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Journal of Peking University (Health Sciences) ›› 2021, Vol. 53 ›› Issue (5): 1012-1016. doi: 10.19723/j.issn.1671-167X.2021.05.034

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  • Received:2020-07-22 Online:2021-10-18 Published:2021-10-11
  • Supported by:
    Natural Science Foundation of Fujian Province(2017J01170);Natural Science Foundation of Fujian Province(2019J01184)

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CLC Number: 

  • R593.26

Figure 1

Representative rash in Case 1 A, Gottron’s sign, skin ulcers of metacarpophalangeal joints; B, heliotrope rash and erythema on the face and neck."

Table 1

Clinical manifestations before treatment with TOF"

Items Case 1 Case 2 Case 3 Case 4 Case 5
Gender Female Female Female Female Male
Age/years 44 47 37 36 56
Clinical characteristics
Rash +++ ++ +++ ++ +++
Muscle weakness + - + - +
Arthritis - - + - +
Fever + - + - +
Course of dyspnea/weeks 2 4 4 2 2
Course of RP-ILD/weeks 2 4 4 2 2
Classification of ILD NSIP+DAD NSIP+UIP NSIP+OP NSIP NSIP+DAD
Quantitative score of HRCT 22 13 19 10 25
Laboratory results
Lymphocyte/(×109/L) 0.8 0.7 0.8 1.4 0.8
CK/(IU/L) 52 19 42 84 195
LDH/(IU/L) 668 217 218 360 444
AST/(IU/L) 61 42 33 54 57
Anti-MDA5 antibody + + + + +
Anti-Ro-52 antibody + + + - +
EMG Normal Normal Not done Normal Not done
BALF examination
Cytological examination Neu, 6%;
Lym, 81%;
Mac, 12%		 Neu, 8%;
Lym, 31%;
Mac, 42%		 Not done Neu, 5%;
Lym, 21%;
Mac, 74%		 Neu, 22%;
Lym, 10%;
Mac, 68%
mNGS Pneumocystis jiroveci 603 - - - -
Pre-hospital medication MP+CTX +CSA MP+CTX MP+CTX None None

Figure 2

Pulmonary HRCT in Case 1"

Table 2

Results of relevant indexes of survived patients before and after treatment (n=4)"

Items Baseline After treatment
1 month 3 months 6 months
Quantitative score of HRCT 16.00±2.74 12.50±1.66 7.75±1.93 6.75±3.20
Oxygenation index 257.30±48.71 344.30±31.93 442.50±14.36 448.00±13.86
6-minute walk test/m 150.00±88.98 290.00±42.03 390.00±23.80 435.00±22.40
Pulmonary function index
FVC% 54.50%±4.56% 74.41%±1.16% 74.90%±1.76%
TLC% 52.88%±4.04% 76.45%±4.24% 80.10%±6.67%
DLCO% 52.58%±2.20% 64.33%±1.73% 66.53%±2.67%
ESR/(mm/h) 42.50±19.65 23.50±4.84 11.25±1.49 10.25±2.50
CRP/(mg/L) 9.70±5.52 1.50 (1.32-3.44) 1.00±0.24 1.24±0.69
Serum ferritin/(μg/L) 957.30±168.20 407.50±86.83 153.30±62.65 72.50±32.35
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