Finland | 1 | F | 24 | [ |
HECK L W | 1985 | USA | 1 | F | 17 | [ |
HARA A | 2008 | Japan | 1 | F | 42 | [ |
MARMONT A M | 2014 | Italy | 1 | F | 45 | [ |
MEYER R J | 1978 | China | 1 | F | 56 | [ |
FALLAHI S | 2014 | Iran | 1 | F | 54 | [ |
ARCASOY M O | 2005 | USA | 1 | F | 34 | [ |
MOLLAEIAN A | 2020 | African | 1 | M | 65 | [ |
BISWAL N | 2007 | India | 1 | F | 9 | [ |
NAKAMURA H | 2010 | Japan | 1 | F | 47 | [ |
ILAN Y | 1993 | Iseral | 1 | F | 22 | [ |
TAKIGAWA M | 1974 | Japan | 1 | F | 44 | [ |
HARADA N | 1990 | Japan | 1 | F | 27 | [ |
ATSUMI T | 1991 | Japan | 1 | M | 21 | [ |
JAREK M J | 1996 | USA | 1 | F | 69 | [ |
MINAMI R | 2006 | Japan | 1 | F | 33 | [ |
MURAYAMA J | 2006 | Japan | 1 | F | 48 | [ |
SAEED M | 2021 | Pakistan | 1 | F | 27 | [ |
TOMAC-STOJMENOVIĆ M | 2017 | Croatia | 1 | F | 35 | [ |
DU A X | 2006 | Taiwan, China | 1 | F | 24 | [ |
JIMENO S A | 2001 | Spain | 1 | F | 19 | [ |
表1 51例病例资料
Tab 1 Data of the 51 cases
First author | Published year | Origin of the case | Case sample/n | Gender | Age/year | Reference |
---|---|---|---|---|---|---|
ZHANG L | 2005 | China | 1 | F | 6 | [ |
ZHANG G C | 1991 | China | 1 | M | 44 | [ |
XIE Q B | 2002 | China | 2 | M/F | 54/56 | [ |
CHEN N N | 2006 | China | 1 | F | 26 | [ |
LIU G Q | 2005 | China | 2 | F/F | 41/31 | [ |
HUANG R W | 1998 | China | 1 | F | 43 | [ |
LIU W F | 1999 | China | 1 | F | 21 | [ |
LIU Y | 2017 | China | 1 | M | 19 | [ |
NI X | 2011 | China | 1 | F | 37 | [ |
MITSUNAKA H | 1998 | Japan | 1 | F | 68 | [ |
IWADATE H | 2006 | Japan | 1 | F | 43 | [ |
HABIB G S | 2002 | Iseral | 1 | F | 31 | [ |
LINARDAKI G D | 1999 | Greece | 2 | F/F | 21/28 | [ |
KOYAMA R V | 2014 | Brazil | 1 | F | 52 | [ |
CHOI B G | 2002 | Korea | 1 | F | 31 | [ |
DUCHMANN R | 1995 | Germany | 1 | F | 63 | [ |
FRANCIS D A | 1982 | UK | 1 | F | 64 | [ |
OKADA H | 1994 | Japan | 1 | M | 43 | [ |
MAVRAGANI C P | 2003 | Greece | 1 | F | 38 | [ |
KIELY P D | 1995 | India | 2 | F/F | 22/33 | [ |
ATZENI F | 2003 | Italy | 1 | F | 50 | [ |
DUARTE-SALAZAR C | 2000 | Mexico | 1 | F | 21 | [ |
CHAN A Y K | 2005 | Hongkong, China | 1 | F | 59 | [ |
IDEGUCHI H | 2007 | Japan | 1 | F | 82 | [ |
SUZUKI T | 2003 | Japan | 1 | F | 54 | [ |
GUPTA R K | 2011 | Vietnam | 1 | F | 23 | [ |
FRANZÉN P | 1987 | Finland | 1 | F | 24 | [ |
HECK L W | 1985 | USA | 1 | F | 17 | [ |
HARA A | 2008 | Japan | 1 | F | 42 | [ |
MARMONT A M | 2014 | Italy | 1 | F | 45 | [ |
MEYER R J | 1978 | China | 1 | F | 56 | [ |
FALLAHI S | 2014 | Iran | 1 | F | 54 | [ |
