Clinical predictors of pulmonary hypertension in sarcoidosis

Abstract

Pulmonary hypertension (PH) adversely affects outcome in sarcoidosis and is an important predictor of mortality in these patients. Early and accurate diagnosis of this complication may improve outcome. The current authors hypothesised that integration of 6-min walk test (6MWT) as part of the evaluation leads to an earlier diagnosis of this complication.A total of 162 patients with sarcoidosis underwent 6MWT. Demographic and pulmonary function results were analysed. Patients were further assessed by echocardiography and right heart catheterisation when clinically indicated.Patients with sarcoidosis-associated PH had significantly decreased results on pulmonary function testing. They also walked shorter distances and desaturated to lower levels on 6MWT. On logistic regression analyses, significant predictors of PH were oxygen saturation ,90% on 6MWT (odds ratio (OR) 12.1, 95% confidence interval (CI) 3.66-19.73) and diffusing capacity of the lung for carbon monoxide ,60% predicted (OR 7.3, 95% CI 1.98-24.82). Moreover, by combining the results of oxygen saturation at 6 min with those of echocardiography, the ability to correctly predict the presence of PH by right heart catheterisation was improved.Patients with diffusing capacity of the lung for carbon monoxide ,60% predicted and oxygen desaturation ,90% on 6-min walk test have a high likelihood of pulmonary hypertension and should undergo further evaluation for the presence of this disorder.

Treatment of sarcoidosis-associated pulmonary hypertension: so close, and yet so far

“…While there is increasing evidence that pulmonary hypertension directed therapy improves haemodynamics, the data on change in 6MWD is less clear. Baseline reduced 6MWD has been repeatedly reported in patients with S-APH [5,19]. In the current French study, the 6MWD at presentation was independently associated with mortality.…”

Sarcoidosis Associated Pulmonary Hypertension

“…Bourbonnais & Samavati, 2008;Handa et al, 2006;Sulica et al, 2005) A very common finding in patients with PH is the presence of a disproportionate decrease in the diffusing capacity of the lung for carbon monoxide to the degree of pulmonary impairment and restriction, similar to PAH associated with scleroderma.…”

“…Six minute walk distance is reduced in the majority of patients with sarcoidosis, and this reduction is even more pronounced in patients with SAPH, when compared with sarcoidosis patients without PH. , Bourbonnais & Samavati, 2008 In a prospective study of 142 patients with sarcoidosis, the median 6MWD walked by SAPH patients was 280 m, which was significantly lower compared to the median distance of 411 m, walked by the patients without documented PH. In addition, compared to patients without PH, during a 6MWT, SAPH patients demonstrate more significant degree of oxygen desaturation, increased Borg Dyspnea Score and a lower distance saturation product (DSP, defined as the product between the distance walked and the lowest oxygen saturation achieved during the 6MWT).…”

“…(Sulica et al, 2005) Hypoxemia and decreased functional status, measured by the 6-minute-walk distance, exercise desaturation and decreased distance-saturation product (DSP), tend to be more pronounced in patients with SAPH compared to sarcoidosis patients without PH. (Alhamad et al, 2010;Baughman et al, 2007;Bourbonnais & Samavati, 2008) …”

Tadalafil Therapy for Sarcoidosis‐Associated Pulmonary Hypertension

“…The most commonly affected organ system is the lung, most often in the form of alveolitis and/or parenchymal fibrosis and associated sequelae (such as bronchiectasis and cavity formation). Sarcoidosis-associated pulmonary hypertension (SAPH) is seen in at least 5% of patients with sarcoidosis 1 and more often in patients with advanced, end-stage disease. This higher frequency of SAPH is noted particularly in patients who are being evaluated for or awaiting lung transplantation, and increased mortality is noted in this population as well.…”