Quarterly medical reviewPulmonary hypertension complicating sarcoidosis
Section snippets
Classification of pulmonary hypertension and concept of “out of proportion” pulmonary hypertension
PH is defined by an increased mean pulmonary arterial pressure (mPAP) greater or equal to 25 mmHg at rest on right heart catheterisation (RHC). In an attempt to assist physicians on their clinical practice, an updated clinical classification of PH derived from the Dana Point meeting has been published in 2009 (box 1) [2]. Group 3 refers to “PH due to lung diseases and/or hypoxia”, including interstitial lung diseases (ILDs). Group 5 has been created for “PH with unclear and/or multifactorial
Frequency of sarcoidosis-associated PH
The exact prevalence of PH complicating sarcoidosis remains to establish. The wide distribution in published rates is most likely due to the use of different measurement techniques, selection of diverse patient populations or various stages of disease (table I). Overall, PH affects 1% to 6% patients with sarcoidosis [4], [5], [6], [7], [8] but it is much more frequent in advanced lung disease [9] or in symptomatic patients [10].
The only available prospective study conducted by Handa et al. has
Pathogenesis of sarcoidosis-associated PH
As already indicated above, sarcoidosis is classified in the group 5 of PH classification but essentially, the mechanisms of sarcoidosis-associated PH may fit into all five categories [2], [3].
Diagnosis of sarcoidosis-associated PH
The clinical picture of underlying respiratory disorder can mask PH and delay its recognition. Several symptoms should, yet, prompt diagnostic intervention: dyspnoea more severe than one would expect from functional impairment, chest pain, palpitations, near syncope on exertion. Physical signs include a loud P2 component to the second heart sound, a fixed, split S2, a holosystolic murmur of tricuspid regurgitation (TR) and a diastolic murmur of pulmonic regurgitation. About one quarter of
Screening of sarcoidosis-associated PH
As PH bears a severe prognosis in sarcoidosis, early diagnosis and consideration of treatment options may be keys to improve patients’ outcome. Nonetheless, there is no consistent single clinical criteria that can be used to adequately segregate sarcoidosis patients with a high or low risk for PH. Owing the limited accuracy of TTE, this should not serve as the only guide to determine who requires further invasive intervention. In light of recent data, RHC seems particularly important in
Clinical impact and prognosis of sarcoidosis-associated PH
PH is a debilitating condition in sarcoidosis patients, which accounts for refractory dyspnoea [10] and reduced exercise capacity [12], [59]. Additionally, the burden of PH on functional status and employment status is substantial [9]. Transplantation candidates with PH are more likely to need some or total assistance with their activities of daily living (nearly 70% of those with mPAP ≥ 40 mmHg) and PH increases the risk of being unemployed due to disease [9]. Furthermore, PH is well known to
Treatment of sarcoidosis-associated PH
In view of limited publications, no recommendation can be drawn on the optimal therapeutic strategy for sarcoidosis-associated PH, which should take into account the prominent underlying mechanism of PH. Obviously, supportive therapy is the cornerstone of management, including supplemental oxygen in patients who are hypoxemic at rest or during exercise and diuretics as needed.
With respect to therapy directed to sarcoidosis, published results are somewhat discrepant. PH can worsen despite
Conclusion
PH is a challenging complication of sarcoidosis both in terms of diagnosis and management. There are recent shreds of evidence suggesting that sarcoidosis-associated PH may be more complex than just the result of parenchymal lung disease and hypoxemia. Intrinsic vasculopathy may play an important role. The presence of PH signifies a grave prognosis in patients with sarcoidosis. PAH-targeted therapy is tempting in sarcoidosis-associated PH but the lack of conclusive data leaves many
Disclosure of interest
the authors declare that they have no conflicts of interest concerning this article.
Glossary
- CCB
- calcium channel blockers
- ILDs
- interstitial lung diseases
- IPF
- idiopathic pulmonary fibrosis
- PAH
- pulmonary arterial hypertension
- PH
- pulmonary hypertension
- PVOD
- pulmonary veno-occlusive disease
- PWP
- pulmonary wedge pressure
- RHC
- right heart catheterisation
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Cited by (28)
Sarcoidosis
2021, Encyclopedia of Respiratory Medicine, Second EditionThe long-term prognostic significance of sarcoidosis-associated pulmonary hypertension – A cohort study
2019, Clinical ImmunologyCitation Excerpt :Our study included all existing measures as a possible indication for PH diagnosis. Nunes et al. [13] reported that PH affects 1% to 6% of patients with sarcoidosis. It was further shown that pulmonary arterial mean pressure was over 35 mmHg in half of the patients with sarcoidosis [16].
Sarcoidosis Lung
2018, Revue du Rhumatisme MonographiesPulmonary hypertension in patients with interstitial lung disease
2018, Pulmonary Pharmacology and TherapeuticsCitation Excerpt :Extrapulmonary manifestations and multifactorial mechanisms of PH development include: i) capillaries destruction due to chronic hypoxaemia ii) compression of pulmonary arteries by lymph nodes or fibrosis; iii) vasoactive factors leading to pulmonary vasoconstriction, iv) specific granulomatous vasculitis and v) complete loss of capillaries and arterioles due to parenchymal destruction [170]. Interestingly, PH in sarcoidosis is not associated with the degree of pulmonary fibrosis and functional impairment [170,171]. With regards to treatment, current evidence is limited to treatment of the underlying disease.
Lung sarcoidosis: Clinical features and therapeutic issues
2016, Revue de Medecine InterneSarcoidosis-associated pulmonary hypertension
2016, Revue des Maladies Respiratoires Actualites