Editorial article
Pulmonary hypertension in scleroderma
Hipertensión pulmonar en la esclerodermia
Vicent Fonollosa-Pla
, Carmen Pilar Simeón-Aznar
Corresponding author
Servicio de Medicina Interna, Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Universitario Vall d’Hebron, Universidad Autónoma de Barcelona, Barcelona, Spain
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"tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "21" "paginaFinal" => "23" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Vicent Fonollosa-Pla, Carmen Pilar Simeón-Aznar" "autores" => array:2 [ 0 => array:4 [ "nombre" => "Vicent" "apellidos" => "Fonollosa-Pla" "email" => array:1 [ 0 => "vfonollo@vhebron.net" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Carmen Pilar" "apellidos" => "Simeón-Aznar" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Medicina Interna, Unidad de Enfermedades Autoinmunes Sistémicas, Hospital Universitario Vall d’Hebron, Universidad Autónoma de Barcelona, Barcelona, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Hipertensión pulmonar en la esclerodermia" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">In the early nineties, Carwile LeRoy published an article in the American Journal of Medicine whose title could not escape the well-founded pessimism pervading the diagnosis of pulmonary hypertension in patients with connective tissue diseases: Pulmonary Hypertension: The bête noire of the diffuse connective tissue diseases.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">1</span></a> Undoubtedly, this expression came as a result of the reality that accompanied this sad pathological situation, which had already been collected, perhaps too many years before, in the following text, making it even more disturbing: “The CREST syndrome is not always benign because, after a prolonged clinical course, it may develop a progressive pulmonary vascular obliteration, pulmonary hypertension and death, in the absence of significant pulmonary fibrosis.”</p><p id="par0010" class="elsevierStylePara elsevierViewall">This was the conclusion reached by Salerni et al.,<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">2</span></a> in 1977, after describing and analyzing 10 cases of patients with scleroderma (SCL) that developed pulmonary hypertension (PH) during the clinical course. And they could not be more eloquent. Two messages emerge from their words: one that PH is a manifestation to take into account in patients with SCL, and secondly, it is a serious complication with a capacity to cause a fatal outcome for the patient.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Among the connective tissue diseases (CTD) that may develop PH, the SCL is in first place and with a considerable difference compared to other connective tissue diseases. In the records of patients with CTD and pulmonary arterial hypertension (PAH), SCL monopolizes about 65% of cases.<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">3</span></a> And in turn, the prevalence of PAH in patients with SCL is estimated at around 10–12%, with only a slight predominance of the limited form over the diffuse one.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">4</span></a> PH in SCL is part of the group 1 (PAH) of the PH classification, but it can also have its expression in the form of pulmonary veno-occlusive disease; as secondary to left heart disease; in connection with diffuse interstitial lung disease (ILD) and, more rarely, as PH caused by chronic thromboembolic disease.<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">5</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Just by themselves, patients with SCL constitute a population group at risk of developing PH, regardless of any clinical aspects that may have been linked to its presence. Among them: the limited clinical form, the onset of SCL in later stages of life, patients with more than 10 years of disease progression and reduced diffusion capacity for carbon monoxide (DLCO). Other manifestations that have been associated with PH are: positive for centromere antibodies, intense Raynaud's phenomenon and severe digital ischemia.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">6</span></a> Some studies have established in a more precise manner the characteristics that define the patient with SCL and PH: above 60<span class="elsevierStyleHsp" style=""></span>years of age, mostly women, limited form and DLCO less than 50%.<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">7</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The clinical manifestations in patients with SCL and PH are, in the early stages, of poor symptomatic expression. Exertional dyspnea is, in many cases, the predominant symptom and acquires a justified relevance, regarding diagnosis suspicion, when it has no explainable origin. The rest of associated symptoms can include: cough, syncope, palpitations and chest pain. Along with exertional dyspnea, from an inconclusive cause, the decrease in DLCO with a FVC/DLCO ratio >1.6–1.8 is a sign that almost certainly gives evidence of an obliteration of the pulmonary vascular bed. The Doppler echocardiography can then confirm: tricuspid regurgitation speed greater than 3<span class="elsevierStyleHsp" style=""></span>m/s and/or pulmonary arterial systolic pressure above 40–50<span class="elsevierStyleHsp" style=""></span>mmHg. Increased Nt-proBNP natriuretic factor completes the set of signs that warn of the presence of PH in the SCL.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The diagnosis is confirmed by right heart catheterization when it demonstrates a mean pulmonary arterial pressure (mPAP) ≥25<span class="elsevierStyleHsp" style=""></span>mmHg at rest and a pulmonary capillary pressure (PCP) ≤15<span class="elsevierStyleHsp" style=""></span>mmHg, if it is PAH. When PCP is ≥15<span class="elsevierStyleHsp" style=""></span>mmHg, the origin of the PH should be sought in a left heart disease, not uncommon, by the way, in patients with SCL. PAH can also be, as already mentioned, secondary to ILD, but sometimes is an addition to the presence of the ILD. This PAH may be due to a primary vascular injury–with a mPAP, then, disproportionate to the degree of restrictive pattern registered–, which greatly worsens the prognosis of the patient.</p><p id="par0035" class="elsevierStylePara elsevierViewall">PH is, together with the diffuse clinical form, the late onset of the disease, pulmonary fibrosis and renal crisis, a poor prognosis factor, and lung involvement, both in the form of ILD and/or PAH, is in the leading positions regarding causes of death by SCL.