Introduction
Epidemiology
Prognosis
Classification of PH in sarcoidosis
WHO group 1 | Pulmonary arterial hypertension | |
WHO group 2 | Pulmonary hypertension due to left heart disease | |
WHO group 3 | Pulmonary hypertension due to lung disease and/or hypoxaemia | |
WHO group 4 | Chronic thromboembolic pulmonary hypertension or other pulmonary artery obstructions | |
WHO group 5 | Pulmonary hypertension with unclear multifactorial mechanism | |
5.1 | Hematologic disorders: myeloproliferative disorders, splenectomy, chronic haemolytic disorders | |
5.2 | Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis lymphangioleiomyomatisos | |
5.3 | Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders | |
5.4 | Others: pulmonary tumoral thrombotic microangiopathy, fibrosing mediastinitis, chronic renal failure, segmental pulmonary hypertension |
Aetiology of PH in sarcoidosis
Pre-capillary PH
Destruction of distal capillary bed and resultant hypoxaemia
Specific vasculopathy
Local increased vasoreactivity
Extrinsic compression of pulmonary vessels
Portal hypertension
Pulmonary veno-occlusive disease
Post-capillary PH
Diagnosis
Suspicion of PH
Pulmonary function test
Exercise testing
Chest computed tomography
Cardiac magnetic resonance imaging
Specific PH analysis
Biomarkers
Electrocardiogram
Echocardiography
Echocardiographic pulmonary hypertension probability | Peak tricuspid regurgitation velocity (m/s) | Other signsa for pulmonary hypertension on echocardiography |
---|---|---|
Low | ≤2.8 or not measurable | No |
Intermediate | ≤2.8 or not measurable | Yes |
2.9–3.4 | No | |
High | 2.9–3.4 | Yes |
>3.4 | Not required |
The ventricles | Right ventricle/left ventricle basal diameter ratio >1.0 | Flattening of the interventricular septum (left ventricular eccentricity index >1.1 in systole and/or diastole) | |
Pulmonary artery | Pulmonary arterial acceleration time <105 msec and/or midsystolic notching | Early diastolic pulmonary regurgitation velocity >2.2 m/sec | PA diameter >25 mm |
Inferior vena cava and right atrium | Inferior cava diameter >21 mm with decreased inspiratory collapse (<50 % with a sniff or <20 % with quiet inspiration) | Right atrial area (end-systole >18 cm2) |
Right heart catheterisation
Recommendations for clinical practice
Management
Sarcoidosis-targeted treatment
Anti-inflammatory and immunomodulatory agents
Oxygen therapy
PH-targeted therapy
Author | Year and type | Treatment |
N
| mPAP | PVR | CO | Other outcomes and adverse events |
---|---|---|---|---|---|---|---|
Prostacyclin analogues
| |||||||
Preston [15] | 2001 CS | Nitric oxide | 8 | ↓ 18 % | ↓ 31 % | ↑ 12 % | |
Preston [15] | 2001 CS | Epoprostenol | 6 | ↔ | ↓ 25 % | ↑ 25 % | |
Fisher [16] | 2006 CS | Epoprostenol | 7 | ↓ 21 % | ↓ 45 % | ↑ 44 % | ↑ NYHA 1–2 classes Decreased Pao2 in 3/7 One death |
Baughman [45] | 2009 CS | Iloprost | 15 | ↓ 15 % | ↓ 45 % | – | ↑ 12 % 6MWT ↑ QOL Decrease in Pao2 in 2/15 |
Endothelin receptor antagonists
| |||||||
Judson [46] | 2011 CS | Ambrisentan | 21 | – | – | – | ↔ 6MWT, NYHA, QOL 38 % discontinued due to ↑ oedema and dyspnoea |
Baughman [26] | 2014 RCT | Bosentan | 35 | ↓ 11 % | ↓ 28 % | – | ↔ 6MWT |
Phosphodiesterase 5 inhibitors
| |||||||
Milman [9] | 2008 CS | Sildenafil | 12 | ↓ 19 % | ↓ 48 % | ↑ 36 % | ↔ 6MWT |
Combined studies
| |||||||
Barnett [25] | 2009 CS | Sildenafil, bosentan, epoprostenol | 22 | ↓ 20 % | ↓ 39 % | ↔ | ↑ 6MWT |
Dobarro [47] | 2013 CS | Bosentan (2) or sildenafil (9) | 11 | ↔ | ↔ | ↔ | ↑ 6MWT ↔ NYHA |