Pulmonary Vascular Manifestations of Mixed Connective Tissue Disease

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The pulmonary vasculature

Pulmonary circulation is a high-capacitance, low-resistance system able to accommodate significant increases in cardiac output with little increase in pulmonary artery (PA) pressure. Through the recruitment of closed vessels and the distension of previously opened vasculature, pulmonary circulation can accommodate a fourfold increase in flow with relatively small increases in PA pressure. The lung vasculature normally accepts the entire blood output from the right ventricle, and, although the

Pulmonary arterial hypertension

Clinically, pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure of greater than 25 mm Hg at rest and greater than 30 mm Hg with exercise in patients with a normal pulmonary capillary wedge pressure. Patients who have PAH commonly present with the insidious onset of dyspnea on exertion. The diagnosis is often delayed because more common causes of dyspnea are considered first. Fatigue is another common manifestation of PAH, and frequently patients and clinicians

Mixed connective tissue disease and pulmonary arterial hypertension

Severe pulmonary hypertension (Fig. 2) is a potentially devastating manifestation of MCTD, associated with high morbidity and mortality. Esther and colleagues [27] performed a prospective evaluation of pulmonary function in 26 patients who had MCTD. Twenty of the 26 patients had a decreased diffusing capacity on pulmonary function tests. Nine of the patients underwent right heart catheterization, and seven were diagnosed with PAH. Weiner-Kronish and colleagues [28] reported on a series of five

Treatment of pulmonary arterial hypertension associated with connective tissue disease

There have been no trials specifically addressing the therapy of MCTD-associated PAH. A number of studies investigating the treatment of pulmonary hypertension have included patients who had PAH associated with MCTD or the “scleroderma spectrum of disease.” Therapy for PAH includes anticoagulation, oxygen, diuretics, and pulmonary vascular vasodilators. We restrict our discussion to the use of FDA-approved medications for the treatment of PAH with a brief discussion of potential future

Other pulmonary manifestations of mixed connective tissue disease

Pleuropulmonary manifestations of MCTD are more common than previously recognized. The initial description of MCTD did not discuss pulmonary involvement of this disease. Subsequent publications have estimated that 20% to 80% of patients have respiratory involvement [1], [62], [63], [64]. The potential respiratory manifestations of MCTD are listed in Box 1. Three of these respiratory diseases—interstitial disease, pleural effusions, and pulmonary hemorrhage—are discussed below.

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