Expert opinion and patients' in-depth interviews on the impact of pulmonary complications in systemic sclerosis.

OBJECTIVE: To qualitatively explore the perceptions and opinions of experts dealing with systemic sclerosis (SSc) and patients with SSc on the impact of the disease and pulmonary complications on economic status, psycho-social wellbeing and the diagnostic and therapeutic journey, and to identify which strategies/interventions may be useful to address patients' and their family's needs. METHODS: An expert meeting was conducted using the NGT to discuss the consequences of pulmonary complications on the Italian SSc community. The direct experience of five patients with SSc and pulmonary complications was described through in-depth interviews conducted by psychologists. RESULTS: The experts' meeting and patients' in-depth interviews underline the complexity of SSc and the consequences of pulmonary involvement on patients' and caregivers' health-related quality of life, working ability, psychological wellbeing and social interactions. Panellists suggest that improved communication between physicians, associations and institutions could help protect the working status of patients with SSc. Granting patients disability benefits, providing access to part-time jobs and productivity-focused training could also help decrease the economic burden of the disease. A multidisciplinary approach is recommended to reduce treatment burden, together with the implementation of standard diagnostic and therapeutic paths and increased use of telemedicine via platforms that ensure secure health data sharing. Both patients and caregivers may benefit from psychological support. CONCLUSION: SSc and pulmonary fibrosis have profound consequences on patients' and caregivers' health-related quality of life, working ability, psychological wellbeing and social interactions. Some activities may help patients and families deal with these aspects of the disease.

How do systemic sclerosis manifestations influence patients' lives? Results from a survey on patients and caregivers.

OBJECTIVE: To investigate the patient- and caregiver-reported impact of systemic sclerosis (SSc) manifestations (hand/feet/joint involvement and pulmonary complications) on the diagnostic and therapeutic journey, working productivity, and social life. METHODS: Two questionnaires (one for the patients, n = 260 and one for the caregivers, n = 47) were designed in collaboration with the patients' association Gruppo Italiano per la Lotta alla Sclerodermia (GILS). Validated questionnaires were combined with specific questions relevant to the Italian scenario. RESULTS: Pulmonary fibrosis and hand/feet/joint involvement have a major impact on patient's working status: (85.3% of patients with pulmonary fibrosis and 72.6% with hand/feet/joint involvement report loss of job/job change due to SSc. Productivity was affected as well: 60.6% of the patients (75% of those with fibrosis) reported that working productivity in the previous 4 weeks was restricted by physical limitations. The disease has a significant impact on patients' life, limiting the ability to conduct common activities, especially those related to movement, such as object manipulation (61.1%), doing small manual jobs (44.0%), writing (38.9%), and an increased impact in case of pulmonary fibrosis and hands/feet/joints involvement. Half of the patients also present some difficulties in eating-related activities a Patients also experience poorer social life, personal relationships, and sexual life. Caregivers are also deeply influenced by the manifestations of SSc. Pulmonary fibrosis and hand/feet/joint involvement represent an additional challenge. CONCLUSION: Pulmonary fibrosis and hand/feet/joint involvement are extremely burdensome complications for both SSc patients and caregivers, decreasing work productivity, limiting relationship and social life, and impacting psychological status and everyday activities.