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. 2017 Aug;6(3):163–171. doi: 10.5582/irdr.2017.01044

Table 2. Studies on Association of antiphospholipid antibody with pulmonary hypertension.

Author/Year Type of Study Number of subjects Results
Karmochkine et al. (55) 1996 Prospective n = 38 PH (9PPH+29 SPH) aPL (11/30) +aPL in pre capillary PAH
(4/9) +aPL in PPH (44%)
(7/29) +aPL in SPH (24%)
 
Wolf et al. (45) 2000 Prospective n = 116: CTEPH
n = 83: PPH
(25/116) CTEPH + APA
(8/83) PPH +APA
Among 33 CTEPH or PPH patients:
    (22) + IgG
    (7) + IgG+IgM
    (4) + IgM,
High titers of antibody 20% in CTEPH & 10% in PPH
 
Tanaseanu et al. (56) 2007 Prospective 30 SLE (15PH+/15PH-)

30 COPD (15 PH+/15PH-)
Frequency of aPL antibody:
    SLE: (15/15(100%) in SLE/+PH) & (33% in SLE/-PH)
    COPD: (8/15(53%) in COPD/+PH) & (13% IN COPD/-PH)
 
Fois et al. (19) 2010 Retrospective 12 patients: SLE/+PH
9 SLE/+ isolated PH (Group1); 1 SLE/+ PH due to left heart disease(group2); 2 SLE/+PH related to lung disease (group3)
(9/12) SLE/+PH had a + aCL antibody (frequency 75%)

2 SLE/+PH related due to lung disease: +aCL antibody 2/2 in this group
 
Kamel et al. (57) 2011 Cross-sectional Total SLE: 74
SLE/+PH: 8
7/8 (88%): SLE with PH/ + (aPL IgG), p = 0.08 Linear regression analyisis: + aCL were significantly associated with PAH occurrence in SLE with p value of 0.006
 
Cefle et al. (18) 2011 Retrospective 10: SLE/+PH
97: SLE/-PH
SLE/+PH: Total 10
    2: + aCL IgG
    4: + both aCL IgG & + aCL IgM
    1: + aCL IgG, + aCLIgM, + LAC
    1: + aCL IgM, + LAC
    aPL positivity: SLE/+PH (80%) vs. SLE/-PH (36%), p < 0.005.
 
Ware et al. (58) 2015 Cross-sectional, observational n = 50 SLE
23/50: aPL +
11/23: aPL+/+PH
(11/23) aPL+/+PH
(14/ 23) + aCL
(3 /23) + LAC
(16/23) + Anti-beta-2-glycoprotein I antibodies
(11) + 2 or more antibodies
(PHT) 11/23 APA+ & 2/ 27 APA −
Moderate to severe PH (7/ 11) APA +
 
Zuily S et al. (59) 2017 Meta analysis 4,480 SLE Prevalence of PH: + aPL(12.3%) vs. - aPL (7.3%)
The highest risk for PH: +LAC & + IgG aCL
Low risk: IgM aCL & anti-β-2-glycoprotein I antibodies

aCL: anticardiolipin antibodies; aPL: antiphospholipid antibodies; COPD: chronic obstructive pulmonary disease; CTEPH: Chronic thromboembolic pulmonary hypertension; LAC: lupus anticoagulant; PAH: pulmonary arterial hypertension; PH: Pulmonary hypertension; PPH: primary pulmonary hypertension; SLE: systemic lupus erythematosus; SPH: secondary pulmonary hypertension.