Causes and predictors of death among Finnish patients with systemic sclerosis.

OBJECTIVE: The aim of this study was to assess causes and predictors of death among Finnish patients with systemic sclerosis (SSc). METHOD: Medical records of patients registered with the ICD-10 code M34 from 1996 to 2018 in two university hospitals were reviewed retrospectively. Clinical data were collected until the end of 2020. Death certificates were obtained from Statistics Finland up to August 2021. Using death certificates and patient records, the cause of death for each patient was determined. The mean age at death, median time from SSc diagnosis, and factors predicting death were analysed. RESULTS: Among 313 SSc patients, 91 deaths occurred between April 2000 and September 2020. Overall 5 and 10 year survival rates were 88.4% and 80.2%, respectively. SSc was the most common primary cause of death (n = 35) and interstitial lung disease (ILD) was the most common SSc-related cause of death (n = 13). Moreover, 52% of the patients with diffuse SSc and 33% of those with limited cutaneous SSc died as a result of SSc itself. Patients who died because of SSc were significantly younger [mean ± sd age 65.6 ± 12.7 years, 95% confidence interval (CI) 61.2-70.1] than those who died from other causes (74.2 ± 9.6 years, 95% CI 71.5-76.9) (p = 0.0006). ILD, pulmonary arterial hypertension, gastrointestinal involvement, male gender, and older age at disease onset predicted death. CONCLUSION: The disease itself was the major cause of death among Finnish SSc patients, in both diffuse and limited forms of SSc.

Systemic sclerosis: changes in the incidence rates in the Finnish population during the years 1999-2018.

OBJECTIVE: The aim of our study was to examine changes in the incidence of systemic sclerosis (SSc) in Finland using two different classification criteria. METHOD: Medical records of patients who had been registered with ICD-10 code M34 from 1999 to 2018 in two university hospitals were reviewed retrospectively. This period was divided into 5 year periods: 1999-2003, 2004-2008, 2009-2013, and 2014-2018. Using American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) 2013 criteria and clinical findings, we reclassified patients into four groups: diffuse SSc, limited SSc, sine SSc, or early SSc. In the same population, we also investigated whether the ACR 1980 criteria were fulfilled. RESULTS: In 1999-2018, 246 new patients with SSc and 45 patients with early SSc were identified using ACR/EULAR 2013 criteria. Of these patients, 70 fulfilled the ACR 1980 criteria. Using ACR/EULAR 2013 criteria, the increase in new diagnoses was statistically significant when comparing the fourth period with the first period (p = 0.0012). The increase was due to a rise in limited SSc. Mean annual incidence rates in these groups were 0.9, 1.2, 1.9, and 2.8 per 100 000 inhabitants ≥ 16 years old. An increasing trend was also seen when using ACR 1980 criteria, but this was not statistically significant. CONCLUSION: The incidence of SSc increased during the period between 1999-2003 and 2014-2018 using ACR/EULAR 2013, but not using ACR 1980 criteria. The increase was detected within a limited SSc subclass, owing to more sensitive classification criteria.

The validity of systemic sclerosis diagnoses in two university hospitals in Finland.

OBJECTIVE: This study aimed to determine the validity of systemic sclerosis (SSc) diagnoses in Finnish university hospitals. METHOD: Electronic medical records for 385 patients with a registered diagnosis of SSc (ICD-10 code M34) in two Finnish university hospitals from 2008 to 2018 were reviewed to assess whether each patient's diagnosis was correct. RESULTS: The positive predictive value (PPV) of a diagnosis of SSc was 0.66 when fulfilment of the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SSc was required; the PPV was 0.75 if patients meeting the 2001 LeRoy and Medsger classification criteria for early SSc were also included. When a diagnosis of SSc was made in a department of rheumatology, the PPV was 0.78, and 0.90 when including patients with early SSc. For the more specific diagnosis of limited cutaneous SSc (lcSSc), the PPV was 0.80, and 0.95 when including early SSc. For an lcSSc diagnosis made in rheumatology, the PPV was 0.81, and 0.97 with early SSc included. CONCLUSION: These results demonstrate that in these two Finnish university hospitals, the diagnostic validity of a diagnosis of SSc was good if it was diagnosed in the department of rheumatology. For a more specific diagnosis of lcSSc, the most prevalent form of SSc in Finland, the validity was good even when registered in any department.