Pulmonary Vascular Manifestations of Mixed Connective Tissue Disease
Section snippets
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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58 - Pulmonary Involvement in the Systemic Inflammatory Diseases of Childhood
2019, Kendig's Disorders of the Respiratory Tract in ChildrenFacts and controversies in mixed connective tissue disease
2018, Medicina ClinicaOverlap Syndromes
2016, Kelley and Firestein's Textbook of Rheumatology: Volumes 1-2, Tenth EditionPulmonary manifestations of Sjögren syndrome, systemic lupus erythematosus, and mixed connective tissue disease
2015, Rheumatic Disease Clinics of North AmericaCitation Excerpt :Treatment of PAH in MCTD is similar to other CTDs such as scleroderma.81 Serositis in MCTD is common, with an estimated incidence varying from 6% to 50%, but pleuritic pain is only occasionally the presenting symptom; effusions are typically exudates and may be self-limiting and transient.81 Pulmonary manifestations in SS, SLE, and MCTD have tremendous clinical relevance, and a significant effect on prognosis, worsening the morbidity, mortality, and functional capacity of these patients.
Histopathology of lung disease in the connective tissue diseases
2015, Rheumatic Disease Clinics of North AmericaCitation Excerpt :Organizing pneumonia and diffuse alveolar damage have been occasionally described in MCTD patients and have been suggested to represent acute exacerbations of underlying pulmonary disease.49,62 Although pulmonary fibrosis with radiologic features of both NSIP and UIP has been described, histologic descriptions of chronic ILD in MCTD are scarce, limited to case reports or small series, and usually lack definitive histologic classification.62–64 Pulmonary vascular disease is a prominent and often predominant feature of MCTD-associated lung involvement, correlating with the clinical manifestations of severe and sometimes fatal pulmonary hypertension.
Mixed Connective Tissue Disease and Undifferentiated Connective Tissue Disease
2015, Textbook of Pediatric Rheumatology