Version June 2024
| Category | Risk factor | Comments |
| Symptoms and signs | Unexplained dyspnea | Cough and wheezing not typically associated with SAPH Many of these manifestations overlap with pulmonary parenchymal and cardiac sarcoidosis Symptoms such as weight gain with fluid retention that may be consistent with right heart failure may be due to corticosteroid therapy |
| Dyspnea that is disproportionately severe in comparison to other imaging, spirometry, and lung volume assessment | ||
| Dyspnea that is persistent or severe despite anti-inflammatory therapy | ||
| Dyspnea that is new in onset | ||
| Dyspnea associated with worsening functional class | ||
| Exertional chest pain, syncope, or other manifestations of right-sided heart failure | ||
| PFT | DLCO is disproportionately lower than that expected for the degree of lung function | While PFT can be supportive, they have poor predictive capacity for SAPH; near-normal lung function does not exclude SAPH |
| DLCO is <50% predicted in the absence of pulmonary fibrosis on imaging | ||
| DLCO is <30% with evidence of pulmonary fibrosis (ie, stage 4 disease) | ||
| Decrease in DLCO by 15% without change in lung volumes | ||
| Chest imaging | Pulmonary fibrosis (ie, stage 4 disease) | Although severe fibrosis increases the risk of SAPH, some patients have SAPH in the absence of significant underlying lung disease |
| Either fibrosis or mediastinal adenopathy (calcified or noncalcified) that results in compression of pulmonary vessels | ||
| Enlarged pulmonary artery diameter (corrected to body surface area) or ratio of pulmonary artery to ascending aortic diameter >1 | ||
| Enlarged right ventricle | ||
| Oxygenation parameters | Hypoxemia at rest or with ambulation <90% | These features are not specific and can be affected by a variety of factors including musculoskeletal disease, cardiac dysfunction, parenchymal lung disease, depression, and fatigue |
| 6MWD <350 meters | ||
| Laboratory | Elevated BNP or N-terminal pro-BNP | These tests may also indicate cardiac sarcoidosis |
| Electrocardiography | P-pulmonale, right axis deviation, right ventricular hypertrophy or strain, and right bundle branch block | While these findings can be observed in patients with cardiac sarcoidosis, they are more likely to indicate SAPH |
6MWD: 6-minute walk distance; BNP: B-type natriuretic peptide; DLCO: diffusion capacity for carbon monoxide; PFT: pulmonary function testing; PH: pulmonary hypertension; SAPH: sarcoidosis-associated pulmonary hypertension; TTE: transthoracic echocardiography.
* One or more of these features should raise the suspicion for SAPH and prompt evaluation with TTE.آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟
