scholarly journals Serum defensin levels in patients with systemic sclerosis

2020 ◽  
Vol 60 (1) ◽  
Author(s):  
Tugce Emiroglu Gedik ◽  
Hamit Kucuk ◽  
Berna Goker ◽  
Seminur Haznedaroglu ◽  
Hatice Pasaoglu ◽  
...  

Abstract Background Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and lung as well as involvement of kidney, gastrointestinal system and heart. Aetiology and exact mechanism of disease is poorly understood. The association between antimicrobial peptides (AMPs) and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis have been reported. A small number of studies have examined the role of AMPs on autoimmune diseases which has not been studied in scleroderma yet. We aimed to investigate AMP serum levels and their association with disease characteristics of SSc. Methods Forty-two patients (40 female, mean age 42 years) and 38 healthy subjects (32 female, mean age 38 years) were enrolled. For SSc patients, the following data were recorded: disease subset (limited/diffuse), autoantibodies (antinuclear, anti-centromere (ACA), and anti-SCL-70), blood tests, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP), modified Rodnan skin score, presence and history of digital ulcers, kidney, gastrointestinal disease and lung involvement assessed by computed tomography and pulmonary function tests. Association between serum AMPs and disease characteristics were analysed. Results Twenty-nine of the patients had diffuse (69%) and 13 of the patients had limited (31%) systemic sclerosis. Average disease duration was 5.5 years. Pulmonary involvement was detected in 20 patients (47.6%). Serum concentration of alpha defensin was higher than healthy subjects (563 ± 415 vs 377 ± 269 ng/mL, p = 0.02). However, no difference was observed for beta-1 and beta-2 defensins in SSc patients and healthy controls. In sub-group analysis patients with interstitial lung disease had higher levels of alpha defensin than those without lung involvement (684 ± 473 vs 430 ± 299 ng/ml, p = 0.04). There was also correlation between alfa defensin serum concentrations and CRP (r = 0.34). Conclusions Alpha defensin levels are increased in scleroderma patients and correlated with lung involvement indicating a role in the pathogenesis of disease. Trial registration This study is not a clinical trial study.

2018 ◽  
Vol 15 (1) ◽  
pp. 74-78
Author(s):  
Mohammadali Nazarinia ◽  
Asghar Zare ◽  
Mohammad javad Fallahi ◽  
Mesbah Shams

Background:Systemic sclerosis is a disorder of connective tissue with unknown cause, affecting the skin and internal organs, characterized by fibrotic changes.Objective:To determine the correlation between serum homocysteine level and interstitial lung involvement in systemic sclerosis. </P><P> Materials and Methods: In this case – control study, 59 patients who fulfilled the ACR/EULAR classification criteria for systemic sclerosis and were referred to Hafez Hospital of Shiraz, Iran, were included as the case group. Fifty nine healthy subjects were involved as the control group. Patients were divided into two groups based on interstitial lung involvement and two subtypes, diffuse and limited type. Serum homocysteine, vitamin B12, and folate levels compared between the controls, and cases groups.Results:Of 59 case and control group, 53 (%89.8) were female and the mean age did not differ in both groups (P=0.929). Thirty five (%59.3) patients had interstitial lung involvement and 38(%64.4) had diffuse cutaneous systemic sclerosis. The mean serum homocysteine level was 13.9±6.3 µmol/L in the case and 13.7±9.2 µmol/L in the control group (P=0.86). The mean serum homocysteine level did not differ between the patients with and without interstitial lung involvement (P=0.52). The patients with lung involvement was older than those without lung involvement (P=0.004). Lung disease was more common in diffuse type (P=0.014).Conclusion:In our study, serum homocysteine level did not differ between the patients and healthy subjects. Also, there was no correlation between serum homocysteine level and lung involvement, but lung involvement was more common in older patients and also diffuse subtype.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1569.2-1569
Author(s):  
A. Argibay ◽  
I. Novo ◽  
M. Ávila ◽  
P. Diéguez González ◽  
M. Estévez Gil ◽  
...  

