Pulmonary Arterial Hypertension Associated With Connective Tissue Disease and Immunosuppressive Therapy

Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine.
Circulation Journal (Impact Factor: 3.94). 10/2011; 75(11):2543-4. DOI: 10.1253/circj.CJ-11-1037
Source: PubMed
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    ABSTRACT: Gerekçe: Bağ dokusu hastaliklariyla ilişkili pulmoner arteriyel hiper- tansiyon (BDH-PAH) tarihsel olarak kötü bir prognoza sahiptir; sis- temik skleroza bağli pulmoner arteriyel hipertansiyonu (SSc-PAH) olan hastalarin %45'inde sağkalim orani 1 yildir. Ancak, artik daha fazla tedavi olanaklari gündeme gelmektedir. Amaç: U.K. pulmoner hipertansiyon servisinde BDH-PAH tanisi al- miş bütün hastalarin sağkalimlarini ve özelliklerini araştirmak. Yöntem: Ocak 2001 ve Haziran 2006 arasinda ardişik olarak BAH- PAH tanisi almiş olan bütün insidan olgularin ulusal sisteme kay- dedilmesi. Ölçümler ve Ana Sonuçlar: BDH-PAH'i olan hastalara (429; %73'ü SSc-PAH) kateter yöntemi ile tani kondu. İzole SSc-PAH hastalarin- da bir yillik ve üç yillik sağkalim oranlari %78 ve %47 idi. Solunum sistemi hastaliği ile ilişkili SSc-PAH hastasi olanlarda sağkalim daha kötü iken (3 yillik sağkalim %28; p=0,005) egzersizin tetiklediği SSc- PAH hastalari arasinda sağkalim çok iyiydi (3 yillik sağkalim %86; p=
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    ABSTRACT: Interstitial lung diseases (ILDs) are a distinct type of chronic respiratory disorder that can result in pulmonary hypertension. There are numerous causes of ILD but all are characterized by dyspnea and abnormal lung function, with arterial oxygen desaturation occurring as the disease advances. Patients with idiopathic pulmonary fibrosis (IPF), a relentlessly progressive form of ILD, are particularly likely to develop pulmonary hypertension. Both chronic hypoxemia with subsequent pulmonary vasoconstriction and obliteration of the pulmonary vascular bed as a result of interstitial fibrosis have traditionally been considered the pathways by which pulmonary hypertension develops in ILD. Not all patients with pulmonary hypertension from ILD have a severe restrictive ventilatory deficit or pulmonary fibrosis, however, suggesting that other pathologic mechanisms may contribute to the development of pulmonary vascular disease and cor pulmonale in patients with ILD. Sarcoidosis, pulmonary Langerhans cell histiocytosis (PLCH), and lymphangioleiomyomatosis (LAM) are types of ILD that may directly involve the pulmonary vasculature, in addition to causing interstitial fibrosis. Collagen vascular diseases such as scleroderma may cause pulmonary hypertension as a result of progressive ILD or by direct pulmonary vascular involvement. In patients with chronic lung disease, worsening dyspnea and hypoxemia are often mistaken for progressive ILD rather than recognized as heralding the onset of pulmonary hypertension. Although physical findings, echocardiography results, and serum brain natriuretic peptide (BNP) levels can suggest the presence of pulmonary hypertension, right-sided heart catheterization remains the diagnostic gold standard. In this chapter, we review the current classification schemes of both pulmonary hypertension and ILD. The epidemiology of pulmonary hypertension in specific types of ILD is discussed. New information about the origin, pathogenesis, and pathologic findings in ILD-related pulmonary hypertension is described. Clinical manifestations and means of diagnosis are reviewed. We end the chapter with a focus on current treatment options and prognosis.

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