Incidence of Pulmonary Hypertension and Its Clinical Relevance in Patients with Interstitial Pneumonias: Comparison between Idiopathic and Collagen Vascular Disease Associated Interstitial Pneumonias

Handa, Tomohiro; Nagai, Sonoko; Sato, Miki; Ueda, Shuko; Yukawa, Naoichiro; Fushimi, Yasutaka; Ito, Yutaka; Ohta, Keiji; Mimori, Tsuneyo; Mishima, Michiaki; Izumi, Tohru

doi:10.2169/internalmedicine.46.6342

Cited by 26 publications

(22 citation statements)

References 26 publications

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“…In general, the prevalence of PH was similar in studies from Europe [18,25,[40][41][42][43][44][45][47][48][49], North America [5,6,26,28,46,[50][51][52][53][54][55][56][57][58][59][60][61][62][63][64][65][66][67], Asia and Africa [37,[68][69][70][71][72][73][74][75], although the majority of evidence was reported from North America (n=23, 53%). Across studies, the prevalence of PH among IPF patients ranged from 3% to 86%; most estimates tended to be between 30% and 50% (figure 3) [25, 43, 44, 47, 50, 52-66, 68, 71-73].…”

Section: Pulmonary Hypertensionmentioning

confidence: 84%

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

et al. 2015

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Idiopathic pulmonary fibrosis (IPF) is associated with a fatal prognosis and manifests in patients over 60 years old who may have comorbidities. The prevalence and impact of comorbidities on the clinical course of IPF is unclear.This systematic literature review examined the prevalence of comorbidities and mortality associated with comorbidities in IPF patients. Relevant observational studies published in English from January 1990 to January 2015 identified via MEDLINE and EMBASE were included; bibliographies of articles were also searched.Among the 126 studies included, prevalence of pulmonary hypertension (PH) was 3-86%, 6-91% for obstructive sleep apnoea, 3-48% for lung cancer and 6-67% for chronic obstructive pulmonary disease (COPD). Nonrespiratory comorbidities included ischaemic heart disease (IHD) (3-68%) and gastrooesophageal reflux (GER) (0-94%). Mortality was highest among patients with IPF and lung cancer. Most studies assessed relatively small samples of patients with IPF.PH, COPD, lung cancer, GER and IHD are significant comorbidities; differences in IPF severity, case definitions and patient characteristics limited the comparability of findings. The identification and prompt treatment of comorbidities may have a clinically significant impact on overall outcome that is meaningful for patients with IPF. @ERSpublications Identification and prompt treatment of comorbidities has a clinically significant impact on outcome for IPF patients

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Section: Pulmonary Hypertensionmentioning

confidence: 84%

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

et al. 2015

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“…Data on the prevalence and clinical features of PH in idiopathic NSIP are sparse and basically unknown; in this setting, the level of PH is usually mild-tomoderate [49]. In an echocardiographic study, HANDA et al [50] reported the incidence of PH to be 28% among 70 patients with idiopathic interstitial pneumonia, 11 of which had NSIP. The mean systolic PAP in this group was 30.2 mmHg.…”

Section: Other Ilds Associated With Phmentioning

confidence: 99%

Pulmonary hypertension in chronic interstitial lung diseases

Caminati¹,

Cassandro²,

Harari³

2013

Eur Respir Rev

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Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed “out-of-proportion” PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity) and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH. At present, no specific therapies have been approved for the treatment of PH in patients with ILDs

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“…Consequently, the diagnosis of PH may be missed in ILD patients until signs of right heart failure develop [4,[20][21][22][23][24][25]. Moreover, specific and effective treatment for pulmonary arterial hypertension (PAH) has become available only recently and the previous lack of therapeutic options has also certainly abrogated the necessity of identifying ILD patients with associated PH.…”

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confidence: 99%

Pulmonary hypertension in interstitial lung disease: Fig. 1—

2008

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In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40%) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs.

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Incidence of Pulmonary Hypertension and Its Clinical Relevance in Patients with Interstitial Pneumonias: Comparison between Idiopathic and Collagen Vascular Disease Associated Interstitial Pneumonias

Abstract: Object This study was undertaken to investigate the frequency of pulmonary hypertension (PH), and clinical parameters associated with PH in patients with idiopathic interstitial pneumonias (IIPs) and collagen vascular disease-associated interstitial pneumonias (CVD-IPs

Cited by 26 publications

References 26 publications

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

Pulmonary hypertension in chronic interstitial lung diseases

Pulmonary hypertension in interstitial lung disease: Fig. 1—

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