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Pulmonary capillary endothelial metabolic dysfunction: Severity in pulmonary arterial hypertension related to connective tissue disease versus idiopathic pulmonary arterial hypertension

Pulmonary capillary endothelial metabolic dysfunction: Severity in pulmonary arterial hypertension related to connective tissue disease versus idiopat

Pulmonary capillary endothelial metabolic dysfunction: Severity in pulmonary arterial hypertension related to connective tissue disease versus idiopathic pulmonary arterial hypertension   (Citations: 5)
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Objective. Pulmonary endothelial dysfunction is intertwined with the development and progression of pulmonary arterial hypertension (PAH). Pulmonary endothelium is an active metabolic tissue in healthy human subjects. This study was undertaken to deter- mine the effects of PAH on pulmonary endothelial angiotensin-converting enzyme (ACE) activity and to identify differences between common PAH types, i.e., PAH related to connective tissue disease (PAH-CTD) versus idiopathic PAH (IPAH). Methods. Nineteen patients with PAH-CTD, 25 patients with IPAH, and 23 control subjects were eval- uated. The single-pass transpulmonary percent metab- olism (%M) and hydrolysis (both reflecting enzyme activity per capillary) of an ACE synthetic substrate were determined. In addition, the calculated functional capillary surface area (FCSA), normalized to body surface area (BSA), was determined. Results. The %M values in patients with PAH- CTD (mean SEM 53.6 3.6%) were significantly reduced compared with those in control subjects (P < 0.01) and those in patients with IPAH (P < 0.03), but were similar between the IPAH and control groups (mean SEM 66.2 3.6% and 74.7 2.7%, respec- tively). Substrate hydrolysis was also significantly re- duced in patients with PAH-CTD. The FCSA/BSA was significantly reduced in patients with PAH-CTD (mean SEM 1,068 118 ml/minute/m 2 ) and in patients with IPAH (1,443 186 ml/minute/m 2 ) com- pared with that in controls (2,948 245 ml/minute/m 2 ; P < 0.01 for both). At a given cardiac index, the FCSA/BSA tended to be lower in the PAH-CTD group than in the IPAH group. Moreover, unlike in IPAH, a linear relationship between the FCSA/BSA and the diffusing capacity for carbon monoxide (DLCO) was observed in PAH-CTD (r 0.54, P < 0.03). Conclusion. The metabolically functional pulmo- nary capillary bed appears to be reduced to an equal extent in PAH-CTD and IPAH. However, %M and hydrolysis appear to be reduced in PAH-CTD but not in IPAH, reflecting relatively diminished ACE activity on the pulmonary capillary endothelial cells of patients with PAH-CTD, and showing that pulmonary endothe- lial metabolic function differs between PAH types. This study also provides the first functional evidence that a
Journal: Arthritis and Rheumatism , vol. 58, no. 4, pp. 1156-1164, 2008
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    • ...One clinical study found reduced metabolism of ACE synthetic substrate in the pulmonary vascular bed of PAH-CTD patients, but not in primary PAH patients [8]...

    Yuko Takahashiet al. Autoantibodies to angiotensin-converting enzyme 2 in patients with con...

    • ...subtypes, namely, PAH related to connective tissue disease (PAH-CTD) and idiopathic PAH (IPAH) [51]...
    • ...Additional important information is given by the fact that PAH-CTD patients exhibited a linear relationship between FCSA/BSA and carbon monoxide diffusing capacity (DL CO ), providing the first functional evidence that the reduced DL CO values in PAH-CTD are related to the degree of FCSA loss [51]...
    • ...Asterisk p < 0.05 versus the other groups in the upper panel and the middle panel and p < 0.05 versus controls in the lower panel. (From Langleben et al [51]...

    Stylianos E. Orfanoset al. Metabolic and Clearance Function at the Pulmonary Microvascular Endoth...

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