Background:Systemic sclerosis (SSc) is a chronic, connective tissue disease with an autoimmune pattern characterized by inflammation, fibrosis and microcirculation changes leading to internal organs malfunctions. The gastrointestinal tract (GIT) is affected in up to 90% of patients with SSc. Any part of the GIT from the mouth to the anus can be affected. There are few descriptive studies about SSc-related GIT involvement.Objectives:We aimed to characterize the GIT involvement in patients with SSc.Methods:This retrospective study included all patients from SSc cohort of our autoimmune diseases unit in a tertiary referral centre. All patients fulfilled SSc criteria proposed by the American College of Rheumatology. All subjects’ histories were evaluated. Laboratory and imaging results were obtained from the hospital files. Patients with digestive manifestations were compared with patients without GIT involvement. Chi2 and t-student were used, using the statistical package SPSS25.0.Results:83 subjects with SSc were included, 68 (81,9%) of them were women. The mean age at the onset of SSc was 62,1 ± 15,3 years (range 26-89) with a mean follow-up of 9,6 ± 7,4 years. 80,7% of patients had limited SSc, 12% diffuse SSc, 4.8% SSc sine scleroderma and 2,4% early SSc. Considering the immunological profile 12 (14,5%) had Scl70 antibodies, 49 (59%) anticentromere and 21 (25,3%) had ANA antibodies without specificity for anti-Scl70 or anticentromere. 37,3% patients had lung involvement, 20,5% scleroderma and 30,1% digital ulcers. 79,5% of SSc patients were treated with proton pump inhibitors or H2 blockers. 53 (63,9%) patients with SSc had GIT involvement. In 11 patients (20,7%) digestive involvement was diagnosed before SSc (mean 26,2 months). Esophageal involvement occurred in 83%, gastric involvement in 28,3%, intestine involvement in 24,5% and liver and biliary tree involvement in 26,4%. See table 1. No significant differences in age, sex, SSc subtype, autoantibody profile, lung involvement, skin disease, mortality and therapy were observed between patients with or without GIT manifestations. There were no deaths associated with GIT involvement. The most common pharmacologic therapy used was proton pump inhibitors (86,8%), domperidone (20,8%) and antibiotic rotation (17%).EsophagealGastricIntestinalLiver and biliary tree44/53 (83%)15/53 (28,3%)12/53 (24,5%)14/53 (26,4%)Esophageal motility disorder 8 (15,1%)Gastroparesis 6 (11,3%)Small bacterial overgrowth 7 (13,2%)Primary biliary cholangitis 9 (17%)Gastroesophageal reflux 40 (75,5%)Abdominal pain /nausea 10 (18,9%)Colonic inertia 1 (1,9%)Autoimmune hepatitis 3 (5,7%)Dysphagia 11 (20,8%)Subacute gastritis 7 (13,2%)Diarrhea 6 (11,3%)Cholestatic liver enzymes 11 (20,8%)Flatulence / abdominal discomfort 6 (11,3%)Cirrhosis 2 (3,8%)Conclusion:Almost two thirds of our cohort of SSc have symptomatic gastrointestinal disease. GIT manifestations are heterogeneous. Symptoms are non-specific and overlapping for a particular anatomical site. Esophagus is the most commonly affected. More than seventy-five per cent of patients experience symptoms of gastroesophageal reflux. We did not find differences among patients with and without SSc GIT disease. 17% of patients had a Reynold’s syndrome.References:[1]Alastal Y et al. Gastrointestinal manifestations associated with systemic sclerosis: results from the nationwide inpatient simple. Ann Gastroenterol 2017; 30 (5): 1-6.[2]Savarino E et al. Gastrointestinal motility disorder assessment in systemic sclerosis. Rheumatology. 2013; 52(6):1095–100.[3]Steen VD et al. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis and rheumatism. 2000; 43(11):2437–44.Disclosure of Interests:None declared


2009 ◽  
Vol 44 (12) ◽  
pp. 1226-1234 ◽  
Author(s):  
Serena Panigada ◽  
Angelo Ravelli ◽  
Michela Silvestri ◽  
Claudio Granata ◽  
Silvia Magni-Manzoni ◽  
...  

2021 ◽  
Vol 14 (2) ◽  
pp. 154
Author(s):  
Barbara Ruaro ◽  
Marco Confalonieri ◽  
Marco Matucci-Cerinic ◽  
Francesco Salton ◽  
Paola Confalonieri ◽  
...  

