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N- TERMINAL PRO- BRAIN NATRIURETIC PEPTIDE IN SYSTEMIC SCLEROSIS: NEW INSIGHTS.

N- TERMINAL PRO- BRAIN NATRIURETIC PEPTIDE IN SYSTEMIC SCLEROSIS: NEW INSIGHTS. Dr Soha Eldessouki Ibrahim Lecturer of Rheumatology and Rehabilitation. Ain Shams University. . 2011.

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N- TERMINAL PRO- BRAIN NATRIURETIC PEPTIDE IN SYSTEMIC SCLEROSIS: NEW INSIGHTS.

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  1. N- TERMINAL PRO- BRAIN NATRIURETIC PEPTIDE IN SYSTEMIC SCLEROSIS: NEW INSIGHTS. Dr SohaEldessouki Ibrahim Lecturer of Rheumatology and Rehabilitation. Ain Shams University. 2011

  2. Systemic scleosis (SSc) is a connective tissue disease characterized by predominant T-cell activation, auto antibodies and cytokines release 1-Diffuse micro-vascular injury. 2- Fibroblasts activation . 3-Increased production of collagen. Diffuse vascular damage and sclerosis within the skin and internal organs are due to progressive and wide spread tissue fibrosis. Zhou etal.,2005

  3. PAH is widely recognized to be a major complication of both limited and diffuse cutaneous subtypes of SSc. Dimitroulas etal.,2010 It is the most lethal form of pulmonary vascular disease and is characterized by fibrosis, inflammation and vasculopathy. Mac Gregor etal.,2001

  4. Human brain natriuretic peptide (BNP) is produced and secreted by ventricular myocardium under conditions of sustained volume or pressure overload The BNP is cleaved upon secretion to BNP levels are high in patients with CHF, cardiac infarctionand PAH. NT- pro BNP Mac Gregor etal.,2001

  5. Aim of the work To identify the relation between serum levels of NT- pro BNP and severity of SScas assessed by Extent of skin fibrosis Severity of PAH Degree of pulmonary involvement.

  6. Study group included 25 SSc patients .SSc was diagnosed according to the criteria of American College of Rheumatology1980 and was classified as limited or diffuse cutaneous types according to the criteria of Le Roy et al,1988. Control group included 25 healthy volunteers matching in age and sex.

  7. Clinical skin assessment Theseverity and the extent of skin sclerosis were assessed using modified Rodnan skin score (mRSS) where cutaneous thickness was assessed in 17 body surface areas using 0-3 scale with maximum score of 51. • Rating scale consisted of: 0=normal, 1=thickened, 2= thickened unable to pinch, 3=thickened unable to move. Clements etal., 1995

  8. Assay for serum concentration was done by (ELISA)  NT-pro BNP

  9. Systolic pulmonary artery pressure (sPAP) was measured by echocardiography. PAH was defined as sPAP>40 mm Hg. Choi etal.,2008

  10. Pulmonary function tests (PFTs): The restrictive pattern of pulmonary function was based on: 1- decreased percentage of FVC (%FVC < 80%) 2- decreased percentage of DLCO (%DLCO< 75%) of the predicted values.

  11. Histological skin thickness score The presence and distribution of sclerosis was assessed within the various layers of the dermis: papillary dermis (PD), superficial reticular dermis (SRD), median reticular dermis (MRD) and deep reticular dermis (DRD). • Within each of these four dermal layers, the extent of fibrous thickening was assessed as follows: 0=no fibrosis, 1=light fibrosis, 2 =moderate fibrosis, 3=extensive fibrosis. Verrecchia etal.,2007

  12. This study included 25 SScpatients, 21 females (84%) and 4 males (16%). Their ages ranged from 22-68 years with a mean of 46±10.9 years. The disease duration ranged from 5-11 years with a mean of 9.6±2.9 years.

  13. Comparison between limited and diffuse SSc patients regarding different clinical and laboratory data.

  14. There was a highly significant increase in the mean levels of serum levels of NT- pro BNP in SSc patients compared to controls (400.4±126 fmol/ml vs 103.5±7.1 fmol/ml as t=11 , p< 0.001).

  15. Correlation between NT- pro BNP and modified Rodnann skin score in SSc patients (r =0.93).

  16. Comparison between serum levels of NT- pro BNP and sPAP in SScpatients with restrictive pulmonary involvement and those without.

  17. According to the histological skin thickness scoring system Seven patients with grade 3 Seven patients with grade 2 Five patients with grade 1 Six patients with grade 0

  18. Correlation between NT-proBNP and histological skin thickness score in SSc patients (r=0.92).

  19. Skin specimen of SSc patient showing extensive fibrosis Skin specimen of SSc patient showing epidermal thinning, loss of rete ridges, extensive fibrosis and dense collagen deposition in DRD ( black arrows) with virtual absence of appendages plus moderate fibrosis in the MRD and in the SRD as well as in the PD . Note the presence of foci of peri-vascular inflammatory infiltrate ( black stars) . (H&E ×100)

  20. Conclusion NT-pro BNP may be a useful biological marker for assessing the severity of SScas it has a role in detecting the extent of skin fibrosis, the severity of PAH and the degree of restricted pulmonary involvement in SSc patients.

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