ARCASOY M O | 2005 | USA | 1 | F | 34 | [ |
MOLLAEIAN A | 2020 | African | 1 | M | 65 | [ |
BISWAL N | 2007 | India | 1 | F | 9 | [ |
NAKAMURA H | 2010 | Japan | 1 | F | 47 | [ |
ILAN Y | 1993 | Iseral | 1 | F | 22 | [ |
TAKIGAWA M | 1974 | Japan | 1 | F | 44 | [ |
HARADA N | 1990 | Japan | 1 | F | 27 | [ |
ATSUMI T | 1991 | Japan | 1 | M | 21 | [ |
JAREK M J | 1996 | USA | 1 | F | 69 | [ |
MINAMI R | 2006 | Japan | 1 | F | 33 | [ |
MURAYAMA J | 2006 | Japan | 1 | F | 48 | [ |
SAEED M | 2021 | Pakistan | 1 | F | 27 | [ |
TOMAC-STOJMENOVIĆ M | 2017 | Croatia | 1 | F | 35 | [ |
DU A X | 2006 | Taiwan, China | 1 | F | 24 | [ |
JIMENO S A | 2001 | Spain | 1 | F | 19 | [ |
Demographic data | Number of patient/n (%) | Age/year | Interval between two diagnoses/year |
---|---|---|---|
Diagnosed as SLE first | 22 (43.71) | 35.77 | 6.95 |
Diagnosed as PRCA first | 12 (23.08) | 30.83 | 3.22 |
Diagnosed as SLE and PRCA simultaneously | 18 (34.62) | 37.22 | 0 |
表2 诊断为SLE/PRCA时的患者特征
Tab 2 Characteristics of patients diagnosed with SLE/PRCA
Demographic data | Number of patient/n (%) | Age/year | Interval between two diagnoses/year |
---|---|---|---|
Diagnosed as SLE first | 22 (43.71) | 35.77 | 6.95 |
Diagnosed as PRCA first | 12 (23.08) | 30.83 | 3.22 |
Diagnosed as SLE and PRCA simultaneously | 18 (34.62) | 37.22 | 0 |
Laboratory indicator | Positive case/ reported case/n | Positive rate/% |
---|---|---|
ANA | 47/47 | 100.00 |
Anti-dsDNA antibody | 38/43 | 88.37 |
Hypocomplementemia | 32/39 | 82.05 |
Urinary protein | 23/33 | 69.69 |
ACA | 10/20 | 50.00 |
Anti-Sm antibody | 7/18 | 38.89 |
表3 实验室指标
Tab 3 Laboratory indicators
Laboratory indicator | Positive case/ reported case/n | Positive rate/% |
---|---|---|
ANA | 47/47 | 100.00 |
Anti-dsDNA antibody | 38/43 | 88.37 |
Hypocomplementemia | 32/39 | 82.05 |
Urinary protein | 23/33 | 69.69 |
ACA | 10/20 | 50.00 |
Anti-Sm antibody | 7/18 | 38.89 |
Treatment | Number of patient/n (%) |
---|---|
GC | 51 (98.08) |
BT | 34 (65.38) |
CsA | 13 (25.00) |
IVIG | 9 (17.31) |
CTX | 9 (17.31) |
HCQ | 8 (15.38) |
EPO | 5 (9.62) |
AZA | 5 (9.62) |
MMF | 2 (3.85) |
PE | 2 (3.85) |
RTX | 2 (3.85) |
MTX | 1 (1.92) |
表4 治疗措施
Tab 4 Treatment measures
Treatment | Number of patient/n (%) |
---|---|
GC | 51 (98.08) |
BT | 34 (65.38) |
CsA | 13 (25.00) |
IVIG | 9 (17.31) |
CTX | 9 (17.31) |
HCQ | 8 (15.38) |
EPO | 5 (9.62) |
AZA | 5 (9.62) |
MMF | 2 (3.85) |
PE | 2 (3.85) |
RTX | 2 (3.85) |
MTX | 1 (1.92) |
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