WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension

Abstract

Sarcoidosis-associated pulmonary hypertension (SAPH) is an important complication of advanced sarcoidosis. Over the past few years, there have been several studies dealing with screening, diagnosis and treatment of SAPH. This includes the results of two large SAPH-specific registries. A task force was established by the World Association of Sarcoidosis and Other Granulomatous disease (WASOG) to summarise the current level of knowledge in the area and provide guidance for the management of patients. A group of sarcoidosis and pulmonary hypertension experts participated in this task force. The committee developed a consensus regarding initial screening including who should undergo more specific testing with echocardiogram. Based on the results, the committee agreed upon who should undergo right-heart catheterisation and how to interpret the results. The committee felt there was no specific phenotype of a SAPH patient in whom pulmonary hypertension-specific therapy could be definitively recommended. They recommended that treatment decisions be made jointly with a sarcoidosis and pulmonary hypertension expert. The committee recognised that there were significant defects in the current knowledge regarding SAPH, but felt the statement would be useful in directing future studies.

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…The reported prevalence of PH in patients with sarcoidosis is 6-20% [824]. The causes are multifactorial, including fibrosing lung disease, granulomata in the PAs and/or pulmonary veins, fibrosing mediastinitis and/or extrinsic compression by lymph nodes, pulmonary vasculitis, CTEPH, and PoPH [58,825].…”

Comorbidities of sarcoidosis

“…The exact prevalence of sarcoidosis associated pulmonary hypertension (SAPH) remains uncertain due to significant variability in diagnostic methods and criteria used as well as the populations studied. Overall, PH affects 1–6% of sarcoidosis patients [ 8 ], but it is much more frequent in advanced lung disease, reaching 73.8% of cases listed for lung transplantation [ 9 ]. In the study by Huitema et al., which has prospectively screened 399 consecutive patients from a Dutch cohort using transthoracic echocardiography (TTE) and confirmatory right heart catheterisation (RHC) in those with suspected PH, the prevalence of hemodynamically confirmed SAPH was 2.9% [ 10 ].…”

“…These include specific pulmonary vasculopathy, locally increased vasoreactivity, portal hypertension due to liver involvement, extrinsic vascular compression, and left heart dysfunction. Other lung comorbidities associated with sarcoidosis can also cause PH, including obstructive sleep apnoea (OSA), and thromboembolic disease [ 8 ].…”

“…Proliferative arteriopathy with plexiform lesions, the hallmark of group 1 pulmonary arterial hypertension (PAH), is rare in SAPH. A pulmonary veno-occlusive disease (PVOD)-like phenotype has been occasionally described [ 8 ]. SAPH may be related to extrinsic compression of the proximal PAs or veins by enlarged lymph nodes or fibrosing mediastinitis ( Figure 1 ).…”

“…A statement endorsed by the World Association of Sarcoidosis and Other Granulomatous diseases (WASOG) has recently provided recommendations on SAPH diagnosis. A diagnosis algorithm is proposed in Figure 2 [ 8 ]. Although several clinical, functional and imaging features may raise suspicion of SAPH, there is no consistent single criterion that can adequately distinguish sarcoidosis patients with a high or low risk.…”