Riociguat for interstitial lung disease and pulmonary hypertension: a pilot trial

Hoeper, Marius M.; Halank, Michael; Wilkens, Heinrike; Günther, Andreas; Weimann, G; Gebert, Irmingard; Leuchte, Hanno; Behr, Jürgen

doi:10.1183/09031936.00213911

Cited by 135 publications

(114 citation statements)

References 31 publications

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“…The safety, tolerability and preliminary efficacy of riociguat (a soluble guanylate cyclase stimulator) has been evaluated in an open-label, uncontrolled pilot trial in patients with PH and ILDs with some positive results. Riociguat was well tolerated and was associated with an increase in cardiac output and decrease in PVR at RHC evaluation after 12 weeks of treatment; mean PAP was unchanged [47]. Riociguat shows the potential to improve exercise capacity in some patients (evaluated using 6MWT distance) [47].…”

Section: Treatmentmentioning

confidence: 99%

Pulmonary hypertension in chronic interstitial lung diseases

Caminati¹,

Cassandro²,

Harari³

2013

European Respiratory Review

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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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Section: Treatmentmentioning

confidence: 99%

Pulmonary hypertension in chronic interstitial lung diseases

Caminati¹,

Cassandro²,

Harari³

2013

European Respiratory Review

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“…5 inhibitors) in appropriately designed trials are also necessary to study the effect of these drugs in these patients [18,39,40]. It is important to point out, however, that the presence of emphysema and abnormal pulmonary pathology in patients with CPFE and pulmonary hypertension may be associated with an imbalance in the ventilation/perfusion ratio (V9/Q9), as hypoxic vasoconstriction is one of the main mechanisms to avoid worsening arterial oxygenation.…”

Section: Review: Emphysema In Pulmonary Fibrosis V Cottinmentioning

confidence: 99%

“…It is important to point out, however, that the presence of emphysema and abnormal pulmonary pathology in patients with CPFE and pulmonary hypertension may be associated with an imbalance in the ventilation/perfusion ratio (V9/Q9), as hypoxic vasoconstriction is one of the main mechanisms to avoid worsening arterial oxygenation. Vasodilator drugs can worsen hypoxaemia by inhibiting this mechanism [39][40][41].…”

Section: Review: Emphysema In Pulmonary Fibrosis V Cottinmentioning

confidence: 99%

The impact of emphysema in pulmonary fibrosis

Cottin¹

2013

European Respiratory Review

131

115

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Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Physiological testing of these patients reveals preserved lung volume indices contrasted by markedly impaired diffusion capacity. The incidence of CPFE remains unknown but several case series suggest that this subgroup may comprise up to 35% of patients with IPF. CPFE is a strong determinant of associated pulmonary hypertension (PH). In addition, CPFE has major effects on measures of physiological function, exercise capacity and prognosis, and may affect the results of pulmonary fibrosis trials. Further studies are needed to ascertain the aetiology, morbidity, mortality and management of the CPFE syndrome, with or without PH, and to evaluate novel therapeutic options in CPFE.

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“…Hoeper et al reported the decrease of SaO 2 by 1−2% from baseline in the patients with PH (mPAP > 30 mm Hg) in the course of DPLDs treated with Riociguat in the observational study [64]. Nevertheless, mixed venous oxygen saturation slightly increased.…”

Section: Treatment Considerations Of Ph In the Course Of Dpldsmentioning

confidence: 99%

Pulmonary Hypertension in the Course of Diffuse Parenchymal Lung Diseases—State of Art and Future Considerations

Szturmowicz¹,

Kacprzak²,

Błasińska‐Przerwa³

et al. 2015

Advances in Respiratory Medicine

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Lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. According to the most recent publications, PH in the course of lung diseases develops as a result of both "parenchymal" and vascular pathology, in the patients with genetic predisposition. Prolonged infection (especially viral one) may be an additional promoting factor. Right heart catheterization (RHC), which is an invasive procedure, is the only objective method of diagnosing PH. According to the latest recommendations, the management algorithm of PH and coexisting interstitial lung disease is based on RHC and the results of pulmonary function tests. Majority of the patients develop mild PH in the course of advanced lung disease. Best treatment of underlying lung pathology combined with long term oxygen treatment is recommended in this group. In case of severe PH (mean resting pulmonary artery pressure (mPAP) ≥ 35 mm Hg) the alternate cause of PH has to be sought. PAH-specific drugs use should be limited to patients with severe PH participating in clinical trials. In this review, the value of various non-invasive methods (echocardiography, radiological examination, exercise capacity and brain natriuretic peptides assessment) in the process of screening for PH is presented, and the results of recent randomized clinical trials with PAH-specific drugs in patients with diffuse parenchymal lung diseases are discussed.

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Riociguat for interstitial lung disease and pulmonary hypertension: a pilot trial

Cited by 135 publications

References 31 publications

Pulmonary hypertension in chronic interstitial lung diseases

Pulmonary hypertension in chronic interstitial lung diseases

The impact of emphysema in pulmonary fibrosis

Pulmonary Hypertension in the Course of Diffuse Parenchymal Lung Diseases—State of Art and Future Considerations

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