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Table 1 Comparison of characteristics between cluster 1 and cluster 2 in SLE-PAH patients at baseline assessment

From: Clinical characteristics and prognosis in systemic lupus erythematosus-associated pulmonary arterial hypertension based on consensus clustering and risk prediction model

Characteristics

Overall (n = 163)

Cluster 1 (n = 134)

Cluster 2 (n = 29)

P-value*

Demography

 Female, n (%)

157 (96.3)

130 (97.0)

27 (93.1)

0.290

 Median age at diagnosis (years)

37.0 (30.0, 49.0)

38.0 (30.0, 46.5)

33.0 (28.0, 50.5)

0.624

 Duration (Mth)

24.0 (3.0, 84.0)

24.0 (3.0, 84.0)

24.0 (2.0, 78.0)

0.713

Clinical features

 Cutaneous lupus, n (%)

41 (25.5)

33 (24.6)

8 (27.6)

0.739

 Oral or nasal ulcers, n (%)

15 (9.3)

12 (9.0)

3 (10.3)

0.732

 Raynaud’s phenomenon, n (%)

43 (26.5)

37 (21.6)

7 (24.1)

0.702

 Serositis, n (%)

100 (61.1)

77 (57.5)

23 (79.3)

0.028

 Lupus nephritis, n (%)

69 (42.6)

49 (36.6)

21 (72.4)

 < 0.001

 Arthritis, n (%)

48 (29.0)

41 (30.6)

7 (20.4)

0.489

 Alopecia, n (%)

22 (13.6)

16 (11.9)

6 (20.7)

0.233

 Vasculitis, n (%)

7 (4.3)

6 (4.5)

1 (3.4)

1.000

 Neuropsychiatric lupus, n (%)

11 (6.7)

8 (6.0)

3 (10.3)

0.415

 Cardiac disorder, n (%)a

43 (26.4)

30 (22.4)

13 (44.8)

0.013

 Mild ILD, n (%)b

25 (15.4)

21 (15.7)

4 (13.8)

1.000

 APS, n (%)

5 (3.1)

5 (3.7)

0 (0.0)

0.587

 Peripheral thrombosis, n (%)

7 (4.3)

3 (2.2)

4 (13.8)

0.020

 Thrombocytopenia, n (%)c

41 (25.2)

30 (22.4)

11 (37.9)

0.080

 Low complement, n (%)d

138 (84.7)

111 (82.8)

27 (93.1)

0.255

 WHO FC III/IV, n (%)

113 (69.3)

90 (67.2)

23 (79.3)

0.198

 SLEDAI-2 K ≥ 10, n (%)

89 (54.6)

67 (50.0)

22 (75.9)

0.011

 6MWD (m), (IQR)

436.0 (282.0, 534.0)

446.0 (310.5, 535.0)

344.0 (123.0, 468.5)

0.027

Laboratory findings

 White blood cells (109/L)

5.0 (3.7, 6.9)

4.9 (3.6, 6.8)

5.8 (4.0, 8.0)

0.157

 Hemoglobin (g/L)

102.1 (91.5, 121.0)

104.5 (93, 123.1)

96 (75.5, 111.0)

0.026

 Lymphocyte (109/L)

1.0 (0.7, 1.6)

1.1 (0.8, 1.7)

0.9 (0.6, 1.1)

0.008

 PDW (fL)

16.8 (16.4, 17.5)

16.8 (16.4, 17.5)

16.8 (16.1, 17.8)

0.854

 D-dimer (mg/L)

0.4 (0.2, 0.9)

0.4 (0.2, 0.9)

0.7 (0.4, 1.8)

0.014

 NT-proBNP (pg/ml)

3992.1 (1197.0, 12250.9)

2439.5 (913.8, 7357.7)

35000.0 (27201.7, 35000.0)

 < 0.001

 eGFR (ml/min/1.73m2)

107.9 (77.4, 120.2)

111.5 (93.8, 123.0)

35.4 (18.1, 82.4)

 < 0.001

 Creatinine (µmol/L)

58.0 (49.0, 79.0)

56.0 (47.9, 68.3)

155.0 (78.0, 297.5)

 < 0.001

 Serum uric acid (µmol/L)

341.0 (280.0, 466.0)

325.5 (258.5, 411.0)

572.0 (436.0, 611.0)

 < 0.001

 C3 (g/L)

0.6 (0.4, 0.9)

0.7 (0.5, 0.9)

0.4 (0.2, 0.8)

0.002

 C4 (g/L)

0.1 (0.1, 0.2)

0.1 (0.1, 0.2)

0.08 (0.05, 0.2)

