Case 1
|
[44] 2013
|
56/M
|
ITP H. pylori infection
|
Platelet-associated anti-GPIIb/IIIa Ab
|
7.2 (serum)
|
N/A
|
N/A
|
mPSLp, IVIG, CsA
|
N/A
|
Withdrawal from HD (durations, N/A)
|
Alive
|
Case 2
|
[44] 2013
|
56/F
|
N/A
|
None
|
9.05 (serum)
|
N/A
|
6 days
|
mPSLp, PSL, CsA
|
CMV infection
|
Withdrawal from HD after 1 month
|
Alive
|
Case 3
|
[45] 2013
|
47/F
|
N/A
|
None
|
21.9 (serum)
|
PC-type
|
17 days
|
mPSLp, PSL, TCZ
|
CMV pneumonia
|
Withdrawal from HD after 2 months
|
Alive
|
Case 4
|
[28] 2016
|
47/F
|
None
|
N/A
|
4380 (serum) 1600 (CSF)
|
N/A
|
1 day
|
mPSLp, PSL
|
Respiratory failure requiring mechanical ventilation
|
Withdrawal from HD after 3 weeks
|
Alive
|
Case 5
|
[28] 2016
|
76/M
|
None
|
ANA
|
14.1 (N/A)
|
HV-type
|
10 days
|
mPSLp, mPSL sodium succinate
|
Sepsis (due to systemic, Staphylococcus aureus and, CMV infections)
|
Withdrawal from HD after about 2 weeks
|
Died
|
Case 6
|
[42] 2016
|
22/M
|
EBV infection
|
N/A
|
N/A
|
Mixed-type
|
N/A (about 2 weeks)
|
TCZ, RTX, ETP
|
Respiratory failure requiring intubation
|
Withdrawal from HD (durations, N/A)
|
Alive
|
Case 7
|
[43] 2016
|
73/M
|
DM Uveitis
|
ANA
|
5.3 (serum)
|
Biopsy was not performed
|
N/A (about 2 weeks)
|
PSL
|
DVT, DLBCL
|
Withdrawal from HD after 1 week
|
Alive
|
Case 8
|
[46] 2016
|
49/F
|
HTN
|
ANA
|
83.4 (serum)
|
HV-type
|
4 days
|
mPSLp, PSL, TCZ
|
Respiratory failure requiring mechanical ventilation, liver failure, hemorrhage from a rectal ulcer, sepsis due to CNS
|
Withdrawal from, CHDF and HD, After 2 months
|
Alive
|
Case 9
|
[19] 2016
|
48/M
|
None
|
ANA, anti-SS-A Ab anti-TPO Ab, positive direct Coombs test
|
16.8 (serum) 945 (pleural effusion)
|
N/A
|
42 days
|
mPSLp, PSL, IVIG, PE, RTX
|
Cardiogenic shock due to cardiomyopathy
|
Withdrawal from HD after 2 months
|
Alive
|
Case 10
|
[47] 2016
|
72/M
|
HTN, HL
|
None
|
46.4 (N/A)
|
Mixed-type
|
14 days
|
PSL, TCZ
|
Cerebral infarction, necrosis of the ascending colon due to infarction
|
HDF was continued until the patient’s death
|
Died
|
Case 11
|
[48] 2016
|
50/M
|
N/A
|
Decreased ADAMTS13 activity (9.9%)
|
2130 (N/A)
|
HV-type
|
9 days
|
mPSLp, PSL, TCZ
|
N/A
|
Withdrawal from HD after 3 months
|
Alive
|
Case 12
|
[17], 2017
|
59/M
|
DU, Fatty liver
|
RF
|
15.2 (N/A)
|
Biopsy was not performed
|
N/A (about 3 months)
|
mPSLp, PSL, TCZ
|
Bacterial infections (multiple times), PCP, CMV infection, Candida bacteremia
|
Withdrawal from CHDF and HDF after 5 months
|
Alive
|
Case 13
|
[20] 2017
|
72/M
|
N/A
|
Anti-SS-A Ab
|
26.8 (serum)
|
Not performed
|
N/A (about 2 weeks)
|
mPSLp, PSL, CYA, TCZ
|
Temporary cardiac arrest, hypovolemic shock and respiratory failure, Corynebacterium sepsis and pneumonia
|
Withdrawal from CHDF and HD after 3 months
|
Alive
|
Case 14
|
[49] 2017
|
70/M
|
Esophageal cancer
|
λ-type BJP, slightly decreased ADAMTS13 activity
|
33 (serum)
|
Not performed
|
21 days
|
mPSLp, PSL
|
N/A
|
Withdrawal from HD after 1 week
|
Alive
|
Case 15
|
[50] 2017
|
59/M
|
HBV infection
|
None
|
14 (N/A)
|
Mixed-type
|
17 days
|
mPSLp, PSL, TCZ, and CsA
|
Cardiac arrest possibly caused by massive ascites
|
Withdrawal from CRRT and HD after 1 month
|
Alive
|
Case 16
|
[51] 2017
|
38/M
|
DU
|
None
|
26.6 (serum)
|
HV-type
|
4 days
|
mPSLp, PSL, CsA, and TCZ
|
DIC
|
Withdrawal from CHDF and HD after 1 month
|
Alive
|
Case 17
|
[52] 2017
|
24/F
|
N/A
|
During pregnancy
|
842 (serum)
|
Biopsy was not performed
|
8 days
|
mPSLp, PSL
|
Respiratory failure required mechanical ventilation
|
Withdrawal from HD after about 3 weeks
|
Alive
|
Case 18
|
[53] 2017
|
61/F
|
N/A
|
anti-CCP Ab
|
75.9 (serum)
|
HV-type
|
7 days
|
mPSLp, TCZ, RTX
|
Multiple organ failure requiring mechanical ventilation
|
Withdrawal from CRRT and HD (durations, N/A)
|
Alive
|
Case 19
|
[33] 2018
|
50/F
|
Pityriasis rosea
|
ANA, anti-SS-A/SS-B Ab, anti-Tg/TPO Ab
|
15.9 (serum) 5480 (ascites)
|
Mixed-type
|
About 20 months after fist admission
|
mPSLp, PSL, RTX
|
CMV infection ARDS required mechanical ventilation, Septic shock due to cholecystitis
|
Withdrawal from CHDF and HD (durations, N/A)
|
Alive
|
Case 20
|
The present case
|
46/M
|
None
|
ANA, anti-SS-A Ab
|
25.2 (serum) 3310 (ascites)
|
Mixed-type
|
50 days
|
mPSLp, PSL, CsA
|
Cardiac arrest due to MI, bacterial peritonitis, systemic CMV infections, Trichosporon asahii infection in the lungs
|
CHDF was continued until the patient’s death
|
Died
|