Pulmonary hypertension in the crest syndrome variant of systemic sclerosis

Abstract

Pulmonary hypertension (PHT) occurred in 59 (9%) of 673 systemic sclerosis patients seen between 1963 and 1983. In 30 patients, all with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), the pulmonary hypertension was isolated, i.e., independent of other pulmonary or cardiac conditions. In 20 patients, isolated PHT was demonstrated by cardiac catheterization. All had normal or only mildly decreased lung volumes, and mild or no pulmonary interstitial fibrosis on chest roentgenogram. In comparison with 287 CREST syndrome patients without PHT, these 20 patients had markedly reduced diffusing capacity for carbon monoxide (DLco) (mean 39% of predicted normal). In 6 patients, the low DLco antedated clinical evidence of PHT by 1–6 years. At autopsy there was marked intimal fibrosis with hyalinization and smooth muscle hypertrophy in the small‐ and medium‐sized arteries, without significant parenchymal fibrosis or inflammation. Patients with isolated PHT did not respond favorably to vasodilators and had a very poor prognosis, with a 2‐year cumulative survival rate of 40%. A DLco < 45% of predicted in the absence of pulmonary interstitial fibrosis may be an important predictor of the subsequent development of isolated PHT.

Hemodynamic Predictors of Survival in Scleroderma-related Pulmonary Arterial Hypertension

“…Several studies have suggested a significant improvement in overall survival rates from 50% at 1 year in earlier studies (2)(3)(4) to up to 80% and 50% at 1 and 3 years, respectively, in more recent studies (5)(6)(7)(8) However, when compared with other types of PAH, such as idiopathic PAH (IPAH) (4, 6, 7), SSc-related pulmonary arterial hypertension (SSc-PAH) continues to have a less favorable response to modern therapy and worse survival for reasons that remain largely unexplained.…”

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

“…None of our patients had evidence of more than mild interstitial disease. In patients with CTD and 1162 LANGLEBEN ET AL minimal or no interstitial lung disease, a reduction in the DLCO may indicate the onset of PAH, presumably due to loss of perfused microvessels (30,31). However, it has previously been impossible to test the relationship of capillary surface area loss to DLCO levels in vivo in humans with PAH and to examine differences between PAH types.…”

Avaliação da prevalência de hipertensão pulmonar na esclerose sistêmica

“…A primeira série de casos de pacientes com ES e HP foi descrita por Sackner em 1964 (19) ; a partir daí outras séries foram descritas na literatura (14,16,(20)(21)(22)(23)(24)(25)(26) (Tabela 6). A comparação dos achados do presente estudo com outros da literatura se torna difícil em razão das diferenças entre as populações estudadas, a metodologia empregada para a investigação da HP e os critérios utilizados para definir a sua presença.…”

“…A comparação dos achados do presente estudo com outros da literatura se torna difícil em razão das diferenças entre as populações estudadas, a metodologia empregada para a investigação da HP e os critérios utilizados para definir a sua presença. Os estudos que utilizaram o cateterismo (CAT) de ventrículo direito para diagnóstico demonstraram uma prevalência de 5% a 12%, enquanto aqueles que empregaram o ECO revelaram taxas de prevalência mais alta, entre 13% e 35% (14,16,(19)(20)(21)(22)(23)(24)(25) .…”