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Research ArticleArticle

Telangiectases in Scleroderma: A Potential Clinical Marker of Pulmonary Arterial Hypertension

AMI A. SHAH, FREDRICK M. WIGLEY and LAURA K. HUMMERS
The Journal of Rheumatology January 2010, 37 (1) 98-104; DOI: https://doi.org/10.3899/jrheum.090697
AMI A. SHAH
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  • For correspondence: ashah32@jhmi.edu
FREDRICK M. WIGLEY
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LAURA K. HUMMERS
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Article Figures & Data

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  • Figure 1.
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    Figure 1.

    Two different types of telangiectasia: (A) matted; (B) stellate.

  • Figure 2.
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    Figure 2.

    The mean telangiectasia score increased by 2.5 for every 10-year increase in scleroderma disease duration (p < 0.001).

  • Figure 3.
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    Figure 3.

    The mean RVSP increased by 10.8 mm Hg for every 10-point increase in telangiectasia score (p = 0.001).

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    Figure 4.

    Total telangiectasia score by pulmonary arterial hypertension (PAH) status. PAH is defined as either an estimated right ventricular systolic pressure (RVSP) ≥ 45 mm Hg or right-heart catheterization–confirmed PAH.

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    Table 1.

    Baseline characteristics of study participants.

    VariablesTelangiectasia Score 0–3, n = 54Telangiectasia Score 4–7, n = 50Telangiectasia Score 8+, n = 43p
    Age, yrs, mean (SD)47.8 (11.1)57.6 (13.9)58.5 (12.1)< 0.0001
    Women, no. (%)47 (87.0)41 (82.0)39 (90.7)0.52
    Race, no. (%)0.007
      White33 (61.1)37 (74.0)39 (90.7)
      African American16 (29.6)9 (18.0)3 (7.0)
      Asian5 (9.3)2 (4.0)0 (0)
      Other0 (0)2 (4.0)1 (2.3)
    Scleroderma classification, no. (%)0.19
      Limited28 (51.9)29 (58.0)30 (69.8)
      Diffuse26 (48.1)21 (42.0)13 (30.2)
    Disease duration, yrs, mean (SD)6.2 (5.5)8.4 (5.2)14.6 (10.9)< 0.0001
    Smoking, no. (%)0.03
      Never36 (66.7)25 (50.0)18 (41.8)
      Former10 (18.5)21 (42.0)19 (44.2)
      Current8 (14.8)4 (8.0)6 (14.0)
    Vascular disease, no. (%)
      Peripheral vascular disease1 (1.9)3 (6.0)0 (0)0.21
      Coronary artery disease2 (3.7)7 (14.0)4 (9.3)0.18
      Atherosclerotic CVD0 (0)0 (0)1 (2.3)0.29
      Hypertension8 (14.8)14 (28.0)11 (25.6)0.22
    Renal disease, no. (%)3 (5.6)5 (10.0)7 (16.3)0.23
    FVC (% predicted), mean (SD), n = 14373.8 (20.5)74.2 (17.9)73.5 (21.2)0.98
    Autoantibody status, no. (%)
      Centromere, n = 1466 (11.1)13 (26.0)13 (31.0)0.04
      Scl-70, n = 14617 (31.5)8 (16.0)6 (14.3)0.08
      Ribonucleoprotein, n = 1428 (15.1)6 (12.5)1 (2.4)0.11
    Medication use, no. (%)
      Calcium channel blocker28 (51.9)26 (52.0)24 (55.8)0.94
      Phosphodiesterase inhibitor1 (1.9)3 (6.0)12 (27.9)< 0.001
      Endothelin antagonist4 (7.4)2 (4.0)3 (7.0)0.76
      Prostacyclin1 (1.9)1 (2.0)3 (7.0)0.44
      Antiplatelet10 (18.5)15 (30.0)14 (32.6)0.24
      Anticoagulation1 (1.9)6 (12.0)5 (11.6)0.07
      ACE inhibitor or ARB20 (37.0)15 (30.0)29 (44.2)0.37
      Statin11 (20.4)14 (28.0)10 (23.3)0.65
    • CVD: cerebrovascular disease; FVC: forced vital capacity; Scl: scleroderma; ACE: angiotensin-converting enzyme, ARB: angiotensin receptor blocker.

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    Table 2.

    Mean RVSP and DLCO by tertile of telangiectasia score.

    VariablesTelangiectasia Score 0–3, n = 54Telangiectasia Score 4–7, n = 50Telangiectasia Score 8+, n = 43p
    RVSP, mean (SD)34.4 (8.8)38.1 (15.6)48.1 (25.4)0.0007
    DLCO, mean (SD)69.8 (26.0)70.6 (25.9)61.9 (27.8)0.24
    • RVSP: right ventricular systolic pressure; DLCO: diffusion capacity.

    • View popup
    Table 3.

    Crude and adjusted change in mean RVSP.

    ßCrude (95% CI)pßAdjusted* (95% CI)p
    Telangiectasia score (per 10-point increase)10.8(4.5, 17.2)0.00110.9(3.6, 18.3)0.004
    Age (per 10 yrs)4.0(1.8, 6.1)< 0.0012.0(−0.5, 4.6)0.12
    Race/ethnicity**0.560.55
      Black−2.3(−10, 5.5)3.6(−4.1, 11.3)
      Hispanic−18.9(−44.5, 6.7)−16.1(−40.0, 7.8)
      Asian/Pacific Islander−5.1(−20.1, 9.9)3.1(−11.6, 17.7)
      Mid-East/Arabian−10.6(−46.7, 25.4)−5.6(−39.9, 28.7)
    Scleroderma subtype (diffuse vs limited)−6.1(−12.1, −0.1)0.047−1.8(−8.6, 4.9)0.59
    Scleroderma disease duration (per 10 yrs)3.3(−0.2, 7.0)0.07−0.5(−4.5, 3.6)0.81
    Autoantibody status
      Anticentromere5.8(−1.4, 12.9)0.114.0(−3.7, 11.7)0.31
      Anti-Scl 70−0.1(−7.4, 7.3)0.994.8(−2.5, 12.1)0.19
      Anti-RNP5.6(−3.9, 15.1)0.258.6(−1.0, 18.2)0.08
    Smoking history0.020.26
      Former vs never8.7(2.3, 15.1)5.0(−1.7–11.7)
      Current vs never−0.6(−10.0, 8.7)−1.9(−11.3, 7.4)
    • ↵* Adjusted model includes telangiectasia score, age, race, scleroderma subtype, disease duration, autoantibody status and smoking status.

    • ↵** White (reference). RVSP: right ventricular systolic pressure; CI: confidence interval; Scl: scleroderma; RNP: tktktktk.

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Telangiectases in Scleroderma: A Potential Clinical Marker of Pulmonary Arterial Hypertension
AMI A. SHAH, FREDRICK M. WIGLEY, LAURA K. HUMMERS
The Journal of Rheumatology Jan 2010, 37 (1) 98-104; DOI: 10.3899/jrheum.090697

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Telangiectases in Scleroderma: A Potential Clinical Marker of Pulmonary Arterial Hypertension
AMI A. SHAH, FREDRICK M. WIGLEY, LAURA K. HUMMERS
The Journal of Rheumatology Jan 2010, 37 (1) 98-104; DOI: 10.3899/jrheum.090697
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