Patient reported experiences of Swedish patients being investigated for cancer during the Covid-19 pandemic.

BACKGROUND: Patient reported experiences in individuals being investigated for cancer have been recorded in a nationwide survey in Sweden, providing an opportunity to assess the impact of the Covid-19-pandemic. MATERIAL AND METHODS: Questionnaires from 45920 patients were analyzed to assess the experience of being investigated for cancer. Data from before the Covid-19-pandemic (2018-2019) was compared to data acquired during the pandemic (2020-2021), using chi-square and Wilcoxon rank sum tests. Both, patients who were cleared from suspicion of cancer and those who were diagnosed with cancer were included. RESULTS: Fewer patients in total visited health services during the pandemic. However, patients that did seek help did so to a similar extent during as prior to the pandemic. Patient waiting time was perceived to be shorter during the pandemic and judged as neither too long nor too short by most patients. The emotional support to patients improved during the pandemic, whereas the support to next of kin declined. A majority of patients received the results from the investigation in a meeting with the physician. Although there was a preference for receiving results in a meeting with the physician, the pandemic has brought an increasing interest in receiving results by phone. CONCLUSION: Swedish cancer healthcare has shown resilience during the Covid-19-pandemic, maintaining high patient satisfaction while working under conditions of extraordinary pressure. Patients became more open to alternatives to physical "in person" health care visits which could lead to more digital visits in the future. However, support to significant others demands special attention.

The Use of IL-17 and IL-23 Inhibitors in Swedish Clinical Practice: A Register-Based Analysis.

BACKGROUND: Interleukin (IL) inhibitors have made completely cleared skin achievable for many patients with moderate to severe psoriasis in clinical trial settings. Few observational studies assess treatment response in accordance with treatment goals in guidelines. OBJECTIVES: The aim of the study was to analyze the treatment response of IL-17/IL-23 inhibitors in clinical practice and the proportions of patients that reach the treatment target of the Psoriasis Area and Severity Index (PASI) < 3 and the Dermatology Life Quality Index (DLQI) ≤5. METHODS: A longitudinal, observational study based on the Swedish National Registry for Systemic Treatment of Psoriasis, PsoReg. Patients using IL-17/IL-23 inhibitors with assessments of PASI, DLQI, and EQ-5D before (maximum 6 months) and after (3-12 months) initiation of IL-17/IL-23 were included. RESULTS: In total, 333 patients using IL-17/IL-23 inhibitors were included. Eighty percent (n = 266) received IL-17 inhibitors, and 20% (n = 67) received IL-23 inhibitors. Sixty-six percent of patients reached both PASI <3 and DLQI ≤5, 23% reached one target, and 11% reached none. The mean (SD) PASI, DLQI, and EQ-5D improvements were 6.75 (6.99), 7.14 (7.97), and 0.126 (0.296), respectively. There was no statistically significant difference in outcomes between IL-17 and IL-23 inhibitor treatment groups. CONCLUSIONS: IL-17/IL-23 inhibitors are effective in clinical practice, but there is still an unmet therapeutic need in moderate to severe psoriasis.

Economic Burden of Palmoplantar Pustulosis in Sweden: A Population-based Register Study.

The aim of this study was to estimate the economic burden of palmoplantar pustulosis, a chronic relapsing skin condition commonly occurring in combination with psoriasis vulgaris. Using data from the Swedish National Patient Register and Swedish Prescribed Drug Register for 2015, the study estimated all-cause and palmoplantar pustulosis-specific healthcare resource use (inpatient stays, physician visits and drug use) for 14,715 patients with palmoplantar pustulosis, and compared these both with matched controls from the general population and with patients with psoriasis vulgaris (without palmoplantar pustulosis). Mean annual direct costs for a patient with palmoplantar pustulosis was higher compared with costs for the general population (3,000 vs 1,700 Euro, p < 0.001). Compared with psoriasis vulgaris, more patients with palmoplantar pustulosis had inpatient stays, but fewer had physician visits and psoriasis-related drugs; the overall costs were similar. Only a small fraction of the costs of physician visits and inpatient stays for patients with palmoplantar pustulosis were attributable to specific palmoplantar pustulosis problems, indicating a clear comorbidity burden in palmoplantar pustulosis.

Prolonged Sick Leave Before and After Diagnosis of Generalized Pustular Psoriasis: A Swedish Population-based Register Study.

The aim of this study was to analyse sick leave in generalized pustular psoriasis, the most severe form of pustular psoriasis. Prolonged sick leave of >14 days was analysed for 502 patients with generalized pustular psoriasis compared with controls with psoriasis vulgaris and matched controls from the general population. Using data from the Swedish National Patient Register, and the Longitudinal integrated database for health insurance and labour market studies, the study estimated the mean number of sick leave days in the year of first diagnosis of generalized pustular psoriasis (index year) and for 2 years before and after the index year. Patients with generalized pustular psoriasis were on sick leave to a larger extent than both control populations for all study years. The number of sick leave days peaked in the index year and then reduced. Compared with the control populations, sick leave in generalized pustular psoriasis was already higher prior to diagnosis, indicating delayed diagnosis and/or a comorbidity burden.

Perception of information to Swedish melanoma patients in routine clinical practice - a cross-sectional survey.

BACKGROUND: Comprehensible, relevant information empowers patients, allowing them to take an active part in their care. We aim to investigate how Swedish melanoma patients perceive information provided in routine clinical practice and explore the correlation between satisfaction with information, symptoms and functioning scales, and quality of life. METHODS: A cross-sectional study where EORTC QLQ-C30, EORTC QLQ-INFO25 and EQ-5D-3L questionnaires were sent to 1213 patients by post and 792 responded (65%). RESULTS: Only 0.5% reported that they wished to have received less information. The amount of information received and the satisfaction with that information was age-dependent, where older patients reported receiving less information than younger patients. Middle-aged patients were more satisfied with the information compared to both younger and older patients. The perception of having received sufficient information correlated negatively with anxiety. Higher satisfaction with the information also correlated positively with scores for functioning scales and negatively with degree of symptoms. No difference was perceived in information levels between disease stage apart from the scale "information about other services", where patients with more severe disease reported receiving more information. Men and women reported equal satisfaction concerning the information received. CONCLUSIONS: Patients lack sufficient information and age affects the perception of it. It is of utmost importance to tailor the information to suit the individual needs of a given patient, as satisfaction with the information received correlates with the patient's well-being.

Prevalence and incidence of generalized pustular psoriasis in Sweden: a population-based register study.

BACKGROUND: Generalized pustular psoriasis (GPP) is a severe form of pustular psoriasis with generalized eruption of sterile pustules, often along with systemic symptoms. There is a scarcity of population-based estimates of GPP prevalence and incidence. OBJECTIVES: To estimate (i) the prevalence and incidence of GPP in the Swedish general population and (ii) the prevalence of psoriasis vulgaris within the GPP population. METHODS: We identified cases (2004-2015) with one ICD-10 diagnostic code (base case) for GPP within the Swedish National Patient Register, which covers inpatient and outpatient secondary care. Cases were linked to the Swedish Total Population Register, and point prevalence was estimated as on 31 December 2015. In two alternative analyses we changed case definitions to: (i) requiring two visits (strict case 1) and (ii) requiring two visits of which one was within dermatology/internal medicine (strict case 2). RESULTS: The base case point prevalence of GPP was estimated at 9.1 per 100 000 (women, 11.2; men, 7.0) and the annual prevalence in 2015 was estimated at 1.53 per 100 000. Among the GPP population, 43% also had a psoriasis vulgaris code. The incidence of GPP in 2015 was estimated at 0.82 per 100 000 (women, 0.93; men, 0.74). The criteria used had an impact on prevalence and incidence estimates: prevalence strict case 1 gave 3.8 per 100 000 and incidence strict case 1 gave 0.42 per 100 000. CONCLUSIONS: Results indicate that the estimated GPP population in Sweden is within the range of previous published estimates. However, estimates were sensitive to the GPP case criteria used. The findings enhance demands for studies using validated diagnostic algorithms.

Impact of Bariatric Surgery on Moderate to Severe Psoriasis: A Retrospective Nationwide Registry Study.

Studies of the effects of bariatric surgery on psoriasis are few, with conflicting results. By linking the Swedish National Register for Systemic Treatment of Psoriasis (PsoReg) with the Scandinavian Obesity Surgery Registry (SOReg), individuals with psoriasis who had undergone bariatric surgery in Sweden during 2008 to 2018 were identified, and matched with data for patients with psoriasis in PsoReg. Psoriasis Area Severity Index (PASI) and Dermatology Life Quality Index (DLQI) were compared between the groups. Altogether, 50 operated individuals (median body mass index (BMI) 38.7 kg/m2]) and 91 non-operated individuals (median BMI 33.0 kg/m2) were included. Control of disease at baseline was good in both groups. Linear mixed models showed no significant difference in psoriasis disease burden, measured as changes in mean PASI (ΔPASI) (-1.2, p = 0.43) and DLQI (ΔDLQI) (-2.2, p = 0.34). In summary, this study demonstrated no significant effect of bariatric surgery on psoriasis disease burden in patients with relatively well-controlled moderate to severe psoriasis.

Anti-TNF treatment during pregnancy and birth outcomes: A population-based study from Denmark, Finland, and Sweden.

PURPOSE: To study the risk of preterm birth, caesarean section, and small for gestational age after anti-tumor necrosis factor agent treatment (anti-TNF) in pregnancy. METHODS: Population-based study including women with inflammatory bowel disease, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, and psoriasis, and their infants born 2006 to 2013 from the national health registers in Denmark, Finland, and Sweden. Women treated with anti-TNF were compared with women with nonbiologic systemic treatment. Adalimumab, etanercept, and infliximab were compared pairwise. Continuation of treatment in early pregnancy was compared with discontinuation. Odds ratios with 95% confidence intervals were calculated in logistic regression models adjusted for country and maternal characteristics. RESULTS: Among 1 633 909 births, 1027 infants were to women treated with anti-TNF and 9399 to women with nonbiologic systemic treatment. Compared with non-biologic systemic treatment, women with anti-TNF treatment had a higher risk of preterm birth, odds ratio 1.61 (1.29-2.02) and caesarean section, 1.57 (1.35-1.82). The odds ratio for small for gestational age was 1.36 (0.96-1.92). In pairwise comparisons, infliximab was associated with a higher risk of severely small for gestational age for inflammatory joint and skin diseases but not for inflammatory bowel disease. Discontinuation of anti-TNF had opposite effects on preterm birth for inflammatory bowel disease and inflammatory joint and skin diseases. CONCLUSIONS: Anti-TNF agents were associated with increased risks of preterm birth, caesarean section, and small for gestational age. However, the diverse findings across disease groups may indicate an association related to the underlying disease activity, rather than to agent-specific effects.

Periodontal Ehlers-Danlos Syndrome Is Caused by Mutations in C1R and C1S, which Encode Subcomponents C1r and C1s of Complement.

Periodontal Ehlers-Danlos syndrome (pEDS) is an autosomal-dominant disorder characterized by early-onset periodontitis leading to premature loss of teeth, joint hypermobility, and mild skin findings. A locus was mapped to an approximately 5.8 Mb region at 12p13.1 but no candidate gene was identified. In an international consortium we recruited 19 independent families comprising 107 individuals with pEDS to identify the locus, characterize the clinical details in those with defined genetic causes, and try to understand the physiological basis of the condition. In 17 of these families, we identified heterozygous missense or in-frame insertion/deletion mutations in C1R (15 families) or C1S (2 families), contiguous genes in the mapped locus that encode subunits C1r and C1s of the first component of the classical complement pathway. These two proteins form a heterotetramer that then combines with six C1q subunits. Pathogenic variants involve the subunit interfaces or inter-domain hinges of C1r and C1s and are associated with intracellular retention and mild endoplasmic reticulum enlargement. Clinical features of affected individuals in these families include rapidly progressing periodontitis with onset in the teens or childhood, a previously unrecognized lack of attached gingiva, pretibial hyperpigmentation, skin and vascular fragility, easy bruising, and variable musculoskeletal symptoms. Our findings open a connection between the inflammatory classical complement pathway and connective tissue homeostasis.

How is disease severity associated with quality of life in psoriasis patients? Evidence from a longitudinal population-based study in Sweden.

BACKGROUND: Assessing the impact of disease severity on generic quality of life (QOL) is a critical step in outcomes research and in the development of decision-analytic models structured around health states defined by clinical measures. While data from routine clinical practice found in healthcare registers are increasingly used for research, more attention should be paid to understanding the relationship between clinical measures of disease severity and QOL. The purpose of this work was therefore to investigate this relationship in psoriasis using a population-based dataset. METHODS: Severity was measured by the Psoriasis Area and Severity Index (PASI), which combines severity of erythema, induration, and desquamation into a single value ranging from 0 to 72. The generic EQ-5D-3L utility instrument, under the UK tariff, was used to measure QOL. The association between PASI and EQ-5D-3L was estimated using a population-based dataset of 2674 patients with moderate to severe psoriasis enrolled over ten years in the Swedish psoriasis register (PsoReg). Given the repeated measurement of patients in the register data, a longitudinal fixed-effects model was employed to control for unobserved patient-level heterogeneity. RESULTS: Marginal changes in PASI are associated with a non-linear response in EQ-5D-3L: Moving from PASI 10 to 9 (1 to 0) is associated with an increase of 0.0135 (0.0174) in EQ-5D-3L. Furthermore, unobserved patient-level heterogeneity appears to be an important source of confounding when estimating the relationship between QOL and PASI. CONCLUSIONS: Using register data to estimate the impact of disease severity on QOL while controlling for unobserved patient-level heterogeneity shows that PASI appears to have a larger impact on QOL than previously estimated. Routine collection of generic QOL data in registers should be encouraged to enable similar applications in other disease areas. TRIAL REGISTRATION: Not applicable.

Real-world outcome analysis of continuously and intermittently treated patients with moderate to severe psoriasis after switching to a biologic agent.

BACKGROUND: Clinical studies of continuous versus intermittent biologic therapy for moderate to severe psoriasis demonstrate improved efficacy with continuous treatment. OBJECTIVE: To analyse Swedish real-world data of continuously and intermittently treated biologic-naïve patients after switching to a biologic agent. METHODS: This is an observational study based on PsoReg, the Swedish registry for systemic psoriasis treatment. Outcome effects in biologic-naïve patients who switched to a biologic agent (n = 351) were analysed in groups of continuous, intermittent and terminated treatment. RESULTS: Intermittently treated patients (n = 50) reported higher Psoriasis Area and Severity Index and Dermatology Life Quality Index values after switching than patients with continuous (n = 260) or terminated treatment (n = 41). STUDY LIMITATIONS: The reason for intermittent treatment was not recorded. The intermittently treated patients may be a heterogeneous group and a limitation is that it cannot be determined whether less than continuous use was offered to handle negative aspects. CONCLUSION: Patients with continuous biologic treatment tend to achieve better outcomes compared to intermittently treated patients.

Resource use in patients with psoriasis after the introduction of biologics in Sweden.

The introduction of biologics has changed treatment patterns as well as costs in patients with psoriasis. This study was performed to estimate direct and indirect costs of the psoriasis population in Sweden, and to analyse changes in costs between 2006 and 2009. The study population was identified in national registers. Direct costs included health care visits with primary psoriasis diagnoses in specialist care and drugs relevant for treating psoriasis. Productivity loss, including costs of long-term sick leave and disability pension, was estimated as the difference between psoriasis patients and matched controls from the general population. Total direct cost increased from SEK 348 million (~ €39) in 2006 to SEK 459 million (~ €51) in 2009, whereas the total productivity loss decreased from SEK 1,646 (~ €183) to 1,618 million (~ €180) between 2006 and 2009. Although direct costs, especially for biologic agents, have increased for patients with psoriasis over time, this study indicates that costs related to productivity loss are still more substantial.

EDAR-induced hypohidrotic ectodermal dysplasia: a clinical study on signs and symptoms in individuals with a heterozygous c.1072C > T mutation.

BACKGROUND: Mutations in the EDAR-gene cause hypohidrotic ectodermal dysplasia, however, the oral phenotype has been described in a limited number of cases. The aim of the present study was to clinically describe individuals with the c.1072C > T mutation (p. Arg358X) in the EDAR gene with respect to dental signs and saliva secretion, symptoms from other ectodermal structures and to assess orofacial function. METHODS: Individuals in three families living in Sweden, where some members had a known c.1072C > T mutation in the EDAR gene with an autosomal dominant inheritance (AD), were included in a clinical investigation on oral signs and symptoms and self-reported symptoms from other ectodermal structures (n = 37). Confirmation of the c.1072C > T mutation in the EDAR gene were performed by genomic sequencing. Orofacial function was evaluated with NOT-S. RESULTS: The mutation was identified in 17 of 37 family members. The mean number of missing teeth due to agenesis was 10.3 ± 4.1, (range 4-17) in the mutation group and 0.1 ± 0.3, (range 0-1) in the non-mutation group (p < 0.01). All individuals with the mutation were missing the maxillary lateral incisors and one or more of the mandibular incisors; and 81.3% were missing all four. Stimulated saliva secretion was 0.9 ± 0.5 ml/min in the mutation group vs 1.7 ± 0.6 ml/min in the non-mutation group (p < 0.01). Reduced ability to sweat was reported by 82% in the mutation group and by 20% in the non-mutation group (p < 0.01). The mean NOT-S score was 3.0 ± 1.9 (range 0-6) in the mutation group and 1.5 ± 1.1 (range 0-5) in the non-mutation group (p < 0.01). Lisping was present in 56% of individuals in the mutation group. CONCLUSIONS: Individuals with a c.1072C > T mutation in the EDAR-gene displayed a typical pattern of congenitally missing teeth in the frontal area with functional consequences. They therefore have a need for special attention in dental care, both with reference to tooth agenesis and low salivary secretion with an increased risk for caries. Sweating problems were the most frequently reported symptom from other ectodermal structures.

Health-related quality of life in patients with generalized pustular psoriasis - a Swedish register study.

BACKGROUND: Real-world data on health-related quality of life (HRQoL) in generalized pustular psoriasis (GPP) are scarce and studies have been restricted in terms of instruments used for assessments. OBJECTIVE: To assess generic and dermatology-specific HRQoL of patients with GPP compared with patients with plaque psoriasis using real-world data from the Swedish National Register for Systemic Treatment of Psoriasis. METHODS: Cross-sectional data from 2006 to 2021 including 7041 individuals with plaque psoriasis without GPP and 80 patients with GPP, of which 19% also had plaque psoriasis. Total scores for the EuroQol-5 Dimensions (EQ-5D) and Dermatology Life Quality Index (DLQI), as well as degree of severity within the instruments' dimensions/questions, were compared between patient groups. RESULTS: EQ-5D scores were significantly (p < .01) lower (worse) in patients with GPP (mean [standard deviation (SD)] 0.613 [0.346]) vs. patients with plaque psoriasis (mean [SD] 0.715 [0.274]), indicating lower generic HRQoL of patients with GPP. Significantly (p < .01) higher (worse) total DLQI scores were observed for patients with GPP (mean [SD] 10.6 [8.9]) compared with patients with plaque psoriasis (mean [SD] 7.7 [7.1]), with proportionally more patients with GPP having severe (20% vs. 16%) and very severe (17% vs. 8%) problems. The worsened scores for GPP vs. plaque psoriasis were consistent across EQ-5D dimensions and DLQI questions. CONCLUSIONS: Individuals with GPP have a considerable impairment in both generic and dermatology-specific HRQoL. The HRQoL was significantly worse in individuals with GPP compared to individuals with plaque psoriasis. The significant HRQoL impairment of GPP shows the potential value of better healthcare interventions for this multisystem disease.

The study assessed health-related quality of life (HRQoL) in patients with generalized pustular psoriasis (GPP) compared to patients with plaque psoriasis using real-world data from the Swedish National Register for Systemic Treatment of Psoriasis.The results showed significantly worse HRQoL scores by two different HRQoL instruments (EuroQol-5 Dimensions [EQ-5D] and Dermatology Life Quality Index [DLQI]) in patients with GPP compared to patients with plaque psoriasis.The study indicates that individuals with GPP have a considerable impairment in both generic and dermatology-specific HRQoL.

Health-related quality of life in patients with palmoplantar pustulosis - a Swedish register study.

BACKGROUND: Real-world data on health-related quality of life (HRQoL) in palmoplantar pustulosis (PPP) are scarce and few studies have analysed the generic HRQoL. OBJECTIVES: To assess HRQoL using the generic EQ-5D instrument and the Dermatology Life Quality Index (DLQI) instrument in PPP compared to plaque psoriasis. METHODS: Cross-sectional data from PsoReg, the Swedish National Registry for Systemic Treatment of Psoriasis (2006-2021), were examined. The study included 306 patients with PPP, out of which 22% had concomitant plaque psoriasis (n = 68), and 7041 patients with plaque psoriasis only. EQ-5D and DLQI were compared between patients with PPP and patients with plaque psoriasis, overall and stratified by sex. A subgroup analysis compared outcomes for patients with PPP vs. patients with severe plaque psoriasis (Psoriasis Area and Severity Index ≥10). Multiple regression analyses were performed to control for potential confounders (age, sex, comorbidities, lifestyle factors). RESULTS: Patients with PPP were to a larger extent female (79% vs. 37%, p < .01) and older (mean [SD] age 59.9 [11.9] vs. 50.7 [16.0] years, p < .01) than patients with plaque psoriasis. EQ-5D values were significantly lower (worse) in patients with PPP (mean [SD] 0.622 [0.309]) compared to patients with plaque psoriasis (mean [SD] 0.715 [0.274]). No significant difference was observed compared to patients with severe plaque psoriasis (p = .237). DLQI was comparable in PPP and plaque psoriasis patients (p = .117). In the regression analyses, PPP only and PPP with plaque psoriasis were associated with lower EQ-5D values of 0.065 (p < .01) and 0.061 points (p < .10) compared to plaque psoriasis patients. CONCLUSIONS: PPP had a substantial negative impact on patients' generic and dermatology-specific HRQoL. Patients with PPP were worse off in terms of generic HRQoL compared with patients with plaque psoriasis when controlling for the impact of potential confounders.

Real-world data on health-related quality of life in palmoplantar pustulosis (PPP) are scarce and previous studies have been predominantly restricted to the Dermatology Life Quality Index.This study also shows a significant impairment of the generic HRQoL (assessed by the generic EQ-5D instrument) in patients with PPP.Patients with PPP rated their generic HRQoL worse than patients with plaque psoriasis.

The Disruptive Force of Real-World Evidence.

Evidence-based medicine was in the past primarily based on the (meta-)analysis of randomized clinical trials (RCTs) [...].