The Real Face of Borderline Pulmonary Hypertension in Connective Tissue Disease (original) (raw)
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Pathology of Pulmonary Hypertension
Clinics in Chest Medicine, 2013
The focus on the pathological changes underlying pulmonary hypertension (PH) have dominated the early investigations of this disease first described late in the 19 th century. Pulmonary vascular pathology continues to play an important role in the present age of cell and molecular investigation of the pathogenesis of PH. This importance stems from the permanent quest to correlate pulmonary vascular remodeling with the altered pulmonary vascular hemodynamics, a critical advancement in the late 40's and early 50's with a wide impact on our present understanding of the disease. However, as it happens to most descriptive tools applied to medical sciences, the pathological insight into the extent and type of a particular form of pulmonary vascular remodeling has fallen short of establishing cause and effect relationships in the natural history of PH, and has had a limited impact on diagnosis and therapy. These limitations derive largely from the reliance of our current knowledge on studies of autopsies, as lung tissue is rarely available for histopathology during the course of the disease.
Connective Tissue Disease Associated Pulmonary Hypertension
Contemporary Cardiology™, 2008
Although rare in its idiopathic form, pulmonary arterial hypertension (PAH) is not uncommon in association with various associated medical conditions, most notably connective tissue disease (CTD). In particular, it develops in approximately 10% of patients with systemic sclerosis and so these patients are increasingly screened to enable early detection. The response of patients with systemic sclerosis to PAHspecific therapy appears to be worse than in other forms of PAH. Survival in systemic sclerosis-associated PAH is inferior to that observed in idiopathic PAH. Potential reasons for this include differences in age, the nature of the underlying pulmonary vasculopathy and the ability of the right ventricle to cope with increased afterload between patients with systemic sclerosis-associated PAH and idiopathic PAH, while coexisting cardiac and pulmonary disease is common in systemic sclerosis-associated PAH. Other forms of connective tissue-associated PAH have been less well studied, however PAH associated with systemic lupus erythematosus (SLE) has a better prognosis than systemic sclerosis-associated PAH and likely responds to immunosuppression.
Pictorial review of the pulmonary vasculature: from arteries to veins
Insights into Imaging, 2018
Pathology of the pulmonary vasculature involves an impressive array of both congenital and acquired conditions. While some of these disorders are benign, disruption of the pulmonary vasculature is often incompatible with life, making these conditions critical to identify on imaging. Many reviews of pulmonary vascular pathology approach the pulmonary arteries, pulmonary veins and bronchial arteries as individual topics. The goal of this review is to provide an integrated overview of the high-yield features of all major disorders of the pulmonary vasculature. This approach provides a more cohesive and comprehensive conceptualisation of respiratory pathology. In this review, we present both the salient clinical and imaging features of congenital and acquired disorders of the pulmonary vasculature, to assist the radiologist in identifying pathology and forming a robust differential diagnosis tailored to the presenting patient. Teaching Points • Abnormalities of the pulmonary vasculature are both congenital and acquired. • Pathology of a single pulmonary vascular territory often affects the entire pulmonary vasculature. • Anomalous pulmonary venous flow is named as a function of its location and severity. • Bronchial arteries often undergo dilatation secondary to cardio-respiratory pathology.
Pulmonary arterial hypertension
2004
ulmonary hypertension is usually classified as primary (idiopathic) or secondary. 1 It is now clear, however, that there are conditions within the category of secondary pulmonary hypertension that resemble primary pulmonary hypertension in their histopathological features and their response to treatment. For this reason, the World Health Organization (WHO) classified pulmonary hypertension into five groups on the basis of mechanisms, rather than associated conditions ). The most recent revision of the WHO classification uses consistent terminology and defines pulmonary hypertension more precisely than previous versions. 2 Group I of the WHO classification, designated pulmonary arterial hypertension, is the principal focus of this review.
Journal of postgraduate medicine
Mixed connective tissue disease (MCTD) has features common to lupus, scleroderma and myositis with high levels of antibodies to U1 ribonucleoprotein (U1 RNP). Identification of a high incidence of pulmonary artery hypertension (PAH) has changed its prospect. We report the largest series from India. Rheumatology unit of a tertiary care centre in India; prospective. Patients seen between January 2002 and June 2004, satisfying the Kasukawa criteria were enrolled. All patients had a complete laboratory work-up including pulmonary function test, 2-D echocardiography, and Schirmer's test, antinuclear antibodies (ANA) and antibodies to extractable nuclear antigens. HRCT of chest was done where indicated. All patients were given standard treatment and followed up regularly. Out of 1500 patients, thirteen (one male) were diagnosed to have MCTD. The median follow-up period was 18 months [Interquartile range (IQR) 12-22]. The median age of onset of symptoms was 36 years (IQR 22-39) and the...