Systemic sclerosis (SSc) patients are often affected by interstitial lung disease (ILD) and, although there have been recent treatment advances, it remains the leading cause of death among SSc, with a 10-year mortality up to 40%. African Americans and subjects with diffuse cutaneous SSc or anti-topoisomerase 1 antibodies are most commonly affected. Currently, early ILD diagnosis can be made, and it is pivotal to improve the prognosis. The diagnostic mainstay test for SSc-ILD is high-resolution computed tomography for the morphology and pulmonary function tests for the functional aspects. Treatment planning and intensity are guided by the disease severity and risk of progression. Traditionally, therapy has depended on combinations of immunosuppressants, particularly cyclophosphamide and mycophenolate mofetil, which can be supplemented by targeted biological and antifibrotic therapies. Benefits have been observed in trials on hematopoietic autologous stem cell transplantation for patients with progressive SSc, whilst lung transplantation is reserved for refractory SSc-ILD cases. Herein, recent advances in SSc-ILD treatment will be explored.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1592.2-1593
Author(s):  
T. Miyagi ◽  
T. Kameda ◽  
S. Nakashima ◽  
H. Shimada ◽  
R. Wakiya ◽  
...  

Background:Systemic sclerosis (SSc) have various organ involvements including pulmonary hypertension (PH), digital ulcers (DU), and interstitial lung disease (ILD). On the other hand, Nailfold capillary (NFC) abnormalities (enlarged/giant capillaries, fresh or old hemorrhages, avascular areas, ramified/bushy capillaries) detected by capillaroscopy are included in ACR/EULAR classification criteria for SSc as one of important findings. In addition, many studies have reported the relationship between NFC abnormalities and organ involvements (DU, PH) [1][2]. However, there are a few reports about the relationship between NFC abnormalities and ILD.Objectives:We clarify the association with NFC abnormalities and ILD in SSc patients.Methods:We enrolled SSc patients without PH from January 2016 to December 2019 in our institution. SSc patients were diagnosed according to EULAR classification criteria in 2013. ILD was detected by chest CT scans. We assessed severity of ILD with pulmonary function tests (PFT). Abnormal PFT was defined as vital capacity (%VC) or diffusion capacity (DLCO) < 70%. NFC abnormalities were detected with “OptiPiX capillaroscopy Clinic 1.7.x” and the number of capillaries was measured per 1mm in 2nd to 5th fingers of both hand. We defined enlarged and giant capillaries as >30 µm and >50 µm, respectively.Results:We enrolled 59 SSc patients (54 females, 5 males). Mean age is 65.0 ± 8.0 years. Thirty-one patients (52.5%) were complicated with ILD. Mean capillary counts are 6.6/mm. The number of patients with each NFC abnormalities (enlarged capillaries, giant capillaries, microhemorrhages, ramified, avascular areas) are 42, 32, 48, 38, and 33 cases, respectively. Two cases did not have NFC abnormalities. SSc patients with giant capillaries had fewer ILD complications (p <0.05, odds ratio 0.183 [0.059 – 0.57]). Other NFC abnormalities were not associated with ILD in SSc patients. We inspected %VC of 23 patients and DLCO of 20 patients with ILD. Eleven patients had abnormal PFT (5 patients had abnormal %VC and 9 patients had abnormal DLCO). Most of them had not enlarged capillaries than patient with normal PFT (odds ratio 0.11 [0.016 – 0.81]). Other NFC abnormalities including giant capillaries were not associated with abnormal PFT.Conclusion:We investigated the relationship between NFC abnormalities and ILD conplications in SSc patients. NFC abnormalities are associated with ILD complicacion and severity of ILD. It was suggested that no giant capillary in SSc patients may predict ILD complication. Moreover, no enlarged capillary may predict the severe ILD.References:[1]Valeria Riccieri et al. Systemic sclerosis patients with and without pulmonary arterial hypertension: a nailfold capillaroscopy study. Rheumatology, Volume 52, Issue 8, 1 August 2013, Pages 1525–1528[2]Maurizio Cutolo et al. Nailfold Videocapillaroscopic Features and Other Clinical Risk Factors for Digital Ulcers in Systemic Sclerosis: A Multicenter, Prospective Cohort Study. Arthritis Rheumatol. 2016 Oct; 68(10): 2527–2539.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1596.2-1596
Author(s):  
S. Pavlov-Dolijanovic ◽  
V. Koletic ◽  
N. Vujasinovic Stupar ◽  
N. Damjanov

Background:Many treatments have been tried in therapy systemic sclerosis (SSc) patients but use of hyperbaric oxygen therapy (HBOT) is very limited.Objectives:To assess the effects of HBOT to quality of life and state of microcirculation in SSc patients.Methods:18 female patients aged 29-68 years (mean 57 years) with limited SSc and digital or leg ulcers were included in this work. The HBOT protocol comprised 20 sessions 5 day/weekly, 60 min, 100% oxygen at 2.2 ATA. The treated patients were evaluated at baseline and after 20 HBOT sessions. Evaluation consisted of physical examination, capillaroscopy, pulmonary function tests, biochemical analyses, socio-demographic and clinimetric questionnaires: Systemic Sclerosis Questionnaire (SySQ) and Health Assessment Disaability index Questionnaire (HAQ-DI).Results:Mean value [before: after, mean (range)] for SySQ [15.5 (4-48) vs 9.0 (3-31)], HAQ-DI [0.60 (0-2.88) vs 0.35 (0 -1.75)], erythrocyte sedimentation rate [21 (4-42) vs 12 (3-27)], forced vital capacity (96.61±14.44% vs 115.94±16.69%), diffusing lung capacity of carbon monoxide (73.61±6.63% vs 87.33±9.30%) significantly improved after HBOT sessions (p<0.001). There was no significant changes in the total number of capillaries (325 vs 338, p=0.235), mean number of enlarged capillaries (21 vs 27, p=0.182), giant capillaries (14 vs 14, p=0.235) and ramified/bushy capillaries (14 vs 13, p=0.178) before and after HOBT. All patients had digital ulcers, and 5 patients had bilateral lesions (digital and leg ulcers). Mean size of ulceration before HBOT was 12x11mm, and after therapy was 4x4mm (p<0.001). Three patients had digital gangrene. Amputation was not require for any.Conclusion:Our data confirm the efficacy of HBOT in treating SSc patients. Further studies are required to evaluate the protocol and to understand the durattion of the clinical effect.References:[1]Mirasoglu B, Bagli BS, Aktas S. Hyperbaric oxygen therapy for chronic ulcers in systemic sclerosis - case series. Int J Dermatol. 2017;56(6):636-640.[2]Gerodimos C, Stefanidou S, Kotsiou M, et al. Hyperbaric oxygen treatment of intractable ulcers in a systemic sclerosis patient.Aristotle Un Med J. 2013;(40)3:19-22.[3]Wallace DJ, Silverman S, Goldstein J, Hughes D. Use of hyperbaric oxygen in rheumatic diseases: case report and critical analysis. Lupus. 1995;4(3):172-5.Disclosure of Interests:Slavica Pavlov-Dolijanovic: None declared, Vesna Koletic: None declared, Nada Vujasinovic Stupar: None declared, Nemanja Damjanov Grant/research support from: from AbbVie, Pfizer, and Roche, Consultant of: AbbVie, Gedeon Richter, Merck, Novartis, Pfizer, and Roche, Speakers bureau: AbbVie, Gedeon Richter, Merck, Novartis, Pfizer, and Roche


Diagnostics ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 1880
Author(s):  
Giuseppe Murdaca ◽  
Simone Caprioli ◽  
Alessandro Tonacci ◽  
Lucia Billeci ◽  
Monica Greco ◽  
...  

