Division Of Immunology Research Articles

Comparative accuracy of CA-153 and KL-6 as diagnostic and prognostic biomarkers for interstitial lung disease

BackgroundsTo discern the potential of KL-6 and CA-153 as diagnostic tools for interstitial lung disease (ILD), understand their relationship with GAP (Gender, Age, and Physiology) stage, and analyze their predictive capacities alongside CT features. This research aims to enhance ILD detection and management in autoimmune patients, emphasizing the diagnostic utility of these biomarkers. MethodsFrom Mar 2017 to Mar 2024, 398 patients from Taichung Veterans General Hospital’s Division of Allergy, Immunology, and Rheumatology with autoimmune diseases were prospectively enrolled. ILD diagnoses were confirmed using High-Resolution Computed Tomography (HRCT) or lung biopsy and characterized by radiologists. GAP scores were calculated for IPF prognosis. 583 serum samples were collected and tested for KL-6, CA-153, CA-199, and CA-125 using specific assays. Statistical analyses compared patients, assessed variables, determined missingness, and predicted ILD, with tools like ROC analysis and logistic regressions. Analyses were performed with IBM SPSS and MedCalc. ResultsILD patients were older, predominantly male, and had more smokers compared to non-ILD. Both KL-6 and CA-153 were higher in ILD and showed a significant, but non-interchangeable correlation. Age, BMI, smoking, and biomarker levels influenced ILD odds, with KL-6 and CA-153 being the strongest predictors. HRCT imaging highlighted these markers’ roles, especially in detecting specific features. Both markers also strongly associated with GAP stages. Stratified analyses emphasized KL-6′s significance in predicting ILD across both AD and non-AD groups. Complete data sensitivity analysis reinforced KL-6 and CA-153 as key ILD predictors. ConclusionsThis research emphasizes CA-153 as a feasible, cost-effective alternative to KL-6 in diagnosing and monitoring ILD. Both CA-153 and KL-6 levels were notably elevated in ILD patients, displaying a strong correlation, especially at CA-153 levels of ≤100 U/ml. They both also have significant associations with CT characteristics and GAP stages. The study reinforces the potential of CA-153, particularly in settings where KL-6 testing might be inaccessible or expensive.

Immuno-Diagnostic Interest in Monitoring CD16+CD56+ (Natural Killer) Cells and CD19+CD45+ (B Lymphocytes) in Individuals Newly Diagnosed with HIV in a Tertiary Care Center.

Monitoring multiple cellular markers of immune cells may provide a more accurate evaluation of the immune status of people living with human immunodeficiency virus (PLHIV). This study assessed the value of CD16+CD56+ cells (NK cells) and CD19+ lymphocytes (B cells) phenotyping in indicating viral load, AIDS status, and treatment efficacy. A retrospective, laboratory-based study was conducted at the Diagnostic immunology division of a referral tertiary hospital. It involved 82 newly diagnosed HIV patients treated between 2009-2016. We explored three objectives: (1) the paired change in CD16+CD56+ and CD19+CD45+ cells counts and percentages from baseline to 2-to-6 months after treatment; (2) the association of these phenotypes with 5 gradual categories of viral load; and (3) the accuracy of CD16+CD56+ and CD19+CD45+ cells counts in indicating AIDS stage defined as CD4+ < 200 cells/mm3. The second and third objectives were tested using a pooled analysis (N = 300-373). The median CD19+CD45+ and CD16+CD56+ counts increased by 1.9-fold and 1.3-fold after treatment respectively (p < 0.001). A negative correlation of viral load with both CD16+CD56+ (ρ = -0.29, p < 0.001) and CD19+CD45+ (ρ = -0.34, p < 0.001) counts was observed. CD16+CD56+ count < 73 cells/mm3 and CD19+CD45+ count < 166.5 were indicative for AIDS with 95.5% and 63.6% sensitivity respectively. Findings advocate for the usefulness of CD16+CD56+ and CD19+CD45+ phenotyping in characterizing the severity of HIV infection and its impact on both the humoral and cellular immunity, as well as monitoring the effectiveness of treatment.

Clinical and laboratory features in health care volunteers with inactivated SARS-CoV-2 vaccination.

To better optimize the inactivated vaccine-induced immune response and improve vaccine protection efficiency, a preliminary study was conducted on the influencing factors of producing neutralizing antibody (NAb) titers against the inactivated coronavirus disease 2019 (COVID-19) vaccine. A total of 91 health care volunteers were enrolled from the Immunology Division of the Laboratory Department of Chongqing General Hospital from February to March 2021. The study had a cross-sectional design. All of the volunteers were scheduled to receive a complete dose regimen of the inactivated severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine and the vaccination interval between 2 doses was 14 days. Clinical and laboratory features were collected for further analysis. The NAb titers gradually increased after COVID-19 vaccination, and 72.53% (n = 66) of the volunteers had NAbs after the second dose. Eight variables, including CD16+CD56+ NK cell level before the first dose (HR = 0.94, p = 0.02), CD16+CD56+ NK cell level after the second dose (HR = 0.94, p = 0.03), interleukin (IL)-2 level before the first dose (HR = 2.09, p = 0.05), mean corpuscular volume (HR = 0.86, p = 0.02), serum urea level (HR = 0.69, p = 0.05), increment of CD19+ B cells (HR = 0.86, p = 0.03), increment of CD4+/CD8+ T cells (HR = 0.21, p = 0.03), and increment of the IL-6 level (HR = 0.75, p = 0.04) demonstrated a correlation with the NAb titers after COVID-19 vaccination. In the multivariate logistical regression analysis, the serum urea level (HR = 2.32, P = 0.03) and increment of CD19+ B cells (HR = 1.96, p = 0.03) were positively correlated with the NAb titers. The principal component analysis effectively distinguished the response after COVID-19 vaccination. The Pearson correlation analysis indicated that the CD19+ B cell level (r = 0.23, p < 0.001) and IL-2 (r = 0.24, p < 0.001) and IL-6 levels (r = 0.22, p < 0.001) were weakly positively correlated with the concentration of NAbs. The NAbs titers of the inactivated vaccines were positively correlated with the ratio of CD19+ B cell, IL-6, and IL-2 levels in the serum, which provide clinical guidance for inactivated SARS-CoV-2 vaccines.

Immune Dysfunction from Radiation Exposure.

The hematopoietic system is highly sensitive to ionizing radiation. Damage to the immune system may result in opportunistic infections and hemorrhage, which could lead to mortality. Inflammation triggered by tissue damage can also lead to additional local or widespread tissue damage. The immune system is responsible for tissue repair and restoration, which is made more challenging when it is in the process of self-recovery. Because of these challenges, the Radiation and Nuclear Countermeasures Program (RNCP) and the Basic Immunology Branch (BIB) under the Division of Allergy, Immunology, and Transplantation (DAIT) within the National Institute of Allergy and Infectious Diseases (NIAID), along with partners from the Biomedical Advanced Research and Development Authority (BARDA), and the Radiation Injury Treatment Network (RITN) sponsored a two-day meeting titled Immune Dysfunction from Radiation Exposure held on September 9-10, 2020. The intent was to discuss the manifestations and mechanisms of radiation-induced immune dysfunction in people and animals, identify knowledge gaps, and discuss possible treatments to restore immune function and enhance tissue repair after irradiation.

Immune Dysfunction from Radiation Exposure.

Exposure to ionizing radiation causes acute damage and loss of bone marrow and peripheral immune cells that can result in high mortality due to reduced resistance to infections and hemorrhage. Besides these acute effects, tissue damage from radiation can trigger inflammatory responses, leading to progressive and chronic tissue damage by radiation-induced loss of immune cell types that are required for resolving tissue injuries. Understanding the mechanisms involved in radiation-induced immune system injury and repair will provide new insights for developing medical countermeasures that help restore immune homeostasis. For these reasons, The Radiation and Nuclear Countermeasures Program (RNCP) and the Basic Immunology Branch (BIB) under the Division of Allergy, Immunology, and Transplantation (DAIT) within the National Institute of Allergy and Infectious Diseases (NIAID) convened a two-day workshop, along with partners from the Biomedical Advanced Research and Development Authority (BARDA), and the Radiation Injury Treatment Network (RITN). This workshop, titled "Immune Dysfunction from Radiation Exposure," was held virtually on September 9-10, 2020; this Commentary provides a high-level overview of what was discussed at the meeting.

Retrospective Study: Management of Atopic Dermatitis

Background: Atopic dermatitis (AD) is a chronic skin inflammatory disease characterized by mild to severe itching, relapses, and mostly appears in infants and children. Although there have been current advances in the management of AD, satisfactory treatment has not been achieved. Purpose: To evaluate the treatment of patients with AD in order to provide better management. Methods: A retrospective study of newly diagnosed AD patients at the Allergy and Immunology Division of the Outpatient Unit, Dermatology and Venereology Clinic, Prof. Dr. R.D. Kandou Hospital Manado from 2019-2021. Result: Antihistamines were the most widely prescribed medication, administered to 108 patients of all patients’ visits, and 94 (87%) of them were given cetirizine (the most widely prescribed type). Emollients were used adequately; they included petroleum jelly in 82 patients (90.1%) and urea 10% cream in 9 patients (9.9%). Conclusion: There were 108 AD patients in Dermatology and Venereology Clinic, Prof. Dr. R.D. Kandou Hospital Manado from 2019-2021. Treatment with antihistamine and emollient therapy gave satisfactory results.

Perspectives of women in science: data sharing in primary immunodeficiency

Jessica Willett Pachul is a Clinical Nurse Specialist in the Division of Immunology &amp; Allergy at the Hospital for Sick Children in Toronto, Canada. In this commentary, she discusses the implications that the collection and sharing of health data can have in the diagnosis and treatment of primary immunodeficiency (PID), as well as in research and development in the field.

Allergic Contact Dermatitis and Orthodontics.

Pediatric Allergy, Immunology, and PulmonologyVol. 36, No. 2 Expert CommentaryAllergic Contact Dermatitis and OrthodonticsErin BantaErin BantaAddress correspondence to: Erin Banta, MD, Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, NYU Langone Hospital-Long Island, NYU Langone Health, 120 Mineola Blvd, Suite 410, Mineola, NY 11501, USA E-mail Address: erin.banta@nyulangone.orgDivision of Rheumatology, Allergy and Immunology, Department of Internal Medicine, NYU Langone Hospital-Long Island, NYU Langone Health, Mineola, New York, USA.Search for more papers by this authorPublished Online:16 Jun 2023https://doi.org/10.1089/ped.2023.0050AboutSectionsView articleView Full TextPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail View article"Allergic Contact Dermatitis and Orthodontics." Pediatric Allergy, Immunology, and Pulmonology, 36(2), pp. 50–51FiguresReferencesRelatedDetails Volume 36Issue 2Jun 2023 InformationCopyright 2023, Mary Ann Liebert, Inc., publishersTo cite this article:Erin Banta.Allergic Contact Dermatitis and Orthodontics.Pediatric Allergy, Immunology, and Pulmonology.Jun 2023.50-51.http://doi.org/10.1089/ped.2023.0050Published in Volume: 36 Issue 2: June 16, 2023Online Ahead of Print:May 30, 2023PDF download

The Second Qatar Allergy and Immunology Conference: An Editorial.

The 2nd Qatar Allergy and Immunology Conference was organized by the Adult Allergy and Immunology Division of Hamad Medical Corporation (HMC) over two days as a hybrid conference on the 10th and 11th of February 2023 at ITQAN Clinical Simulation and Innovation Centre. The conference was preceded by two pre-conference workshops directed toward physicians and nurses on the 9th of February, 2023. The workshops aimed to promote awareness about allergic and immunologic diseases among physicians and nurses. The 2nd Qatar Allergy and Immunology Conference represented a great learning platform for healthcare professionals and opportunities to update knowledge, present research, and meet professionals since allergy and immunology practice is a rapidly evolving science focusing on allergic and immunologic conditions and their advanced treatment options. The conference’s scientific program focused on the following topics: airway allergy and asthma, skin allergies, diagnosis and management of primary immunodeficiency diseases, and novel therapeutics for allergic diseases. One of the significant achievements of this year’s conference is conducting a focused research symposium that provides a flagship platform for medical students and faculty scholars to present their research experiences and up-to-date health topics and to share their experiments and research findings with a broader community. It aimed to gather scientists and physicians from across Qatar and internationally with recent updates regarding the role of precision medicine in allergy and immunology science and practice. HMC supported the event in partnership with the Qatar Allergy and Immunology Society (QAIS) (a chapter of the Qatar Medical Association). It was accredited for 21.25 CPD Category 1 as defined by the Ministry of Public Health’s Department of Healthcare Professions. The conference hosted many expert speakers from Qatar, the Gulf region, and internationally. The total number of participants was 1082, comprised of physicians, nurses, and scientists. We received 34 abstracts, 32 were accepted for poster presentations, and the best six were selected for the oral presentation session. Another achievement of our conference is advocating the Arabic language. One lecture and one oral abstract were presented in Arabic to demonstrate the importance and simplicity of using the Arabic language in medical education and science.

Safety of biologicals in severe asthma patients having COVID-19.

Pediatric PulmonologyEarly View CLINICAL CORRESPONDENCE Safety of biologicals in severe asthma patients having COVID-19 Öner Özdemir MD, Corresponding Author Öner Özdemir MD [email protected] orcid.org/0000-0002-5338-9561 Department of Pediatrics, Division of Allergy and Immunology, Research and Training Hospital of Sakarya University Medical Faculty, Adapazarı, Sakarya, Türkiye Correspondence Öner Özdemir, MD, Division of Allergy and Immunology, Department of Pediatrics, Faculty of Medicine, Sakarya University, Research and Training Hospital of Sakarya University, Adnan Menderes Cad., Sağlık Sok., No: 195, Adapazarı, Sakarya, Türkiye. Email: [email protected] Contribution: Conceptualization, ​Investigation, Writing - original draft, Writing - review & editing, Methodology, SupervisionSearch for more papers by this author Öner Özdemir MD, Corresponding Author Öner Özdemir MD [email protected] orcid.org/0000-0002-5338-9561 Department of Pediatrics, Division of Allergy and Immunology, Research and Training Hospital of Sakarya University Medical Faculty, Adapazarı, Sakarya, Türkiye Correspondence Öner Özdemir, MD, Division of Allergy and Immunology, Department of Pediatrics, Faculty of Medicine, Sakarya University, Research and Training Hospital of Sakarya University, Adnan Menderes Cad., Sağlık Sok., No: 195, Adapazarı, Sakarya, Türkiye. Email: [email protected] Contribution: Conceptualization, ​Investigation, Writing - original draft, Writing - review & editing, Methodology, SupervisionSearch for more papers by this author First published: 10 May 2023 https://doi.org/10.1002/ppul.26450Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation

Editorial introductions.

Editorial introductions.

Can Human Milk Oligosaccharides Modulate the Allergic Response and Improve the Management of Cow’s Milk Protein Allergy?

During this symposium, leading experts in paediatric allergy and immunology reviewed new evidence for the role of human milk oligosaccharides (HMO) in supporting the development of the infant microbiota and modulating the immune system, thereby improving the clinical management of cow’s milk protein allergy (CMPA). Liam O’Mahony, University College Cork, Ireland, explored the mechanisms by which HMOs can modify the gut microbiome and beneficially influence allergic and infectious responses in both healthy infants and those with CMPA. New data from the CINNAMON study were showcased by Claire Boulangé, Nestlé Institute of Health Sciences, Lausanne, Switzerland, highlighting key mechanisms by which specific HMOs can support the microbiome and modulate metabolome production that may lead to important immune benefits in CMPA. Finally, Anna Nowak-Węgrzyn, Professor of Pediatrics and Chief of the Division of Pediatric Allergy and Immunology at the Grossman School of Medicine, New York University (NYU) Langone Health, USA, presented results from the Platypus study, in which infants with moderate-to-severe CMPA were fed an amino acid (AA)-based formula containing two HMOs. Symptoms of CMPA decreased significantly in infants fed the HMO-supplemented formula, and these clinical improvements were accompanied by normal growth and positive changes to the faecal microbiome. Collectively, these findings translate to important immune benefits and a key role for HMO-supplemented formula in the clinical management of CMPA.

A Personal Perspective on My Scientific Career

A Personal Perspective on My Scientific Career

Expanding spectrum, intrafamilial diversity, and therapeutic challenges from 15 patients with heterozygous CARD11-associated diseases: A single center experience.

CARD11-associated diseases are monogenic inborn errors of immunity involving immunodeficiency, predisposition to malignancy and immune dysregulation such as lymphoproliferation, inflammation, atopic and autoimmune manifestations. Defects inCARD11 can present as mutations that confer a complete or a partial loss of function (LOF) or contrarily, a gain of function (GOF) of the affected gene product. We report clinical characteristics, immunophenotypes and genotypes of 15 patients from our center presenting with CARD11-associated diseases. Index cases are pediatric patients followed in our immunology division who had access to next generation sequencing studies. Variant significance was defined by functional analysis in cultured cells transfected with a wild type and/or with mutated hCARD11 constructs. Cytoplasmic aggregation of CARD11 products was evaluated by immunofluorescence. Nine index patients with 9 unique heterozygous CARD11 variants were identified. At the time of the identification, 7 variants previously unreported required functional validation. Altogether, four variants showed a GOF effect as well a spontaneous aggregation in the cytoplasm, leading to B cell expansion with NF-κB and T cell anergy (BENTA) diagnosis. Additional four variants showing a LOF activity were considered as causative of CARD11-associated atopy with dominant interference of NF-kB signaling (CADINS). The remaining variant exhibited a neutral functional assay excluding its carrier from further analysis. Family segregation studies expanded to 15 individuals the number of patients presenting CARD11-associated disease. A thorough clinical, immunophenotypical, and therapeutic management evaluation was performed on these patients (5 BENTA and 10 CADINS). A remarkable variability of disease expression was clearly noted among BENTA as well as in CADINS patients, even within multiplex families. Identification of novel CARD11 variants required functional studies to validate their pathogenic activity. In our cohort BENTA phenotype exhibited a more severe and expanded clinical spectrum than previously reported, e.g., severe hematological and extra hematological autoimmunity and 3 fatal outcomes. The growing number of patients with dysmorphic facial features strengthen the inclusion of extra-immune characteristics as part of the CADINS spectrum. CARD11-associated diseases represent a challenging group of disorders from the diagnostic and therapeutic standpoint, especially BENTA cases that can undergo a more severe progression than previously described.

Using the Electronic Medical Record to Increase Laboratory Test Monitoring in Ocular Inflammation Patients: A Quality Improvement Study.

Treatment of chronic, noninfectious ocular inflammation includes corticosteroids, disease-modifying antirheumatic medications, and biologics. To mitigate adverse effects associated with the use of these medications, routine laboratory test monitoring is recommended throughout treatment. We evaluated the effectiveness of an alert added to the electronic medical record (EMR) to aid in laboratory test monitoring for patients prescribed these high-risk medications. A prospective, interventional study assessed the effect of the alert within the EMR on laboratory test ordering at the Division of Ocular Immunology at the Wilmer Eye Institute. The primary outcome measure was the change in number of ordered laboratory tests at 3, 6, and 12 months after the alert activation compared with pre-intervention levels and overall through the study period. The laboratory tests that were monitored included complete blood count, comprehensive metabolic panel, dual-energy x-ray absorptiometry (DXA) scanning, fasting lipid panel, and interferon gamma release assays. The laboratory test orders for 153 patients on high risk medications were analyzed. Only the frequency of ordering the DXA and interferon gamma release assays increased significantly, compared with baseline, throughout the study. Conversely, there was a significant decrease in the frequency of ordering of fasting lipid profiles and hemoglobin A 1c at each time point and for complete blood count and comprehensive metabolic panel at the 6-month time point. An EMR alert results in increased laboratory test ordering initially for tests drawn on a yearly basis, but the effect on more frequently ordered tests wanes with time if the alert can be silenced by the provider. Nonetheless, it provides a novel mechanism to increase laboratory ordering in patients on high-risk medications that can be adapted for use in other EMR software. Future studies are needed to assess whether physician laboratory test ordering behavior is altered throughout the study period with the use of a non-silencable alert.

Editorial introductions.

Editorial introductions.

Impact of extramural DAIT/NIAID pharmacy programs, research pharmacist scientist oversight on study performance, and lessons learned

PurposeOver the past two decades, the involvement of a Pharmacist Scientist in clinical settings has improved patient safety, decreased medication errors, and enabled successful conduct of clinical trials and faster product development [1–5]. The impact of an oversight by a Pharmacist Scientist on clinical trial performance and execution in terms of Pharmacy and Investigational Product (IP)-related deviations has not been evaluated by a sponsor. MethodsThis was a retrospective observational study conducted by the Division of Allergy, Immunology and Transplantation (DAIT), National Institute of Allergy and Infectious Diseases (NIAID). We assessed the association of the number of Pharmacy and Investigational Product (IP)-related deviations with Pharmacist oversight and use of DAIT Pharmacy/ Pharmaceutical services in two groups: Intervention Group (IG) and the Control Group (CG). ResultsWe evaluated monitoring data from 116 studies conducted between 2006 through 2020. Forty-one eligible clinical trials were included and analyzed: 13 trials were in the IG with Pharmacist oversight and use of Pharmacy Services; 28 trials were in the CG with no Pharmacist oversight and zero to partial use DAIT Pharmacy/ Pharmaceutical Services. The evaluation revealed the expected risk of having a pharmacy and IP-related deviations were 2.94 times higher (95% CI 1.28, 6.67, = 0.01) in trials not having Pharmacist oversight and zero to partial use of Pharmaceutical/ Pharmacy Program services. This significant finding was associated with having Pharmacist oversight when adjusting for study size (# of sites and patients needed), anticipated study duration, design complexity, and whether recruitment was completed or not. ConclusionWe found a statistically significant association between Pharmacist Scientist involvement and oversight from protocol development to study execution and a reduction in Pharmacy and IP-related deviations.

Risk factors of acute and chronic urticaria in dermatology and venereology outpatient clinic Dr. Soetomo General Academic Teaching Hospital, Surabaya, Indonesia

Urticaria is a skin disorder characterized by localized redness or edema of the mucosa followed by an itching sensation that subsides within a day. Proper treatment not only requires pharmacological treatment but also preventive measures to reduce the impact of risk factors that can worsen the patient's urticaria complaints. This study is an observational study with a retrospective approach that aims to evaluate the risk factors of acute and chronic urticaria patients at Allergy Immunology Division Dermatovenereology Outpatient Clinic Dr. Soetomo General Academic Teaching Hospital, Surabaya, Indonesia from 2018 to 2020. This study found 179 new urticaria patients with the number of acute urticaria patients 114 patients (63.7%) and chronic urticaria 65 patients (36.3%). The analysis was conducted based on the characteristics of the samples gender, age, atopic history with a p-value&gt;0.05. There was a statistically significant correlation between Body Mass Index (BMI) (p=0.003). The multivariable logistic regression analysis showed BMI (OR=1.16; 95%CI=1.05-1.27; p=0.002) and history of allergic rhinitis (OR=4.32; 95%CI=1.25-14.86; p=0.020) were factors that played a significant role in the incidence of chronic urticaria.

The Preventive Role of Physical Activity in Systemic Sclerosis: A Cross-Sectional Study on the Correlation with Clinical Parameters and Disease Progression.

Although exercise is associated with improved health in many medical conditions, little is known about the possible influences of physical activity (PA) habits pre- and post- a diagnosis of systemic sclerosis (SSc) on disease activity and progression. This cross-sectional study assessed, for the first time, self-reported pre- and post-diagnostic PA levels with the aim to verify if changes in these levels were correlated with demographic/anthropometric data (e.g., weight, height, gender, age, BMI), disease duration, diagnostic/clinical parameters (e.g., skin involvement, pulmonary hemodynamic/echocardiographic data, disease activity) related to disease activity and progression, and quality of life in a population-based sample of patients with SSc. Adult participants (n = 34, age 56.6 ± 13.3 years) with SSc (limited cutaneous SSc, lcSSc, n = 20; diffuse cutaneous SSc, dcSSc, n = 9; sine scleroderma SSc, n = 5) were enrolled at the Division of Rheumatology and Clinical Immunology of the Humanitas Research Hospital. All medical data were recorded during periodic clinical visits by a rheumatologist. Moreover, all subjects included in this study completed extensive questionnaires to evaluate their health-related quality of life (HRQOL), and others related to health-related physical activity performed before (PRE) and after (POST) the diagnosis of disease. The linear regression analysis has shown that either a high Sport_index or Leisure_index in the PRE-diagnostic period was correlated with lower disease duration in dcSSc patients. Physical load during sport activity and leisure time accounted for ~61.1% and ~52.6% of the individual variation in disease duration, respectively. In lcSSc patients, a high PRE value related to physical load during sporting activities was correlated with a low pulmonary artery systolic pressure (sPAP) and the POST value of the Work_index was positively correlated with the left ventricular ejection fraction (LVEF), and negatively with creatine kinase levels (CK). Interestingly, the univariate analysis showed that Work_index accounts for ~29.4% of the variance in LVEF. Our analysis clearly reinforces the concept that high levels of physical load may play a role in primary prevention—delaying the onset of the disease in those subjects with a family history of SSc—as well as in secondary prevention, improving SSc management through a positive impact on different clinical parameters of the disease. However, it remains a priority to identify a customized physical load in order to minimize the possible negative effects of PA.

Editorial introductions.

Editorial introductions.