Sarcoidosis-associated pulmonary hypertension: Clinical features and outcomes in Arab patients

Abstract

Background: Pulmonary hypertension (PH) occurs in many patients with interstitial lung disease, including sarcoidosis. We explored the frequency, clinical characteristics and outcomes of PH in Arab patients diagnosed with pulmonary sarcoidosis.

Methods: A retrospective study in three tertiary hospitals was performed on 96 patients who underwent Doppler echocardiography. Demographic and clinical characteristics, physiological studies and computed tomography (CT) results were collected, and compared between patients with and without PH.

Results: Twenty (20.8%) patients were found to have PH. Patients with PH were more likely to be symptomatic (cough, P = 0.008; dyspnea, P = 0.04), to have an advanced radiographic stage (P = 0.001), and to be receiving systemic therapy (P = 0.001), compared to those without PH. Physiological data including pulmonary function test parameters, arterial blood gas levels and oxygen saturation at rest and after exercise were all significantly lower in patients with PH compared to those without PH. Comparison of CT patterns between patients with and without PH showed significant differences in the frequencies of ground-glass opacity (61.5 vs. 28.8%, P = 0.032) and fibrosis (76.9 vs. 44.2%, P = 0.035). In total, four patients died during the study period, including three with evidence of PH.

Conclusions: The frequency of PH in the present study was 20.8%. Clinical, physiologic and radiographic characteristics appeared to differentiate patients with PH from those without PH. The presence of PH contributed to poor outcomes in patients with pulmonary sarcoidosis.

Keywords: Computed tomography; echocardiography; pulmonary function test; pulmonary hypertension; sarcoidosis.

Figure 1

Patient 2: (a) HRCT scan showing widespread nodules with patchy areas of ground glass attenuation. (b) HRCT scan obtained at the 5-year follow-up showing regression of ground glass attenuation with treatment. Clinical characteristics of the patient are shown in Table 4

Figure 2

Patient 3. HRCT scan obtained at the level of tracheal bifurcation demonstrating fibronodular pattern with traction bronchiectasis. Mediastinal lymph nodes enlargement are seen. Clinical characteristics of the patient are shown in Table 4

Figure 3

Patient 4. HRCT scan at the mid lung zones showing conglomeration pattern with background diffuse hyperinflation. Clinical characteristics of the patient are shown in Table 4

Figure 4

Patient 5. HRCT scan at the level of the left atrium demonstrating diffuse ground glass attenuation with fine reticulation and cystic changes. Lymph node enlargement involving the azygoesophageal recess is seen. Clinical characteristics of the patient are shown in Table 4