Background Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure, untreated resulting in right ventricular heart failure. PH can be based on idiopathic pulmonary arterial hypertension (iPAH) or associated with connective tissue diseases such as systemic sclerosis (SSc-PAH). PH is also caused by left heart disease (LHD-PH), chronic obstructive pulmonary disease (COPD-PH), or chronic thromboembolic events (CTEPH).
Objectives In this study analysis of microvascular patterns of microangiopathy of patients with PH have been performed using nailfold videocapillaroscopy (NVC). The benefit of this easy to handle diagnostic tool in PH was evaluated with a distinct focus on SSc patients.
Methods NVC was performed in 81 patients, of whom 54 had PH due to different disease entities. 27 had systemic sclerosis without PAH. 2nd to 5th fingers were bilaterally analyzed. Three pictures of adequate quality for evaluation, at least one from each hand, were scored for capillary density (CD, capillaries per mm), afferent, efferent, apex and total width. Parameters such as hemorrhages, neoangiogenesis and edema or capillary alterations such as ectasia (>20μm), irregular shape, tortuousity or giant shape (>50μm) were qualitatively assessed. For SSc patients, the respective pattern of microangiopathy (“early”, “active” or “late”) was determined.
Results 14.8% of the patients had iPAH, 14.8% LHD-PH, 7.4% COPD-PH and 17.2% CTEPH. 45.7% had SSc, of whom 12.3% had SSc-PAH. In SSc, 32% showed the “early” (CD 6.5±1.6), 41% the “active” (CD 4.2±1.2) and 27% the “late” pattern (CD 3.5±0.9). The CD in SSc-PAH was significantly lower compared to all other PH forms (4.9 vs. 10.2, 10.0, 11.7 and 9.47 in iPAH, LHD-PH, COPD-PH and CTEPH; <0.0001), but did not differ compared to SSc non-PAH (4.7; p=0.73). In general, afferent, efferent, apex and total width of capillaries were larger in SSc-PAH. Ectasia was very common in SSc-PAH (90%), but to some extent also present in other forms, most frequently in COPD-PH (71.4%). Giant capillaries were generally only present in SSc (84.6% and 70%). Hemorrhages occurred in all disease forms of this study, however mostly in SSc (80%) and COPD-PH (85.7%).
Conclusions Assessing capillary density in PH is a powerful tool to discriminate between SSc-PAH and other forms of PH. In this respect, qualitative parameters such as giant shape can support the elucidation of the underlying disease. Besides checking for antinuclear antibodies, NVC should be considered, if the underlying cause of PH is unclear.
Disclosure of Interest None Declared
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