Pul HTN in CTD - Connective tissue disease related...

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Connective tissue disease related Pulmonary arterial hypertension DM Seminar: Dr.Vamsi Krishna
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PAH in connective tissue diseases CTD accounts for 30% of PAH according to US PAH registry. Incidence is on rising trend (40% of all cases after 2002, as compared to 28% before 2002) Mean age is 55+ 15 yrs Females accounted for 87% of cases More severe disease with a lower exercise capacity, a worse functional class, a higher mean pulmonary artery pressure and pulmonary vascular resistance, and lower cardiac index, compared with IPAH A UNITED STATES-BASED REGISTRY FOR PULMONARY ARTERIAL HYPERTENSION: 1982-2006 ERJ Express. Published on September 5, 2007
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PAH in connective tissue diseases High incidence of PAH: Systemic sclerosis Diffuse SSc Limited SSc •S L E Mixed connective tissue disease Case reports and series: Sjogren’s disease Rheumatoid arthritis •A P L A Polymyositis / dermatomyositis
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Systemic sclerosis Pulmonary hypertension may be due to 1. Pulmonary arteriopathy as seen in PPH 2. A consequence of interstitial lung disease 3. Left ventricular diastolic dysfunction 4. Chronic thromboembolic disease The incidence of pulmonary hypertension varies between 6% to 60% of patients with Systemic sclerosis But recent studies with Right heart catheterization showed prevalence of around 15% Ann Rheum Dis. 2003 Nov;62(11):1088-93
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Systemic sclerosis Prevalence of PAH did not differ in patients with Diffuse SSc or limited SSc ( 17 Vs 23%) patients with and without ILD (22% Vs 19%) J Rheumatol. 2007 May;34(5):1005-11 A 52% increase in risk of PAH was demonstrated for every 10 years of age at disease onset Twofold greater risk of PAH (OR, 2.30; CI :1.32 - 3.99) for late-onset (age > or =60 years) vs earlier-onset (< 60 years) disease Chest. 2003 Dec;124(6):2098-104
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SLE Pulmonary hypertension is found in 5–14% of all patients with SLE Lupus. 2004;13(7):506-9 Lupus 2000;9: 338–342 Rheumatol Int 1999;18: 147–151 Pulmonary circulation may be directly involved in 50% of cases Association with other complications including interstitial lung disease or thromboembolic disease in 50% of cases Lupus. 2000;9(5):338-42 Mortality rate of 25% to 50% at 2 years from diagnosis of PAH J Rheumatol. 1999 Sep;26(9):1923-9
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SLE Presence of Raynauds phenomenon is associated with incidence of PAH. J Rheumatol. 1999 Sep;26(9):1923-9 Lupus. 2007;16(7):505-8 Disease activity, organ involvement and anticardiolipin antibodies were not associated with PAH Lupus. 2004;13(7):506-9 Increased values of VEGF and P-selectin in patients with long standing PH are related to severe endothelial dysfunction . VEGF and P-selectin may constitute new therapeutic targets for PH Eur J Med Res. 2007 Apr 26;12(4):145-51
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Rheumatoid arthritis Incidence of pulmonary artery systolic pressure>30 mm Hg significantly higher in patients with RA (26.7% versus 4.5% in controls; p=0.03) 20% of patients had pulmonary hypertension without lung disease or cardiac disease A strong correlation between the PAP and the disease duration (r=0.68, p<0.000) Int J Cardiol. 2007 Aug 7 20 - 27.5% of RA have mild Pulm HTn Scand J Rheumatol. 2004;33(4):244 -5 Rheumatology (Oxford)2000;39:1320-1325
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This note was uploaded on 12/03/2011 for the course MEDICINE 350 taught by Professor Dr.aslam during the Winter '07 term at Medical College.

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Pul HTN in CTD - Connective tissue disease related...

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