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Background: Pulmonary hypertension (PH) is a common complication of interstitial lung disease (ILD) and is associated with worse outcomes and increased mortality. Evaluation of PH is recommended in lung transplant candidates, but there are currently no standardized screening approaches. Trials have identified therapies that are effective in this setting, providing another rationale to routinely screen patients with ILD for PH.Research Question: What screening strategies for identifying PH in patients with ILD are supported by expert consensus?Study Design and Methods: The study convened a panel of 16 pulmonologists with expertise in PH and ILD, and used a modified Delphi consensus process with three surveys to identify PH screening strategies. Survey 1 consisted primarily of open-ended questions. Surveys 2 and 3 were developed from responses to survey 1 and contained statements about PH screening that panelists rated from -5 (strongly disagree) to 5 (strongly agree).Results: Panelists reached consensus on several triggers for suspicion of PH including the following: symptoms, clinical signs, findings on chest CT scan or other imaging, abnormalities in pulse oximetry, elevations in brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP), and unexplained worsening in pulmonary function tests or 6-min walk distance. Echocardiography and BNP/NT-proBNP were identified as screening tools for PH. Right heart catheterization was deemed essential for confirming PH.Interpretation: Many patients with ILD may benefit from early evaluation of PH now that an approved therapy is available. Protocols to evaluate patients with ILD often overlap with evaluations for pulmonary hypertension-interstitial lung disease and can be used to assess the risk of PH. Because standardized approaches are lacking, this consensus statement is intended to aid physicians in the identification of patients with ILD and possible PH, and provide guidance for timely right heart catheterization. [ABSTRACT FROM AUTHOR]

Background: Surgical lung biopsy (SLB) is invasive and not possible in all patients with undiagnosed interstitial lung disease (ILD). We hypothesized that transbronchial biopsy (TBB) findings combined with clinical and high-resolution CT (HRCT) data leads to a confident diagnosis congruent to SLB and therefore avoids the need for SLB in some patients.Methods: We evaluated 33 patients being investigated for suspected ILD who underwent HRCT, TBB, and SLB. First, clinicians, radiologists, and a pathologist reviewed the clinical information and HRCT and TBB findings. Clinicians were asked to provide a diagnosis and were also asked if SLB was needed for a more confident diagnosis. Subsequently, the clinical, HRCT, and SLB data were reviewed, and the same participants were asked to provide a final diagnosis. Clinician consensus and overall agreement between TBB- and SLB-based diagnoses were calculated.Results: Four patients had definite usual interstitial pneumonia (UIP) on HRCT and would not be considered for biopsy using current guidelines. Of the 29 patients without a definitive HRCT diagnosis, the clinicians felt confident of the diagnosis (ie, would not recommend SLB) in six cases. In these cases, there was 100% agreement between TBB and SLB diagnoses. UIP was the most common diagnosis (n = 3) and was associated with an HRCT diagnosis of possible UIP/nonspecific interstitial pneumonia-like. Agreement was poor (33%) between TBB and SLB diagnoses when confidence in the TBB diagnosis was low.Conclusions: Information from TBB, when combined with clinical and HRCT data, may provide enough information to make a confident and accurate diagnosis in approximately 20% to 30% of patients with ILD. [ABSTRACT FROM AUTHOR]

The article reports on a study performed to confirm a technique developed to detect pulmonary hypertension in patients with idiopathic pulmonary fibrosis. The formula is used to forecast the mean pulmonary artery pressure (MPAP) from standard measurements of lung function. Results validated the prediction equation.

The article examines whether diagnostic changes of usual interstitial pneumonia (UIP) can be identified on transbronchial biopsy (TBB) specimens in the United States. The study examines 21 patients with UIP confirmed by surgical lung biopsy and/or lung explant and one patient with UIP confirmed by clinical and radiographic findings. Characteristic histologic features include adequacy of tissue for diagnosis, varying combinations of interstitial fibrosis in a patchwork pattern along with fibroblast foci and/or honeycomb change. The authors concluded that the features can be appreciated on TBB specimens.

A response by David A. Zisman and Anna-Luise A. Katzenstein to a letter to the editor about their article "Transbronchial Biopsy in Usual Interstitial Pneumonia" in the May 2006 issue is presented.

A response by D.A. Zisman and colleagues to a letter to the editor about their article High-resolution chest CT findings do not predict the presence of pulmonary hypertension in advanced idiopathic pulmonary fibrosis in issue 132 is presented.

A response by David A. Zisman to a letter to the editor about the article "Transbronchial biopsy in usual interstitial pneumonia" is presented.