Factors influencing early referral, early diagnosis and management in patients with diffuse cutaneous systemic sclerosis

Abstract

ObjectiveTo gain insight into clinical practice regarding referral, early diagnosis and other aspects of the management of patients with dcSSc in Europe and the USA. MethodsSemi-structured interviews were conducted with 84 rheumatologists (or internal medicine physicians) and 40 dermatologists in different countries (the UK, France, Germany, Italy, Spain and the USA). Physicians were asked to identify key steps in the patient pathway relating to patient presentation, diagnosis and referral, in addition to other treatment and follow-up processes.ResultsThe interviewed physicians reported that late presentation with dcSSc was common, with some patients presenting to primary care physicians after symptoms had persisted for up to 1 year. Awareness of dcSSc is reported to vary widely among primary care physicians. Final diagnosis, generally following guideline-based recommendations, was by rheumatologists in most cases (or internal medicine physicians in France) and they remained responsible for global patient management, with lesser involvement in diagnosis and management by dermatologists. Specialist centres were not well defined and did not exist in all countries.ConclusionPatients and primary healthcare providers can be unaware of the symptoms of dcSSc, therefore presentation and referral to specialist care are often late. Thus, improved awareness among patients and primary care physicians is necessary to facilitate earlier referral and diagnosis. Once referred, more consistent use of the modified Rodnan skin score at diagnosis and follow-up may help to monitor disease progression. Furthermore, establishing specialist centres may help to promote such changes and improve patient care.

Trends in Demographics, Hospitalization Outcomes, Comorbidities, and Mortality Risk among Systemic Sclerosis Patients

“…There was a decreasing trend in the number of admissions for SScl from 2010 to 2014, which may be due to advances in the treatment of the disease with the use of immunosuppressive agents and anti-fibrotic tyrosine kinase inhibitors to help control the pulmonary complications of the disease [ 14 ]. This may also be due to greater recognition of the disease by primary care physicians and quicker referral of the patients to rheumatologists who are then able to make the correct diagnosis and institute better management and follow up on an outpatient basis, thereby preventing unnecessary complications requiring admission [ 15 ].…”

“…This may be due to the socioeconomic and health insurance status of the patients. The decreasing trend seen in patients less than 60 years of age may be because of a greater awareness in people of that age group, or a high index of suspicion amongst primary care physicians who are quickly able to recognize the symptoms [ 15 ]. The increasing trend in admissions of patients between ages 61 and 80 can be credited to a lower index of suspicion in these patients and the presence of other comorbidities that may mask the symptoms or to which the symptoms may be attributed.…”

Predictors of progression in systemic sclerosis patients with interstitial lung disease

“…There are many challenges associated with the diagnosis of SSc-ILD. One potential barrier to diagnosis is a lack of awareness within primary care of SSc, which can lead to late referrals [42]. There is also variability in the degree of involvement of clinical specialties in establishing a diagnosis of SSc-ILD, and a lack of formally designated SSc specialist centres [42].…”

“…There is a need for early systematic screening of patients to identify pulmonary complications, though there is a lack of consensus regarding which tests should be used during screening and follow-up [2,42]. In one study, screening for ILD at diagnosis of SSc using HRCT identified 35% of subjects with no ILD, 51% of subjects with ILD of limited extent (1-20% fibrosis), and 13% of subjects with ILD involving >20% of the lungs [43].…”

Current and future perspectives on management of systemic sclerosis-associated interstitial lung disease

“…There is substantial room for improvement in the screening, diagnosis, and management of SSc-ILD. Delayed diagnosis of SSc often leads to delayed referral to specialty care, preventing patients from receiving the monitoring and management needed to optimize outcomes [82]. A lack of consensus guidelines for screening or early diagnosis of ILD in patients with SSc results in discrepancies in diagnosis and to delayed and missed diagnosis of SSc-ILD in clinical practice.…”