Background Infection due to severe acute respiratory syndrome coronavirus 2 is typically associated with a respiratory syndrome, but gastrointestinal symptoms have been described in early reports from China. However, data from European centres are scarce. Objectives We aimed to characterise the gastrointestinal manifestations of patients with coronavirus disease 2019 (COVID-19) and their disease course. Methods Patients admitted at our centre between March and April 2020 with diagnosis of COVID-19 were included. Asymptomatic patients or those without symptom information were excluded. Clinical features, laboratory data and disease severity (mechanical ventilation, intensive care admission or death) were analysed. Results Two-hundred one patients were included (median age 71 years; 56.2% male). Digestive symptoms were reported by 60 (29.9%) patients during the disease course, being part of the disease presentation in 34 (16.9%). The most frequent were diarrhoea in 36 patients (17.9%). Patients with gastrointestinal symptoms were younger (P = 0.032), had higher haemoglobin levels (P = 0.002) and lower C-reactive protein (P = 0.045) and potassium levels (P = 0.004). Patients with digestive symptoms had less severe disease (28.3 vs. 44.0%; P = 0.038). Regarding liver damage, aspartate aminotransferase (AST) was elevated in 65.2% of patients and alanine aminotransferase (ALT) in 62.7%, but these patients did not present a more severe disease (elevated AST P = 0.062; elevated ALT P = 0.276). Conclusion A significant portion of COVID-19 patients have digestive symptoms, mostly at presentation. This should be taken into account in order to keep a high level of suspicion to reach an early diagnosis and setup infection control measures to control the transmission rate. This subgroup of patients appears to have a less severe disease course.
Background:Antisynthetase syndrome (ASyS) may have different clinical phenotypes and outcomes associated with different anti-aminoacyl RNA-synthetase (anti-ARS) antibodies. Its wide clinical spectrum can include inflammatory myopathy, interstitial lung disease (ILD), arthritis, fever, mechanic’s hands, and Raynaud phenomenon (RP).Objectives:To describe a nationwide, multicentre cohort of Portuguese patients with ASyS.Methods:Retrospective analysis of patients with ASyS from nine Portuguese Rheumatology centers. Data on patients’ signs and symptoms, laboratory results, pulmonary radiological findings (computed tomography) and treatment (immunomodulators) were collected.Results:Among the 70 patients included, 42 patients (60%) were anti-Jo1–positive, 11 (15.7%) were anti-PL12–positive, 10 (14.3%) were anti-PL7–positive, 4 (5.7%) were anti-EJ–positive and 2 (2.9%) were anti-OJ positive. In one patient it was not possible to identify the type of antibody. Antibody overlap was found in 15 patients (21.4%), who were positive for anti-Ro52 antibodies. The general clinical characteristics are shown in Table 1. The diagnostic delay was greater in patients positive for anti-OJ, followed by anti-Jo-1 and anti-PL12. The follow-up was shorter for anti-PL7 and anti-OJ-positive patients. Anti-PL7-positive patients had lower rates of arthritis when compared to anti-Jo1 (p< 0.01). When compared with anti-Jo-1 ARS, myositis was less common in anti-PL12 (p < 0.01). ILD prevalence was similar in the different ARS subgroups. Glucocorticoids (GCs) were the most frequently used class of drugs. A more conservative treatment plan (e.g. GCs plus methotrexate or azathioprine) was the treatment of choice in ASyS with myositis and/or arthritis involvement. Rituximab or mycophenolate mofetil were preferred when lung involvement occurred. Only two deaths were reported, being one associated with lung neoplasia.Conclusion:This is the first study investigating the clinical phenotypes of Portuguese patients with ASyS. These results are generally concordant with data retrieved from international cohorts.References:[1]Mahler M, Miller FW, Fritzler MJ. Idiopathic inflammatory myopathies and the anti-synthetase syndrome: a comprehensive review. Autoimmun Rev 2014;13:367–71.Table 1.Patient characteristics according to the anti-ARS. ILD - interstitial lung disease; IQR- interquartile range; NSIP - Non-specific interstitial pneumonia; UIP - Usual interstitial pneumonia; yrs - yearsVariablesOverall, n=70Jo-1, n=42(60%)PL-12, n=11 (15.7%)PL-7, n=10 (14.3%)EJ, n=4 (5.7%)OJ, n=2 (2.9%)Mean age at onset, yrs52 ± 1546.6 ± 14.455.2 ± 14.756.5±12.556.3±11.273.5±2.1Female, n (%)49 (70)29 (69)9 (81.8)7 (70)2 (50)2 (100)Median age in years at disease onset (IQR)52 (15-75)48 (15-70)59 (20-70)62 (39-73)60 (40-65)73.5 (72-75)Median follow-up time in yrs (IQR)3 (0-32)5 (0-32)3 (0-13)1 (1-4)4 (2-21)1 (0-2)Median diagnostic delay in yrs (IQR)6 (1-33)7 (1-33)7 (2-19)4 (1-23)1.5 (1-2)12.5 (2-21)Myositis, n (%) and Comparison Anti-Jo.1 ARS vs PL-12 and PL-736 (51.4)25 (59.5)3 (27.3)*p < 0.014 (40)p=0.73 (75)-0-ILD, n (%) and Comparison Anti-Jo.1 ARS vs PL-12 and PL-753 (75.7)33 (78.6)8 (72.7)p = 0.986 (60) p=0.564 (100)-1 (50)- ILD pattern - NSIP, n (%)30 (56.6)18 (54.5)6 (75)3 (50)1 (25)0 ILD pattern - UIP, n (%)6 (11.3)3 (9.1)1 (12.5)1 (16.7)1 (25)0 ILD pattern - other specific pattern, n (%)6 (11.3)4 (12.1)02 (33.3)1 (25)0 ILD pattern - non-specific pattern, n (%)11 (15.7)8 (24.2)1 (12.5)01 (25)1 (100)Mechanic’s hands (%), n (%)23 (32.9)14 (33.3)3 (27.3)2 (20)01 (50)General impairment, n (%)18 (25.7)11 (26.2)3 (27.3)2 (20)2 (50)0Fever, n (%)7 (10)4 (9.5)2 (20.2)01 (25)0Raynaud phenomenon, n (%)22 (31.4)11 (26.2)7 (63.6)02 (50)0Arthritis, n (%) and Comparison Anti-Jo.1 ARS vs PL-12 and PL-743 (61.4)29 (69)5 (45.4)p=0.072 (20)*p < 0.012 (50)-1 (50)-Malignancy, n (%)4 (5.7)3 (7.1)1 (9.1)000Deaths, n (%)2 (2.9)2 (2.4)0001 (50)Disclosure of Interests:None declared
Background:Systemic sclerosis (SSc) may present distinctive manifestations and survival in different ethnic and geographic groups.Objectives:To describe the clinical features, treatments, and survival of adult SSc patients registered in Reuma.pt/SSc.Methods:Demographic features, SSc subsets, fulfilment of classification criteria, clinical and immunologic characteristics, comorbidities, medication and deaths were reviewed. Survival was calculated for patients included in the registry within the first 2 years of diagnosis.Results:In total, 1054 patients were included, 87.5% female, mean age at diagnosis 52.7 ± 14.8 years. The most common subset was limited cutaneous (lc)SSc (56.3%), followed by diffuse cutaneous (dc)SSc (17.5%), preclinical SSc (13%), overlap syndrome (9.8%) and SSc sine scleroderma (3.3%). Raynaud’s phenomenon (93.4%) and skin thickening (76.9%) were the most observed manifestations. Gastrointestinal (62.8% vs 47.8%), pulmonary (59.5% vs 23%) and cardiac (12.8% vs 6.9%) involvement were significantly more prevalent in dcSSc compared to lcSSc (Table 1). 52.5% of patients were ACA positive and 21% anti-topoisomerase positive, with significant differences between lcSSc and dcSSc. One third of patients was treated with immunomodulators, 53.6% with vasodilators, 23% received glucocorticoids and 2.3% biologics.During the median follow-up 12.4 years, 83 deaths (7.9%) were verified. The overall 1, 2 and 5 years survival was 98.0%, 96.8% and 92.6% respectively, without significant differences between lcSSc and dcSSc (Figure 1).Conclusion:Reuma.pt/SSc register is useful in routine patient monitoring and contributes to improve knowledge about this rare and complex disease. Clinical features of Portuguese SSc patients are similar to what has been described in other populations although the overall 5-year survival in recently diagnosed patients appears to be higher than previously reported.Table 1.Cumulative clinical and immunologic characteristics of Portuguese SSc patientsClinical and immunologic featuresTotalN=1054Limited cutaneous SScN= 576 (56.3%)Diffuse cutaneous SScN=180 (17.5%)P valueSkin involvement – N(%) N=987688 (90.6)525 (90.7)180 (100)<0.01Skin thickening * – N (%) N= 962680 (76.9)512 (88.9)180 (100)<0.01Digital ulcers – N (%) N=970325(33.5)186 (34.7)4 (51.5)<0.01Raynaud’s Phenomenon – N (%) N=1010943 (93.4)539 (95.7)157 (92.4)0.06Musculoskeletal involvement – N(%) N=972346 (45.6)247 (42.7)99 (55)<0.01Cardiac involvement –N(%) – N=92471 (7.7)36 (6.9)19 (12.8)0.02Renal involvement –N(%) – N= 91717 (1.9)8!1.5)6 (4.1)0.07Gastrointestinal involvement - N(%) N=933508 (48.2)277 (47.8)113 (62.8)<0.01Pulmonary involvement – N(%) N=915261 (28.5)119 (23)88 (59.5)<0.01PAH – N(%) N= 87114 (1.6)10 (2)1 (0.7)0.23Intersticial lung disease – N(%) N=765218 (28.5)100 (22.7)75 (57.7)<0.01Antinuclear antibodies - N(%) N=1040934 (89.8)522 (90.2)154 (88.5)0.57Anti-centromere – N(%) N= 1027540 (52.6)383 (67.1)16 (9.5)<0.01Anti-Scl70 – N(%) N=1020214 (21)12 (3.3)104 (60.1)<0.01Anti-RNA polymerase III – N(%) N=71025 (3.5)12 (3.3)7 (5.6)0.38ComorbiditiesHypertension – N(%) N=431117 (27.1)76 (29.7)67 (20.7)0.1Hyperlipidemia – N(%) N=43171 (13.4)72 (12.2)24 (15.9)0.08Neoplasia – N(%) N=105429 (2.8)12 (2.1)7 (3.9)0.14PDE-5 (phosdiasterase-5); PPIs (proton pump inhibitors); PAH-Pulmonary arterial hypertension confirmed by right heart catheterization. Immunomodulators includes Metothrexate, Leflunomide, Hydroxycloroquine; Azathioprine, Mycophenolate Mofetil and Cyclophosphamide; * Does not include sclerodactyly.Figure 1.Panel A - Survival in years from diagnosis of patients with SSc included in Reuma.pt in the first 2 years of disease (N=472). Panel B - survival according to SSc subset (lcSSc and dcSSC).Disclosure of Interests:None declared
Evidence for the role of sex in the clinical manifestations of systemic sclerosis (SSc) patients is emerging. Some multicenter cohorts have shown that male SSc patients have more severe disease and worse survival. To assess the differences in clinical manifestations and survival in Portuguese SSc patients according to gender. Data from male and female adult SSc patients included in the Rheumatic Diseases Portuguese Register (Reuma.pt) were analysed and compared. Survival was calculated for patients included in Reuma.pt. within the first two years of diagnosis (inception cohort). In total, 1054 adult patients with SSc were included, 12.5% males. No differences in demographic features and comorbidities were found between the sexes, except for a higher rate of cigarette smokers among men. Diffuse cutaneous SSc and anti-topoisomerase antibodies were more prevalent in males than females. Additionally, male patients presented significantly more myositis, interstitial lung disease and gastric involvement. There were no differences in the patterns of drug use between the sexes. During follow-up, more deaths were reported in men than women (12.1% vs 7.3%, p = 0.04). The overall 1-, 3-, and 5-year survivals from diagnosis of the inception cohort (N = 469) for men vs women were 96.4% vs 98.2%, 93% vs 95.9%, and 75.8% vs 93.2%, respectively, with statistically significant differences (p < 0.01). This study confirms the existence of gender differences in clinical and immunological SSc features. Although SSc is less common in men than women, men have a more severe expression of skin and internal organ involvement and worse survival.
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