0.231

 ESR (mm/h)

41.0 (15.0, 77.0)

41.0 (16.5, 77.8)

43.0 (7.2, 82.5)

0.449

 CRP (mg/L)

6.0 (1.5, 21.6)

6.1 (1.5, 21.7)

4.8 (1.4, 18.9)

0.682

Antibodies

 ANA, n (%)

160 (98.2)

132 (98.5)

28 (96.6)

0.447

 Anti-dsDNA antibody, n (%)

77 (47.2)

60 (44.8)

17 (58.6)

0.176

 Anti-SSA antibody, n (%)

95 (59.4)

83 (61.9)

14 (48.3)

0.174

 Anti-SSB antibody, n (%)

32 (19.6)

28 (20.9)

4 (13.8)

0.383

 Anti-Ro52 antibody, n (%)

110 (67.5)

95 (70.9)

15 (51.7)

0.046

 Anti-centromere antibody, n (%)

13 (8.0)

10 (7.5)

3 (10.3)

0.704

 Anti-Scl-70 antibody, n (%)

6 (3.7)

4 (3.0)

2 (6.9)

0.290

 Anti-nRNP/Sm antibody, n (%)

105 (64.8)

89 (66.4)

16 (55.2)

0.251

 Anti-Sm antibody, n (%)

55 (33.7)

46 (34.3)

9 (31.0)

0.734

TTE parameters

 PASP (mmHg)

55.0 (45.0, 80.0)

55.0 (45.8, 80.3)

54.0 (44.5, 74.5)

0.690

 TR (m/s)

3.4 (3.1, 4.1)

3.5 (3.1, 4.1)

3.3 (3.1, 4.0)

0.381

 LVEF (%)

64.0 (61.0, 65.0)

64.0 (62.0, 65.0)

61.0 (54.0, 65.0)

0.015

SLE treatment

 GC alone, n (%)

55 (33.5)

43 (32.1)

12 (41.1)

0.337

 HCQ, n (%)

128 (78.0)

108 (80.6)

20 (69.0)

0.167

 IS

    

  MMF, n (%)

43 (26.4)

34 (25.4)

9 (31.0)

0.530

  CYC, n (%)

39 (23.9)

36 (26.9)

3 (10.3)

0.059

 GC + IS, n (%)

98 (59.7)

85 (63.4)

13 (44.8)

0.064

PTDs, n (%)

93 (57.0)

84 (62.7)

9 (31.0)

0.002

 PDE-5I, n (%)

55 (33.7)

52 (38.8)

3 (10.3)

0.003

 ERA, n (%)

65 (39.9)

58 (43.3)

7 (24.1)

0.056

 PGI2, n (%)

18 (11.1)

15 (11.2)

3 (10.3)

1.000

 Monotherapy, n (%)

68 (41.4)

60 (44.8)

8 (27.6)

0.089

 Combination, n (%)

25 (15.2)

24 (17.9)

1 (3.4)

0.050

  1. Results are expressed as median (interquartile ranger) or number (%). The categorical variables are compared by chi-squared, and Mann–Whitney U test was used for continuous variables
  2. Abbreviations: SLE systemic lupus erythematosus, PAH pulmonary arterial hypertension, ILD interstitial lung disease, APS antiphospholipid syndrome, WHO FC World Health Organization Functional Class, SLEDAI-2 K systemic lupus erythematosus disease activity index-2000, 6MWD 6-min walking distance, PDW platelet distribution width, NT-proBNP N-terminal pro-B-type natriuretic peptide, eGFR estimated glomerular filtration rate, CRP C-reactive protein, ESR erythrocyte sedimentation rate, PASP pulmonary artery systolic pressure, TR tricuspid regurgitation, LVEF left ventricular ejection fraction, ANA antinuclear antibodies, dsDNA double-stranded DNA, RNP ribonucleoprotein, TTE transthoracic echocardiography, GC glucocorticoids, HCQ hydroxychloroquine, IS immunosuppressants, MMF mycophenolate mofetil, CYC cyclophosphamide, PTDs PAH-targeted drugs, PDE-5I phosphodiesterase-5 inhibitor, ERA endothelin receptor antagonist, PGI2 prostacyclin
  3. aCardiac disorders included pericarditis, myocarditis, cardiomyopathy, and valvulopathy
  4. bMild ILD determined by high-resolution computerized tomography
  5. cThrombocytopenia defined as platelets < 100 × 109/L
  6. dLow complement defined as C3 < 0.91 g/l or C4 < 0.14 g/l
  7. *P-value < 0.05 is shown in bold
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Arthritis Research & Therapy

ISSN: 1478-6362

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