Cost-of-illness in systemic sclerosis: a retrospective study of an italian cohort of 106 patients

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

Evaluation of quality of life in patients with systemic sclerosis by the SF-36 questionnaire

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

Developing a rare disease chronic care model: Management of systemic sclerosis (MANOSS) study protocol

Aim The aim of the management of systemic sclerosis (MANOSS) study described in this protocol is to develop a chronic care model, based on a contextual analysis and stakeholder involvement, for patients living with the rare disease systemic sclerosis (SSc) in Switzerland. Design Applying an implementation science approach, this study starts with an explanatory sequential mixed method study for contextual analysis, followed by broad stakeholder involvement for model development and a Delphi study to reach consensus. Methods First, a quantitative cross-sectional survey with patients and healthcare professionals (HPs) will be conducted to identify current practice patterns of chronic illness management and technology readiness. Second, qualitative interviews with patients, family members and HPs will be performed to gain a deeper understanding of care needs identified in the quantitative survey. Third, a model of care will be co-created with input from patients, HPs and other experts. The eHealth enhanced Chronic Care Model will serve as a guiding framework. The new model and corresponding outcome parameters will be refined using a Delphi-study approach to reach consensus on a testable model of care for persons living with SSc. The protocol has received research ethics committee approval in September 2018 by the Swiss Ethics Committee. Discussion The MANOSS study's participatory approach is essential for contextual fit of the model for patients with SSc in this setting. Subsequent feasibility testing and implementation are planned to evaluate the model's value in relation to health disparities faced by this patient population. Impact Patients living with this rare disease lack access to coordinated, specialized care and self-management support from qualified HPs. Reengineering of current care, with consideration for technological opportunities, is warranted to meet patients' and families' needs

Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). RESULTS: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). CONCLUSIONS: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

79To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD).nonenoneGonzález-Gay, Miguel A; Montecucco, Carlomaurizio; Selva-O'Callaghan, Albert; Trallero-Araguas, Ernesto; Molberg, Ovynd; Andersson, Helena; Rojas-Serrano, Jorge; Perez-Roman, Diana Isabel; Bauhammer, Jutta; Fiehn, Christoph; Neri, Rossella; Barsotti, Simone; Lorenz, Hannes M; Doria, Andrea; Ghirardello, Anna; Iannone, Florenzo; Giannini, Margherita; Franceschini, Franco; Cavazzana, Ilaria; Triantafyllias, Konstantinos; Benucci, Maurizio; Infantino, Maria; Manfredi, Mariangela; Conti, Fabrizio; Schwarting, Andreas; Sebastiani, Giandomenico; Iuliano, Annamaria; Emmi, Giacomo; Silvestri, Elena; Govoni, Marcello; Scirè, Carlo Alberto; Furini, Federica; Lopez-Longo, Francisco Javier; Martínez-Barrio, Julia; Sebastiani, Marco; Manfredi, Andreina; Bachiller-Corral, Javier; Sifuentes Giraldo, Walter Alberto; Cimmino, Marco A; Cosso, Claudio; Belotti Masserini, Alessandro; Cagnotto, Giovanni; Codullo, Veronica; Romano, Mariaeva; Paolazzi, Giuseppe; Pellerito, Raffaele; Saketkoo, Lesley Ann; Ortego-Centeno, Norberto; Quartuccio, Luca; Batticciotto, Alberto; Bartoloni Bocci, Elena; Gerli, Roberto; Specker, Christof; Bravi, Elena; Selmi, Carlo; Parisi, Simone; Salaffi, Fausto; Meloni, Federica; Marchioni, Enrico; Pesci, Alberto; Dei, Giulia; Confalonieri, Marco; Tomietto, Paola; Nuno, Laura; Bonella, Francesco; Pipitone, Nicolò; Mera-Valera, Antonio; Perez-Gomez, Nair; Gerzeli, Simone; Lopez-Mejias, Raquel; Matos-Costa, Carlo Jorge; Pereira da Silva, Jose Antonio; Cifrian, José; Alpini, Claudia; Olivieri, Ignazio; Blázquez Cañamero, María Ángeles; Rodriguez Cambrón, Ana Belén; Castañeda, Santos; Cavagna, LorenzoGonzález-Gay, Miguel A; Montecucco, Carlomaurizio; Selva-O'Callaghan, Albert; Trallero-Araguas, Ernesto; Molberg, Ovynd; Andersson, Helena; Rojas-Serrano, Jorge; Perez-Roman, Diana Isabel; Bauhammer, Jutta; Fiehn, Christoph; Neri, Rossella; Barsotti, Simone; Lorenz, Hannes M; Doria, Andrea; Ghirardello, Anna; Iannone, Florenzo; Giannini, Margherita; Franceschini, Franco; Cavazzana, Ilaria; Triantafyllias, Konstantinos; Benucci, Maurizio; Infantino, Maria; Manfredi, Mariangela; Conti, Fabrizio; Schwarting, Andreas; Sebastiani, Giandomenico; Iuliano, Annamaria; Emmi, Giacomo; Silvestri, Elena; Govoni, Marcello; Scirè, Carlo Alberto; Furini, Federica; Lopez-Longo, Francisco Javier; Martínez-Barrio, Julia; Sebastiani, Marco; Manfredi, Andreina; Bachiller-Corral, Javier; Sifuentes Giraldo, Walter Alberto; Cimmino, Marco A; Cosso, Claudio; Belotti Masserini, Alessandro; Cagnotto, Giovanni; Codullo, Veronica; Romano, Mariaeva; Paolazzi, Giuseppe; Pellerito, Raffaele; Saketkoo, Lesley Ann; Ortego-Centeno, Norberto; Quartuccio, Luca; Batticciotto, Alberto; Bartoloni Bocci, Elena; Gerli, Roberto; Specker, Christof; Bravi, Elena; Selmi, Carlo; Parisi, Simone; Salaffi, Fausto; Meloni, Federica; Marchioni, Enrico; Pesci, Alberto; Dei, Giulia; Confalonieri, Marco; Tomietto, Paola; Nuno, Laura; Bonella, Francesco; Pipitone, Nicolò; Mera-Valera, Antonio; Perez-Gomez, Nair; Gerzeli, Simone; Lopez-Mejias, Raquel; Matos-Costa, Carlo Jorge; Pereira da Silva, Jose Antonio; Cifrian, José; Alpini, Claudia; Olivieri, Ignazio; Blázquez Cañamero, María Ángeles; Rodriguez Cambrón, Ana Belén; Castañeda, Santos; Cavagna, Lorenz

Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). RESULTS: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). CONCLUSIONS: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

Timing of onset affects arthritis presentation pattern in antisynthetase syndrome

Objective To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). Methods The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). Results 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). Conclusion In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility