Idiopathic pulmonary fibrosis in the practice of a family doctor

Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology, mainly characterized by a progressive deterioration in lung function due to active fibrosis. It is a variant of chronic progressive fibrosing interstitial pneumonia. The disease most often occurs between the ages of 50 and 70. According to the disease register in the Russian Federation, the prevalence of the disease in the Russian Federation is 4-7 people per hundred thousand of the population [1].Worldwide, the IPF incidence, prevalence and mortality increases over time [2, 3]. In a systematic review of the study by Hutchinson et al. [2] based on data on IPF morbidity and mortality in 21 countries between 1968 and 2012, it was determined that both morbidity and mortality have increased worldwide since 2000.

Five-year follow-up of new cases after a coeliac disease mass screening

ObjectiveWe previously performed a population-based mass screening of coeliac disease in children aged 12 years in two birth cohorts resulting in 296 seropositive children, of whom 242 were diagnosed with coeliac disease after duodenal biopsies. In this follow-up study, we wanted to identify new cases in the screening population that tested negative—either converting from potential coeliac disease (seropositive but normal duodenal mucosa) or converting from seronegative at screening to diagnosed coeliac disease.MethodsAll seropositive children were invited to a follow-up appointment 5 years after the screening with renewed serological testing and recommended endoscopic investigation if seropositive. Seronegative children in the screening study (n=12 353) were linked to the National Swedish Childhood Coeliac Disease Register to find cases diagnosed in healthcare during the same period.ResultsIn total, 230 (77%) came to the follow-up appointment, including 34 of 39 with potential coeliac disease. Of these, 11 (32%) had converted to coeliac disease. One new case was found in the National Swedish Childhood Coeliac Disease Register who received the diagnosis through routine screening in children with type 1 diabetes.ConclusionsThere is a high risk of conversion to coeliac disease among those with potential disease. However, a negative screening test was associated with a very low risk for a clinical diagnosis within a follow-up period of 5 years.

Data from national health registers as endpoints for the Tromsø Study: Correctness and completeness of stroke diagnoses

Aim: To assess whether stroke diagnoses in national health registers are sufficiently correct and complete to replace manual collection of endpoint data for the Tromsø Study, a population-based epidemiological study. Method: Using the Tromsø Study Cardiovascular Disease Register for 2013–2014 as the gold standard, we calculated correctness (defined as positive predictive value, PPV) and completeness (defined as sensitivity) of stroke cases in four different data subsets derived from the Norwegian Patient Register and the Norwegian Stroke Register. We calculated the sensitivity and PPV with 95% confidence intervals (CIs) assuming a normal approximation of the binomial distribution. Results: In the Norwegian Stroke Register we found a sensitivity of 79.8% (95% CI 74.2–85.4) and a PPV of 97.5% (95% CI 95.1–99.9). In the Norwegian Patient Register the sensitivity was 86.4% (95% CI 81.6–91.1) and the PPV was 84.2% (95% CI 79.2–89.2). The overall highest levels were found in a subset based on a linkage between the Norwegian Stroke Register and the Norwegian Patient Register, with a sensitivity of 88.9% (95% CI 84.5–93.3), and a PPV of 89.3% (95% CI 85.0–93.6). Conclusions: Data from the Norwegian Patient Register and from the linked data set between the Norwegian Patient Register and the Norwegian Stroke Register had acceptable levels of correctness and completeness to be considered as endpoint sources for the Tromsø Study Cardiovascular Disease Register. The benefits of using data from national registers as endpoints in epidemiological studies must be weighed against the impact of potentially decreased data quality.

P284 Drug survival and remission rates in ustekinumab treated ulcerative colitis: Results from the Swedish Inflammatory Bowel Disease register (SWIBREG)

Background Randomised controlled trials may not correctly reflect clinical practice. We aimed to assess the clinical effectiveness of ustekinumab in a real-world cohort of patients with ulcerative colitis (UC). Methods This observational, multi-centre cohort study explored ustekinumab treated patients with UC from the Swedish inflammatory bowel disease register (SWIBREG), a nationwide quality register. Prospectively collected clinical data were extracted December 2020. The primary outcome was the 16-week ustekinumab continuation rate. Secondary outcomes included A) drug continuation rate at the end of follow-up, B) corticosteroid-free biochemical remission, defined as f-Calprotectin<250µg/g, and C) corticosteroid-free clinical remission per patient-reported Mayo score, i.e. a rectal bleeding subscore <1 and a stool frequency subscore ≤1 and not greater than baseline. Continuous data are presented as median and (interquartile range). Differences between baseline and follow-up visits were assessed by the Wilcoxon signed-rank test. Results In total, 145 patients were included and followed for a median period of 32 (19-56) weeks. Baseline characteristics are presented in Table 1. The drug continuation rate was 87% (126/145) at 16 weeks and 69% (100/145) at end of follow-up (Figure 1-2). Corticosteroid-free clinical and biochemical remission rates at follow-up visits are shown in Figure 3. The 6-point Mayo score decreased from 5 (3-6) at baseline to 2 (2-4) at 16 weeks (p<0.01) and to 3 (2-4) at last follow-up (p<0.01). F-calprotectin levels decreased from 779 (252-1530) µg/g to 246 (56-844) µg/g at week 16 (p=0.02) and to 142 (36-935) µg/g at last follow-up (p<0.01). Conclusion Ustekinumab was associated with clinical effectiveness in this nationwide real-world treatment refractory cohort.

Transmission of tuberculosis between foreign-born and Finnish-born populations in Finland, 2014–2017

We describe the epidemiology of tuberculosis (TB) and characterized Mycobacterium tuberculosis (M. tuberculosis) isolates to evaluate transmission between foreign-born and Finnish-born populations. Data on TB cases were obtained from the National Infectious Disease Register and denominator data on legal residents and their country of birth from the Population Information System. M. tuberculosis isolates were genotyped by spoligotyping and Mycobacterial Interspersed Repetitive Unit Variable Number Tandem Repeat (MIRU-VNTR). We characterized clusters by age, sex, origin and region of living which included both foreign-born cases and those born in Finland. During 2014–2017, 1015 TB cases were notified; 814 were confirmed by culture. The proportion of foreign-born cases increased from 33.3% to 39.0%. Foreign-born TB cases were younger (median age, 28 vs. 75 years), and had extrapulmonary TB or multidrug-TB more often than Finnish-born cases (P<0.01 for all comparisons). Foreign-born cases were born in 60 different countries; most commonly in Somalia (25.5%). Altogether 795 isolates were genotyped; 31.2% belonged to 80 different clusters (range, 2–13 cases/cluster). Fourteen (17.5%) clusters included isolates from both Finnish-born and foreign-born cases. An epidemiological link between cases was identified by (epidemiological) background information in two clusters. Although the proportion of foreign-born TB cases was considerable, our data suggests that transmission of TB between foreign and Finnish born population is uncommon.

Assessing longer-term health trends: disease registers

The objectives of this chapter are to: describe different types of disease register; explain the principles and requirements of disease registration; explore two examples of disease registration: cancer registration, and the English National Congenital Anomaly and Rare Disease Registration Service (NCARDRS); enable you to use disease registers effectively, with an awareness of their strengths and limitations.

Tuberculosis in people with rheumatic disease in Finland 1995–2007: a nationwide retrospective register study

Objectives RA and its medication, especially TNF-α inhibitors, increase the risk of clinical tuberculosis (TB) infection. We aimed to investigate the clinical manifestations, incidence and temporal changes in TB occurring concurrently with rheumatic diseases (RDs) between 1995 and 2007. Methods We combined the register of the Social Insurance Institution of Finland and the National Infectious Disease Register to find adult patients with reimbursed DMARDs and with a TB notification between 1995 and 2007. After reviewing the medical records, we described their clinical manifestations and medications, explored TB incidence trends using Poisson regression, and compared the incidence of TB with that of the general population. Results We identified 291 patients with both TB and rheumatic disease (RD), 196 of whom had RA. Between 1995 and 2007, the incidence of TB in adult RD decreased from 58.8 to 30.0 per 100 000 (trend P < 0.001, average marginal effect −3.4/100 000 per year, 95% CI −4.4, −2.4). Compared with the general population, the incidence was ∼4-fold. Among RD patients, pulmonary TB was the most common form of TB (72.6%). Disseminated TB was present in 56 (19.6%) patients. Conclusion The incidence of TB among RD patients was ∼4-fold that of the general population, and it declined between 1995 and 2007. Disseminated TB was present in nearly 20% of patients.