<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">8</span></a> The fact that Koh et al.,<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">9</span></a> recorded, in the mid-nineties in a series of 17 patients with SCL and PAH, a survival of 50% at 12 months of diagnosis, or the availability, in early 2000, of new drugs that improved life expectancy for PAH patients, as well as the benefits observed in survival when the diagnosis of PAH and the start of treatment was carried out at an early stage, have been sufficient stimulus to develop a strategy, almost persecutory, with which to detect and treat PAH early in these patients.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">10</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The benefits arising from the practice of screening for PH are obvious, as indicated in the conclusions of the study published in this issue of <span class="elsevierStyleSmallCaps">Medicina Clínica.</span><a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">11</span></a> Several studies show that the survival of patients with SCL and PH is greater when a protocol aimed at discovering the PH is established, rather than the PH being diagnosed following the clinical manifestations of same.<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">12</span></a> Thus, it has been recommended that patients with SCL, even without symptoms of PH, and regardless of the clinical form, should undergo pulmonary function tests, including the DLCO, an echocardiogram and a Nt-proBNP measurement, annually. From the results obtained in these investigations (i.e., FVC/DLCO ratio >1.8; sPAP: 40–50<span class="elsevierStyleHsp" style=""></span>mmHg; Nt-proBNP >210<span class="elsevierStyleHsp" style=""></span>pg/ml) will depend the corresponding right heart catheterization indication. The interest in reaching an early diagnosis of PH in the SCL has raised multiple decision algorithms.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">10</span></a> Most of them value data obtained from the additional tests outlined above, and in some of the studies, such as DETECT, only 4% of patients that were part of the investigation remained undiagnosed of PH.<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">13</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The peculiarities of patients with idiopathic PAH (IPAH) and those with PAH due to SCL have been compared in different series of PH patients. The results have shown that even though the latter (PAH due to SCL) had better hemodynamics, prognosis and, ultimately, survival is worse than in the IPAH cases, with the addition of a worse response to treatment.<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">6,14</span></a> The paradox is explained by evidence pointing to the fact that the contractility of the right ventricle (RV) in patients with SCL and PAH is decreased compared to that of patients with IPAH.<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">10,15</span></a> The differential factor lies in the cardiac condition of the SCL, with its various forms of expression, and especially, cardiopathy by myocardial fibrosis. Thus, in the SCL, the intrinsic ventricular systolic dysfunction is responsible for the poor prognosis and decreased survival of patients with PAH and SCL, more so than the differences observed in pulmonary preload.</p><p id="par0050" class="elsevierStylePara elsevierViewall">As already mentioned, the PH is a serious complication of the SCL and, together with interstitial lung disease, is a major cause of morbidity and mortality. Survival has improved, no doubt, but the figures are still far from reaching the desirable goal. In recent studies, where research has followed a targeted protocol, the survival rate at year 1, 2 and 3 was 93, 88 and 75%, respectively. The same research has found that age >60 years, male gender, functional class <span class="elsevierStyleSmallCaps">IV</span> and DLCO <39% are significant mortality indicators. It has also been established that reduced DLCO and functional class <span class="elsevierStyleSmallCaps">IV status</span> at the time of diagnosis of PAH is a poor prognosis for these patients.<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">16</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The treatment of PAH in the SCL derives from the global clinical trials on HT, with cohorts formed mostly by IPAH patients. This means that the representation of patients with SCL is only partial. Three groups of drugs lead the therapeutic arsenal: prostacyclin and its analogs; phosphodiesterase-5 inhibitors and the endothelin receptor blockers. None of them has obtained differences in survival. The order of their indication has not been established, but the severity of patient status and the experience of the prescribing physician may be considered acceptable guidelines. Among the most recently available drugs, we highlight: selexipag (oral agonist of the prostacyclin receptor), which reduces the risk of morbidity and mortality<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">17</span></a>; riociguat, stimulating soluble guanylate cyclase, independently of the presence of nitric oxide, and improves hemodynamic parameters and functional class<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">18</span></a>; and macitentan, the group endothelin receptor antagonists, which reduces morbidity and mortality compared to placebo,<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">19</span></a> all of them without a specific analysis of the group of patients with SCL and PAH, as it was the case in previous trials. Because a single drug rarely leads to sustained clinical improvement, the combination therapy option has been proposed.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">10</span></a> Lung transplant is the last therapeutic option, with a survival similar to that observed in patients with fibrosis or IPAH.<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">6</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">In 1991, E. Carwile LeRoy noted: pulmonary hypertension was “<span class="elsevierStyleItalic">the bête noire of the connective tissue diseases</span>”, and he was right. In early 2000, J. Varga<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">20</span></a> published an article entitled: <span class="elsevierStyleItalic">Pulmonary Hypertension in Systemic Sclerosis: bête noire no more?</span> Despite the undeniable progress made, we hope we don’t have to wait too long in order to suppress the question mark.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Fonollosa-Pla V, Simeón-Aznar CP. Hipertensión pulmonar en la esclerodermia. Med Clin (Barc). 2016;146:21–23.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:20 [ 0 => array:3 [ "identificador" => "bib0105" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pulmonary hypertension: the bête noire of the diffuse connective tissue diseases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "E.C. 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