Introduction: Systemic sclerosis (SSc) is a systemic immune-mediated disease, featuring fibrosis of the skin and organs, and has the greatest mortality among rheumatic diseases. The nervous system involvement has recently been demonstrated, although actual lung involvement is considered the leading cause of death in SSc and, therefore, should be diagnosed early. Pulmonary function tests are not sensitive enough to be used for screening purposes, thus they should be flanked by other clinical examinations; however, this would lead to a risk of overtesting, with considerable costs for the health system and an unnecessary burden for the patients. To this extent, Machine Learning (ML) algorithms could represent a useful add-on to the current clinical practice for diagnostic purposes and could help retrieve the most useful exams to be carried out for diagnostic purposes. Method: Here, we retrospectively collected high resolution computed tomography, pulmonary function tests, esophageal pH impedance tests, esophageal manometry and reflux disease questionnaires of 38 patients with SSc, applying, with R, different supervised ML algorithms, including lasso, ridge, elastic net, classification and regression trees (CART) and random forest to estimate the most important predictors for pulmonary involvement from such data. Results: In terms of performance, the random forest algorithm outperformed the other classifiers, with an estimated root-mean-square error (RMSE) of 0.810. However, this algorithm was seen to be computationally intensive, leaving room for the usefulness of other classifiers when a shorter response time is needed. Conclusions: Despite the notably small sample size, that could have prevented obtaining fully reliable data, the powerful tools available for ML can be useful for predicting early lung involvement in SSc patients. The use of predictors coming from spirometry and pH impedentiometry together might perform optimally for predicting early lung involvement in SSc.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1592.1-1592
Author(s):  
L. Mendez Diaz ◽  
R. J. Gil Velez ◽  
I. Madroñal García

Background:Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis that is associated with early mortality. The development or progression of ILD can occur at any time, so patients should be monitored regularly, particularly in the first years after diagnosis.Treatment should be considered when the disease is clinically significant, particularly when there is evidence of progression based on a decrease in lung function, progression of fibrosis on the HRCT or worsening of respiratory symptoms.Objectives:-To relate the type of systemic sclerosis (SS) with pulmonary involvement with the radiological pattern.-To study if there is a relationship between the antibodies and the aforementioned affectation.Methods:Retrospective descriptive study of patients treated in our Hospital (2009-2019) by the Rheumatology and Internal Medicine department diagnosed with systemic sclerosis and interstitial lung disease.The data were obtained through the review of medical records.We have included data from patients who have a diagnosis of limited or diffuse systemic sclerosis or overlap with interstitial lung involvement.Results:Of the 213 patients with systemic sclerosis in our database 43 had interstitial lung involvement (20.2%). 79% of the patients with ILD (34) had a non-specific interstitial pneumonia type (NSIP) radiological pattern and 21% of the patients (9) had a pattern of usual interstitial pneumonia (UIP)Among the patients with ILD with a NSIP -type radiological pattern, 19 patients were diagnosed with diffuse SS, 9 patients with overlap syndrome and 6 with limited SS.Of the patients with ILD with radiological pattern type UIP, 5 patients were diagnosed with diffuse SS, 3 patients with overlap syndrome and 1 patient was diagnosed with limited SS.TABLE 1.RELATIONSHIP BETWEEN SS TYPE AND RADIOLOGICAL PATTERNNSIPUIPLIMITED SS6 (17.6%)1 (11.1%)DIFFUSE SS19 (55.8%)5 (55.5%)OVERLAP9 (26.4%)3 (33.3%)Among the patients with the NSIP pattern, 17 had positive SCL70 antibody, 3 positive ANA patients and 1 patient had positive anti-centromere antibody.Of the patients with UIP type interstitial pneumopathy, 8 patients had anti-SCL70 antibody, 3 patients ANA positive antibody and 2 patients anti-centromere positive antibody.TABLE 2.RELATIONSHIP BETWEEN TYPE OF AB AND RADIOLOGICAL PATTERNNSIPUIPAnti SCL70178Anti centromere12ANA33Regarding treatment, 21 patients were taking Mycophenolate, 16 patients required cyclophosphamide and 6 patients rituximab.No patient in our cohort died due to interstitial lung disease.Conclusion:The data obtained are consistent with what is collected in the medical literature.The subtype of scleroderma most related to ILD was diffuse SS. The most frequent antibody was anti-SCL 70.Regarding the treatment,the most used in ILD in our center was the mycophenolate.From our sample analyzed when applying the likelihood ratio (RV) a value of 47,186 is obtained, which has an associated probability of 0, which is less than 0.05, leads to reject the null hypothesis (there is no dependence between antibodies and type of radiological pattern of ILD in SS), concluding that there is dependence between the analyzed variables.After this analysis, we can conclude that in our sample there is a relationship between the type of interstitial pneumopathy pattern and the antibody present in patients with SS.Disclosure of Interests:None declared


Sign in / Sign up

Export Citation Format

Share Document