Pulmonary hypertension has been shown to ...

The combination of pulmonary fibrosis and emphysema (KZSS) was first described in 1990, Wiggins et al .. Further investigation case more detailed, which is now recognized symptoms syndrome: a significant dyspnea, predisposition

this disease among male smokers, normal or nearly normal lung volume resulting from opposite effects

hyperinflation and fibrosis, and also a significant reduction in diffusion capacity

-. Later work showed that a significant pulmonary hypertension also occur in patients with KZSS and related

with short survival >> << -. However, these studies relied on transthoracic echocardiography (TTE) without evidence of right heart catheterization

(RHC) for the diagnosis of pulmonary hypertension, an approach which could have serious limitations. In this issue of European Journal of breathing, Cotten

al. The results of a retrospective multicenter cohort study of patients with pulmonary hypertension and KZSS

confirmed by RHC. Cohort study was derived from populations of patients meeting diagnostic criteria were registered KZSS

to register orphans and diseases, which are then passed annual TTE for the evaluation of pulmonary hypertension. Patients were sent to RHC their doctor managed TTE results. Those with average pulmonary artery pressure

(

r) 25 mmHg, pulmonary capillary wedge pressure of 15 mmHg and pulmonary vascular resistance (PVR) 240 dynes · s · cm

included in the analysis. The patient cohort was a mean follow-RHC after 8 months. This study not only confirms many clinical characteristics KZSS described in previous studies and provides more >> << new and important ideas. First, the average time since diagnosis of KZSS to diagnose pulmonary hypertension is 16 months

suggesting that pulmonary hypertension can occur quickly after diagnosis. Second, patients with pulmonary hypertension lasix generic no prescription

very bad weather, an estimated survival only 60% at 1 year. Thirdly, one-dimensional analysis showed physiologically

consistent pattern in which increasing

P, PVR, and heart rate and decreased cardiac index were significantly associated with higher risk of death. Finally, reduced diffusion capacity >> << lungs for carbon monoxide was associated with increased mortality. This work has several advantages. First, unlike previous studies that require a limitation of the lungs for inclusion in the study, this study used the American thoracic society / European Respiratory Society diagnostic criteria for idiopathic pulmonary fibrosis

(IPF), which were expanded to include patients with normal lung volumes. The revised criteria enlarge >> << generalized research, including a more representative population KZSS patients and avoid possible bias associated with choosing

only in patients with more advanced fibrosis. Secondly, the use of RHC to measure

P & PVR provides a more precise definition of the main exposure, thereby increasing the scientific validity of the observed

relationship between pulmonary hypertension and mortality. Finally, the authors were able to assess the impact of certain

hemodynamic parameters and clinical indicators of long-term survival. The study also has its limitations. A short mean follow-term survival estimates exclude, including the average >> << and 5-year survival, however, the frequency of events at the beginning of observation and high 1 year mortality enough to illustrate

significant effect of pulmonary hypertension on prognosis. Nonprotocolised decision of RHC can enter

selection in patients with the most significant symptoms or severe disease were selected to undergo RHC, and not continue >> << noninvasive monitoring. This shift may have overestimated the impact of pulmonary hypertension on survival. Finally, because

small number of results, the authors are unable to perform multivariate analysis to control mixed. Despite these limitations, this study makes an important contribution to our understanding KZSS as a clinical entity. But

several mechanistic and clinical questions remain. It is unclear whether the occurrence of both processes is simply

coincidence of two diseases with different mechanisms, but the main common risk factor, or whether a general >> << way in some people, leading as fibrosis and emphysema after exposure to cigarette smoke. Experimental animal models

proposed general mechanisms by which tobacco smoke can lead to both emphysema and IPF

, - but these mechanisms have not been substantiated in humans. Pulmonary hypertension has been shown that significantly more common and more severe in KZSS than any >> IPF, or emphysema << alone. It is possible that the prevalence and severity of pulmonary hypertension is simply an additional effect of the two diseases

processes independently associated with pulmonary hypertension. A more intriguing possibility is that there is a common >> << process in susceptible individuals, perhaps indirectly through chronic inflammation induced by cigarette smoke, leading to vascular reconstruction >> << in addition to fibrosis and emphysema. While animal data suggest a general relationship, at least theoretically possible, there is still no human studies to determine whether pulmonary hypertension in KZSS is part of a biological response

or by-product synergy of two different people. At the same time not recognizing emphysema added clinical value for diagnosis of IPF alone? After >> << pulmonary hypertension developed as KZSS and IPF have the same prognosis, and options

drug therapy is unproven. However, in early disease, of course, recognition of emphysema in patients with IPF

- is important to monitor. The prognosis for patients with KZSS closely associated with the development >> << pulmonary hypertension and clinical monitoring should include regular evaluation of pulmonary artery pressure. While

TTE is the most convenient, inaccuracies systolic

P evaluates to echo in patients with progressive lung disease suggests that physicians should have a low threshold for

RHC in cases where the right for, timing and choice of one

compared with double lung transplantation depends on accurate determination of hemodynamics,,,. Also, please note that some of the methods commonly used to monitor patients with IPF may be useful in the management

in KZSS. For example, serial spirometry is commonly used as a measure of disease progression in IPF

however, it was shown that forced vital capacity decreases more slowly in patients with KZSS than in patients with IPF,

, despite similarly poor survival in both groups. Repeated measurements of lung and did not provide an accurate assessment of disease trajectory, and can even imagine

false assurances of disease stability. In such cases, worsening hypoxemia may indicate progression in KZSS

lack of spirometry changes. Because of these difficulties in the clinical picture and monitoring has been suggested that patients with KZSS

should be excluded from future studies IPF. Of course KZSS patients with normal or nearly normal lung function at the beginning and slower average speed reduction

in measured amounts of light can distort the results of studies that use changes in spirometry as a marker of disease. It is also an important factor in future research on the study of prognostic markers in IPF. In addition, although patients with

KZSS can meet all diagnostic criteria for IPF, including the presence of the usual interstitial pneumonia pattern of surgical biopsy

is not known whether KZSS and isolated IPF have a common pathogenetic mechanism. Combination of these patients in prospective studies

therefore may mask observed response to therapy in both groups. In addition, the impact KZSS may have been on

last observation studies and clinical trials should be explored. Our understanding of the combination of fibrosis and pulmonary emphysema is still evolving. Clearly, however, that the availability

emphysema in patients with IPF is essential for survival associated with the development of pulmonary hypertension >> << and important implications for disease surveillance. It is essential that future research

mechanism and management of this syndrome is needed. Many questions remain unanswered, and the pressure builds. .