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Improving definitions for an index of cumulative organ damage in patients with the antiphospholipid syndrome (DIAPS)

Sir, I read the manuscript by Amigo et al. regarding the development and initial validation of a damage index (DIAPS) for patients with thrombotic antiphospholipid syndrome (APS). Now, the importance of evaluating the effect of medical interventions in terms of reducing the chance of disease-specific organ damage over time is clear, and DIAPS would provide clinicians with a useful instrument for the assessment of APS patients. However, there are some issues needing clarification to improve the reliability and accuracy of this valuable index. In the glossary, Amigo et al. defined the item ‘‘Pulmonary arterial hypertension’’ as ‘‘Pulmonary artery pressure >25mmHg at rest or >30mmHg on exercise’’. As noted, they used the term pulmonary arterial hypertension (PAH) instead of pulmonary hypertension (PH). Also, the paper does not define whether cut-offs for pulmonary arterial pressure (PAP) are mean (mPAP) or systolic (sPAP) values, and the methods employed for measuring such pressure are not described. At this point, guidelines and statements from several international consensuses on PH should be included in the DIAPS. First, PH is a pathophysiological disorder defined by mPAP 25mmHg at rest, measured during right heart catheterization, while the term PAH describes a subgroup of patients with pre-capillary PH in the absence of intrinsic lung diseases or chronic thromboembolism. Therefore, PH and PAH are not interchangeable terms. Second, although catheterization is the only method to definitively establish a hemodynamic diagnosis of PH, it is expensive and not easily accessible, thus transthoracic echocardiography is now the most practical modality to assess right heart pressures in individuals with suspected PH. Correspondingly, a diagnosis of PH can be inferred on a combination of peak tricuspid regurgitation velocity and other echocardiographic measurements (Table 1). Notably, these echocardiogram-based definitions of PH are now validated in lupus patients. Finally, a definition of PH on exercise as mPAP >30mmHg is not supported by published data, and, additionally, healthy individuals commonly exceed this threshold, which makes it difficult to define normal PAP values during exercise. A recent study suggests that combining mPAP and total pulmonary resistances may define a pathological hemodynamic response of the pulmonary circulation during exercise. In addition, overall management of APS patients should be directed not only to prevent death, but also to reduce the morbidity resulting from this disease and its therapy. Therefore, it is clear that a clinimetric scale aimed to estimate this morbidity needs to include the main complications related to therapy. In this regard, the inclusion of ischemic stroke in the DIAPS instead of considering any type of cerebrovascular accident is noticeable, thus excluding those patients suffering a hemorrhagic stroke in the setting of anticoagulation therapy. This is particularly relevant given the notion