Evaluation of Macrovascular involvement in Systemic Sclerosis.

Pandiperumal, S (2011) Evaluation of Macrovascular involvement in Systemic Sclerosis. Masters thesis, Madras Medical College, Chennai.


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INTRODUCTION : Systemic sclerosis (SSc) is associated with chronic inflammation, fibrosis and obliterative vasculopathy within the skin and visceral organs. SSc is an autoimmune disorder characterized by widespread vasculopathy. The microvasculature is primarily affected. However, large-vessel disease also occurs in SSc. Accelerated atherosclerosis has been described in some patients with SSc. This occurred despite the absence of classical vascular risk factors. Macrovascular disease was observed in 58% of patients with limited cutaneous SSc (lcSSc). Intermittent claudication, cardiovascular disease (CVD) and cerebrovascular disease were detected in 22%, 15% and 6.5% of SSc patients, respectively. AIMS AND OBJECTIVES : 1. To evaluate macrovascular involvement in SSc with high resolution ultra sonogram (HR-USG) by using the measures of; a. Flow-mediated dilatation (FMD), nitroglycerine mediated dilatation of the brachial artery and b. Carotid intima-media thickness (ccIMT). 2. To find out any correlation between FMD, NMD, ccIMT and SSc disease activity and clinical characteristics. MATERIALS AND METHODS : Patients attending outpatient department and inpatients of Department of Rheumatology, Rajiv Gandhi Government General Hospital, Chennai were recruited during the period from October 2008 to March 2011. 32 eligible cases older than 16 years of age were enrolled. They fulfilled ACR criteria for SSc and were willing to undergo measurement of FMD, NMD and ccIMT. Controls were the healthy relatives of the cases who were age and sex matched. All subjects gave written informed consent to take part in this study, which was approved by the ethical committee. Inclusion Criteria: ACR Systemic Sclerosis: Preliminary Classification Criteria; Major criteria or two minor criteria for diagnosis, Major criterion: Proximal Scleroderma, Minor Criteria: Sclerodactyly, Digital pitting scars or loss of substance from finger pad, o Bibasilar pulmonary fibrosis. Exclusion Criteria: 1. Overlap syndrome, 2. Mixed Connective Tissue Disease (MCTD ), 3. Unclassified Connective Tissue Disease (UCTD), 4. Malignancy, 5. Systemic hypertension, 6. Diabetes mellitus, 7. Smoking, 8. Alcoholism. RESULTS : Total number of cases were 32. The mean age was 36 years. The range was from 19-60 years. Majority of them were females – 24(75%). Female: Male ratio is 3:1. Limited cutaneous disease was observed in 26 (81%). Diffuse cutaneous disease was observed in 6 (19%). Raynaud’s Phenomenon was observed in 21 (91%). Digital pitting scar was observed in 17 (53%). Finger contractures were noted in 11 (34%). Poly Arthralgia was noted in 9 (28%). Digital tip ulcerations was noted in 6 (19%). Pulmonary involvement was noted in 23 (72%). ILD was seen in 23 (72%), ILD with traction bronchiectasis was seen in 2 (6%), ILD with emphysematous changes was seen in 2 (6%) and ILD with pulmonary hypertension was seen in 2 (6%). Cardiac involvement was noted in 6 (19%). ECG changes (ST segment/ T wave changes, ventricular premature beats and intraventricular conduction disturbances) was seen in 4 (12%.5), decreased LVEF by Echo was seen in 4 (12.5%). Gastro Intestinal involvement was noted in 14 (44%), Esophageal dysmotility was seen in 14 (44%), Small bowel dysmotility was seen in 2 (6%). Carpal tunnel syndrome was observed in 3 (9%). Sensory neuropathy was noted in 3 (9%), Vasculitic Neuropathy was seen in 1(3%). Osteonecrosis of both tibiae and calcaneum was noted in 1 (3%). Non nephrotic macro-albuminuria was observed in 1 (3%). The duration of disease varied from 6 months to 17 years . Mean duration of disease was 3.96 years. 28 cases (87.5%) were ANA positive. 4 cases (12.5%) were ANA negative. 15 cases were on pulse cyclophosphamide, 5 were on azathioprine and 2 each were on azathioprine and methotrexate. CONCLUSION : 1. Macrovascular involvement is also seen in SSc patients even in the absence of traditional cardiovascular risk factors. 2. There is an impairment of endothelium dependent vasodilatation indicated by low FMD (8.13%) in SSc patients. 3. The endothelium independent vasodilatation assessed by NMD is still preserved providing an opportunity of nitroglycerine therapy for favourably modifying the course of macrovascular pathology. 4. Carotid atherosclerosis as measured by ccIMT is higher in SSc patients (0.67mm) than in controls (0.57mm). 5. ccIMT has significant correlation (moderate positive) with age, in controls and in SSc patients. 6. There is no moderate or strong correlation between FMD, NMD, ccIMT and SSc disease activity and clinical characteristics. 7. FMD using vascular ultrasonography on brachial artery represents a simple, inexpensive, non-invasive and reproducible tool for the assessment of endothelial function.

Item Type: Thesis (Masters)
Uncontrolled Keywords: Macrovascular Involvement ; Systemic Sclerosis.
Subjects: MEDICAL > Rheumatology
Depositing User: Subramani R
Date Deposited: 16 Aug 2017 00:46
Last Modified: 16 Aug 2017 06:15
URI: http://repository-tnmgrmu.ac.in/id/eprint/1816

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