TITLE

Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis

AUTHOR(S)
Nunes, H.; Humbert, M.; Capron, F.; Brauner, M.; Sitbon, O.; Battesti, J-P.; Simonneau, G.; Valeyre, D.
PUB. DATE
January 2006
SOURCE
Thorax;Jan2006, Vol. 61 Issue 1, p68
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Background: Pulmonary hypertension (PH) is a rare complication of sarcoidosis, although it is not uncommon in advanced disease. Methods: A retrospective series of 22 sarcoidosis patients (16 men) of mean (SD) age 46 (13) years with PH was divided into two groups depending on the absence (stage 0: n = 2, stage II: n =4, stage III: n = 1) or presence (n = 15) of radiographic pulmonary fibrosis at the time of PH diagnosis. Results: In both groups PH was moderate to severe and there was no response to acute vasodilator challenge. In non-fibrotic cases no other cause of PH was found, suggesting a specific sarcoidosis vasculopathy, although no histological specimens were available. In cases with fibrosis there was no correlation between haemodynamics and lung volumes or arterial oxygen tensions, suggesting other mechanisms for PH in addition to pulmonary destruction and hypoxaemia. These included extrinsic arterial compression by lymphadenopathies in three cases and histologically proven pulmonary veno-occlusive disease in the five patients who underwent lung transplantation. Ten patients received high doses of oral prednisone for PH (stage 0: n = 1, stage II: n = 4 and stage IV: n = 5); three patients without pulmonary fibrosis experienced a sustained haemodynamic response. Survival of the overall population was poor (59% at 5 years). Mortality was associated with NYHA functional class IV but not with haemodynamic parameters or with lung function. Conclusion: Two very different phenotypes of sarcoidosis combined with PH are observed depending on the presence or absence of pulmonary fibrosis. PH is a severe complication of sarcoidosis.
ACCESSION #
20089487

 

Related Articles

  • Different KCO and VA combinations exist for the same DLCO value in patients with diffuse parenchymal lung diseases. Pastre, Jean; Plantier, Laurent; Planes, Carole; Borie, Raphaël; Nunes, Hilario; Delclaux, Christophe; Israël-Biet, Dominique // BMC Pulmonary Medicine;9/3/2015, Vol. 15 Issue 1, p1 

    Background: DLCO is the product of the CO transfer coefficient (KCO) by the "accessible" alveolar volume (VA). In theory, the same DLCO may result from various combinations of KCO and VA values, each of which reflect different injury sites and mechanisms. We sought to determine in this study the...

  • Endobronsiyal Kitle Lezyonu Yapan Sarkoidoz. AKPINAR, Serdar; U�AR, Nazire; SERIFOGLU, Irem; AKTAS, Zafer; SIPIT, Tugrul // Akciger Arsivi/Archives of Lung;2010, Vol. 11 Issue 2, p77 

    Sarcoidosis is a multisystemic disorder with unknown etiology which affect mostly lungs and mediastinal lymph nodes. Parenchymal lung disease is common in sarcoidosis, but airways also may be affected. Endobronchial mass lesion is very rare in clinical course of the disease. A female patient...

  • Discriminative Immunophenotype of Bronchoalveolar Lavage CD4 Lymphocytes in Sarcoidosis. Kolopp-Sarda, Marie-Nathalie; Kohier, Chantal; De March, Anne Kennel; Béné, Marie-Christine; Faure, Gilbert // Laboratory Investigation (00236837);Jul2000, Vol. 80 Issue 7, p1065 

    The diagnosis of pulmonary sarcoidosis relies in part on the observation of alveolar CD4+ lymphocytosis. However, this criterion is not fully discriminative because this anomaly is also found in other types of lung diseases. Among other possible distinctive criteria, we investigated the...

  • High-resolution computed tomography to differentiate chronic diffuse interstitial lung diseases with predominant ground-glass pattern using logical analysis of data. Martin, Sophie Grivaud; Kronek, Louis-Philippe; Valeyre, Dominique; Brauner, Nadia; Brillet, Pierre-Yves; Nunes, Hilario; Brauner, Michel W.; Réty, Frédérique // European Radiology;Jun2010, Vol. 20 Issue 6, p1297 

    We evaluated the performance of high-resolution computed tomography (HRCT) to differentiate chronic diffuse interstitial lung diseases (CDILD) with predominant ground-glass pattern by using logical analysis of data (LAD). A total of 162 patients were classified into seven categories: sarcoidosis...

  • Th1/Th2 cytokine pattern in bronchoalveolar lavage fluid and induced sputum in pulmonary sarcoidosis. Tsiligianni, Ioanna; Antoniou, Katerina M.; Kyriakou, Despina; Tzanakis, Nikolaos; Chrysofakis, George; Siafakas, Nikolaos M.; Bouros, Demosthenes // BMC Pulmonary Medicine;2005, Vol. 5, p8 

    Background: Sarcoidosis is thought to be a T-helper type 1 cytokine (Th2 cytokine) mediated disorder. Induced sputum (IS) has been proposed as a useful non-invasive method, mainly for the assessment of the airway diseases. The aim of this study was to explore induced sputum (IS) CD4+Th1...

  • Cervikalna limfadenopatija -- jedina klinička manifestacija sarkoidoze? Čeleketić, Dušica; Nadaškić, Ružica; Krotin, Mirjana; Čemerikić, Vesna; Stojković, Aleksandra; Trpković, Andreja; Vuković, Vladimir // Vojnosanitetski Pregled: Military Medical & Pharmaceutical Journ;Mar2006, Vol. 63 Issue 3, p309 

    Background. Sarcoidosis is a chronic inflammatory disease, commonly found in lungs and hilar lymph nodes, but multiple organs could be involved. The diagnosis is based on specific pathohistology which should be always combined with clinical, radiological and laboratory findings. Case report. A...

  • Exhaled nitric oxide in sarcoidosis. Wilsher, M. I.; Fergusson, W.; Mime, D.; Wells, A. U.; Wilsher, M L; Milne, D // Thorax;Nov2005, Vol. 60 Issue 11, p967 

    Background: Increased production of nitric oxide (NO) by the lower respiratory tract is viewed as a marker of airway inflammation in asthma and bronchiectasis. NO is a potentially important immune modulator, inhibiting the release of several key pro-inflammatory cytokines. As...

  • Ocular and renal sarcoidosis. S Shah; Cole, M. D.; A Nicholls // Journal of the Royal Society of Medicine;Oct1995, Vol. 88 Issue 10, p597P 

    Sarcoidosis commonly presents in young adults with bilateral hilar lymphadenopathy, lung parenchymal disease and/or skin lesions. Ocular symptoms are the presenting feature in up to 10% of cases, but eye involvement can be demonstrated in around a quarter of all patients. Renal disease is much...

  • Apoptosis resistant bronchoalveolar lavage (BAL) Fluid lymphocytes in sarcoidosis. Stridh, H.; Planck, A.; Gigliotti, D.; Eklund, A.; Grunewald, J. // Thorax;Oct2002, Vol. 57 Issue 10, p897 

    Background: Sarcoidosis is a chronic granulomatous lung disease of unknown origin. The accumulation of activated I cells at sites of inflammation represents an early stage in granuloma formation. Since mechanisms governing the normal resolution of inflammatory processes are poorly understood,...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sign out of this library

Other Topics