Chest
The Lesser Circulation in Pulmonary Fibrosis Secondary to Sarcoidosis and Its Relationship to Respiratory Function
Section snippets
METHODS
The data of all patients who had been seen in this laboratory for the past ten years were reviewed. All patients with pulmonary sarcoidosis who had pulmonary function studies as well as cardiac catheterization were selected. Twenty three such patients were found. Of these, eight patients had data which was either not complete or had other complicating diseases and therefore were discarded. Fifteen patients remained and form the basis of this report. Of these cases the diagnosis of sarcoid was
RESULTS
The results of pulmonary function studies and cardiac catheterization are listed in Table 1 and Table 2, respectively.
PULMONARY FUNCTION
There was wide variation in the severity of respiratory function abnormalities. Some patients were within the normal range for some ventilatory functions. In others, there was significant deterioration in all parameters. The VC was normal in two patients (cases 1, 9) and only moderately impaired for the group. No patient fell within the normal range for DCO, the highest being 61 percent of predicted (case 1). For the group the DCO was severely reduced, averaging 8.7 ml/min/mm Hg, 33 percent of
CARDIAC
Only two patients (cases 1, 3) had normal pulmonary artery pressure (Pa mean ≤ 15 mm Hg) and normal TPR (< 3 units). On 11 of the remaining 13 patients either a normal wedge or left ventricular end-diastolic pressure (≤ 12 mm Hg) was measured and an elevated PVR (> 1.5 units) was calculated.
With one exception (case 1), in none of the nine patients who were exercised did TPR and PVR fall as would be expected in normal subjects.12 Oxygen consumption at rest was within the limits of normal for all
RELATION OF PULMONARY AND CARDIAC FUNCTION
These relationships are shown in FIGURE 3, FIGURE 4, FIGURE 5, FIGURE 6 and the multiple correlations are listed in Table 3. The PCO2, pH and FEV1 did not correlate with any measured parameters of pulmonary circulation. The O2 saturation showed correlations with only resting PAM and TPR and these correlations were poor (Fig 3).
The DCO correlated well with findings in the pulmonary circulation (Fig 4). There was a tendency for the DCO to have a curvilinear relation to PVR and TPR and the use of
DISCUSSION
One of the striking findings in this study, in contradistinction to patients with COPD, is the poor correlation with blood gases (1, 9, 13–15). The O2 saturation showed only a weak relationship with resting PA pressure and total pulmonary resistance (Fig 3).
Of additional interest regarding the blood gases were the findings here which are contrary to the generally held view that oxygen saturation is well maintained until late in the course of the disease. If “late” implies the development of
ACKNOWLEDGMENT
We acknowledge our indebtedness to P. Baskette, C. Doyle, A. Varble, J. Waldie and B. Weinheimer for their valuable assistance.
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Pulmonary Hypertension Associated With Sarcoidosis
2018, Sarcoidosis: A Clinician's GuideOpposite behavior of plasma levels surfactant protein type B and receptor for advanced glycation end products in pulmonary sarcoidosis
2013, Respiratory MedicineCitation Excerpt :Similarly, in the present study, a higher plasma concentration of SP-B was detected in the sarcoidosis patients with the worst DLCO, suggesting the hypothesis that lung parenchymal and vascular destructive granulomatous lesions may result in an augmented SP-B leakage from the alveoli into the bloodstream. Indeed, DLCO is strongly related to damages of alveolar-capillary unit, such as a decrease in capillary volume, or an increase in barrier thickness and/or in the ventilation-perfusion mismatch [2,4–7]. Accordingly, VE/VCO2 slope and ΔSaO2 values were significantly increased in the patients with the most impaired lung function, and they significantly correlated with circulating plasma levels of SP-B [14,29,33].
NT-proAtrial Natriuretic Peptide as a possible biomarker of cardiopulmonary involvement in sarcoidosis
2013, European Journal of Internal MedicineCitation Excerpt :Accordingly, perivascular fibrosis or granulomatous pulmonary angiitis has been previously reported in several pathological surveys in these patients [3,4]. Supporting our hypothesis, a significant correlation between DLCO and pulmonary vascular resistance has been widely reported [3,4,7] and a previous study from Nunes and colleagues showed that reduced DLCO values were closely associated to PAH development in patients with sarcoidosis regardless of the presence of pulmonary fibrosis [24]. In the same study, Authors disclosed a close relationship between some HRCT signs, such as ground glass attenuation and septal lines, with pulmonary venous occlusive phenomena in native lungs from transplanted patients [24].
Pulmonary hypertension complicating sarcoidosis
2012, Presse MedicaleCitation Excerpt :However, 31.8 to 50% of patients with sarcoidosis-associated PH develop this complication in the absence of patent pulmonary fibrosis [11,13,14] and a small subset of cases have apparently no underlying lung disease (radiographic stage 0 and I) [6,11,13–15]. Moreover, hemodynamic measurements do not correlate well with spirometric parameters and PaO2 [6,10,13,16] and mPAP is 9 mmHg higher in sarcoidosis than in idiopathic pulmonary fibrosis (IPF) for equivalent respiratory impairment (34.4 versus 25.6 mmHg, P < 0.0001) [17]. Last, the degree of PH is sometimes disproportionate to functional abnormalities [9,11,13,18] and PH may even be more severe when it occurs in patients without fibrotic disease [13].
Pulmonary Hypertension Caused by Sarcoidosis
2008, Clinics in Chest MedicineHilar adenopathy compressing the pulmonary arteries in the course of sarcoidosis
2007, Revue des Maladies Respiratoires
This work was supported by a grant from the Westchester Heart Association.