PURPOSE: The relation between sarcoidosis and pulmonary hypertension (PH) is actually an emergent topic in clinical assessment of sarcoidosis. PH prevalence in sarcoidosis is reported to be <5%.
METHODS: A sarcoidosis case series of 25 patients was observed in a single General Hospital from 1996 to 2005: 7 females and 18 males (mean age 43.1 ± 7.6 years, range 30-58)histologic confirmed. At diagnosis the patients underwent chest X-Ray, octreotide scintigraphy (Octreoscan) with lung uptake index (UI; normal value ≤ 10), and evaluation of systolic pulmonary artery pressure (sPAP) by echocardiography (normal value ≤ 35 mmHg), irrespective of clinical cardiovascular problems. None of patients had Scadding stage IV chest radiographic pulmonary disease. Patients were distributed as follows: stage 0: n=2; stage I: n=14; stage II: n=5; stage III: n=4. 11 (44%) had sPAP above the normal upper limit (mean 44.5 ± 7.9 mmHg, range 36-68). sPAP and radiological stage were highly correlated (stage 0: 2 cases with normal sPAP; stage I: 12 cases with normal sPAP and two cases with sPAP 36 and 38 mmHg, respectively; stage II: mean sPAP 40.8, range 38-42; stage III: mean sPAP 52.8, range 40-68).
RESULTS: When the Scadding stage was compared with sPAP classified as normal/elevate, the correlation was statistically significant (χ2 p-value <0.01). Among PH patients, Octreoscan UI (mean 15.0 ± 3.1, range 12.5-22.3) showed a strong positive correlation with sPAP (R2: 0.87; p<0.01). (Figure) After a median follow up of 6.5 years (range 0.59 - 8.8), survival rate at 5 years was 96% for the whole series, and 91% for the PH patients.
CONCLUSION: We conclude that: i) in a non-selected series of patients with sarcoidosis PH (>35 mmHg, according to accepted cut-off limits) is common, ii) low levels of PH are not associated with poor prognosis, iii) sPAP levels significantly increased with X-Ray stage, iv) sPAP levels were significantly correlated with Octreoscan UI since initial sPAP rise.
CLINICAL IMPLICATIONS: Octreoscan UI is a sensitive technique which may be useful in further clinical investigation.
DISCLOSURE: Roberto Carbone, None.