Aims: It is increasingly important to determine the economic consequences of diseases considering the policy of limited health–care budgets. In this study we evaluated the annual direct and indirect costs of Systemic Sclerosis (SSc) and we tried also to identify any cost predictors. Methods: We studied 106 patients (103 female, 3 male), 57 affected by Limited Systemic Sclerosis (LSSc) and 49 affected by Diffuse Systemic Sclerosis (DSSc). Mean age was 57 years (SD±13,8) and mean disease duration was 8,9 years (SD±7,2). Direct costs: data were calculated referring to DRG (Disease Related Group) expenses for the in-patients. We referred to national pharmacopoeia to calculate the pharmaceutical cost for the out-patients. Indirect costs: we estimated the expense comparing our cases to literature data. Intangible costs: these are attributable to pain and psychological suffering. It is very difficult to express the intangible costs in monetary terms and they are often conveyed as disability and poorer quality-of-life. We used the Health Assessment Questionnaire “HAQ” and the Short Form-36 “SF-36” to evaluate this issues. Results: Our study confirms, the extremely high costs caused by Systemic Sclerosis (total cost’s 2001 year is € 1.173.842,93, and average yearly patient cost is € 11.073,99). Considering an estimated prevalence of 375 cases/106, the total yearly economic impact of SSc in Italy should be € 249.000.000,00. Intangible costs were calculated as modifications of the health status. Average value of the HAQ was significantly higher than the control population (0,94±0,72), average values in the SF-36 were significantly lower than the control population (49,99±19,16 for physical dimension and 58,42±27,71 for mental dimension). The diffuse form of SSc, positivity for anti-Scl 70 antibodies, high skin score and a poor health status (HAQ and SF-36) were found to be cost predictors. Conclusions: As reported in the literature, our study confirms, the extremely high costs for total and single patients caused by Systemic Sclerosis. The DSSc are more expensive than the LSSc approximately 11% (p=0,0067). The direct costs are 30% higher in the DSSc than the LSSc (p<0.001). The indirect and intangible costs are not significantly different. Moreover, our study shows also the possibility of identifying different cost predictors

Aims: to evaluate the quality of life of patients affected by systemic sclerosis (SSc) through the application of the Medical Outcome Survey Short-Form 36 (SF-36) questionnaire and to correlate the results with the disability index of the health assessment questionnaire (HAQ-DI) and the systemic involvement. Methods: we studied 95 (3 M, 91 F) patients affected by SSc (mean age 60 years, range 39-83, mean duration of disease 6 years, range 1-34). The organ system involvement was evaluated by skin score, chest High Resolution Computed Tomography (HRCT), electrocardiography according to Holter, Doppler-echocardiography and oesofagogram. Results: considering the values of the 8 question groups of the SF-36 the most different between the patients and the control population are the values relevant to the physical dimension. The general health values estimating the physical and social dimension are significantly lower in the patients than in the control population (t=9,324; p<0,0001). A very good correlation was found between the DI (r = -0,7903 ; p <0.0001) and all the scores of SF-36. The skin involvement showed a statistically significant correlation with the DI (r=0.3709; p=0.0002) and the PA score of the SF- 36 (r =0.2853; p=0.0051). No other statistically significant correlation was found between any of the SF-36 dimensions and involvement of a specific organ. Conclusion: SF-36 showed to be a valid instrument to evaluate the quality of life and the disability of patients with SSc and it seems to correlate with extent of skin involvement

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level and severe constipation of the colon, 1,2 even if other segments of GI are known to be affected in subclinical form. Objectives Few studies have assessed the morphological features of the stomach in SSc. A 52 years old female with limited SSc from 1979, suffering from dysphagia, pyrosis, epigastric pain, gastro-esophageal reflux, in 1998, underwent to distal esophagectomy for Barrett?s oesophagus and, in 1999, to total gastrectomy with Roux y esophago-jejunal anastomosis. The stomach appeared very small, shrunken, its wall being stiff and increased in consistence. Samples were taken to study the ultrastructural modifications of the gastric wall and, in particular, the muscle coat. Methods Samples of the gastric anterior wall from fundus, corpus and antrum, along the greater curvature, were obtained. The specimens were fixed in 4% glutaraldehyde in 0,1 M phosphate buffer, postfixed in 1% osmium tetroxide in 0,1M phosphate buffer and routinely processed for transmission electron microscopic observation. The ultrathin sections (1 mm) were stained with uranyl acetate and alkaline bismuth subnitrate and observed under Jeol 1010 electron microscope. Results Wide focal areas of fibrosis, characterised by collagen fibres depositions, surrounding smooth muscle cells (SMC) were found. Many SMC were hypercontracted and/or showed a severe disarrangement of the myofilaments. Often, the dense bodies and the dense bands were thickened. Some SMC showed cytoplasmic vacuolisation and swollen mithocondria. Nerve bundles containing many axons were close to vessels and SMC. The axoplasms of nerve fibres were oedematous and poor in neurotubules and filaments, but the nerve endings close to SMC showed normal morphology, with the presence of synaptic vescicles, whose number was seldom decreased. Blood vessels were lined by intact endothelial cells, while the basement membrane appeared or thickened and laminated. The lumen was partially or completely occluded by erythrocytes and neutrophils. Inflammatory infiltrate as well, and many mast cells rich in granules or partially degranulated, were close to vessels and SMC. Conclusion In the muscle coat of stomach focal fibrosis, severe ultrastructural modification of SCM and mild nerve fibres involvement are evident, without signs of endothelial damage.

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