Lung Disease in Sjögren's Syndrome: Insights From a Reference Center's Population.

Background Sjögren's syndrome (SS) is a chronic systemic autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands. Although primarily affecting these glands, the syndrome can also involve several organs. Interstitial lung disease is one of the most severe complications associated with SS.

Psychological impact of life events in systemic lupus erythematosus patients - Differences between flares and remission.

Background: Stress has been linked to worsening symptoms and increased disease activity in patients with Systemic lupus erythematosus (SLE). Life-events are individual stress points, and there is conflicting evidence regarding their role in SLE activity and disease perception.

Methods: Adult SLE patients were recruited for the study.

Idiopathic inflammatory myopathies - The burden of disease: Cohort analysis focusing on damage and comorbidities.

Introduction/background: Idiopathic Inflammatory Myopathies (IIM) continue to be a major clinical challenge worldwide. The exact aetiopathogenesis of this chronic and disabling disease remains elusive, preventing the development of novel and effective therapeutic strategies and leading to a high incidence of damage. The complexity of treating these diseases is even greater due to the numerous comorbidities that affect these patients.

Multiple autoimmune syndrome: Clinical, immunological and genotypic characterization.

Introduction: The existence of subphenotypes common to several autoimmune diseases (AIDs) suggests a shared physiopathology - autoimmune tautology. Multiple Autoimmune Syndrome (MAS) - the coexistence of three or more AIDs in one person-, best illustrates that polyautoimmunity is more than a coincidence.

Objectives: Characterize and compare the monoautoimmune and MAS patients.

Aortic Dissection in a Patient with Novel Frameshift COL5A1 Variant of Classical Ehlers-Danlos Syndrome.

Unlabelled: Classical Ehlers-Danlos syndrome (cEDS) is one of the 13 subtypes of Ehlers-Danlos syndrome, which has the major clinical criteria of hyperextensibility skin, atrophic scars, and generalised joint hypermobility. The occurrence of aortic dissection has been described in some subtypes of Ehlers-Danlos, but it has a rare association with the cEDS subtype. This case report discusses a 39-year-old female with a past medical history of transposition of great arteries with a Senning repair at the age of 18 months and controlled hypertension with medication, who presents a spontaneous distal aortic dissection.

Not so Mutually Exclusive Diseases: A Case of Co-occurrence of Inflammatory Spondyloarthritis and Diffuse Skeletal Hyperostosis in a Young Patient.

Unlabelled: Diffuse idiopathic skeletal hyperostosis (DISH) and axial spondyloarthritis (axial SpA) are differential diagnoses of lower back pain. While the latter is considered to be an inflammatory disease, DISH is thought to be a metabolic condition. The authors report a case of a 34-year-old man who presented with a one-year history of axial lower back pain associated to migratory polyarthritis, buttock and heel pain.

Sjögren's syndrome and pregnancy: a Portuguese case-control study.

Introduction: Pregnancy in patients with autoimmune disorders is associated with an increased risk of adverse outcomes. Sjögren's syndrome (SS) is one of the most common among autoimmune diseases. Presently data regarding the impact of SS on obstetric outcomes are scarce and inconclusive.

Pregnancy and lactation-associated osteoporosis in a Systemic Lupus Erythematosus patient.

Pregnancy and lactation-associated osteoporosis (PLO) is a rare disease that occurs in late pregnancy or early postpartum and is associated with multiple vertebral fractures. We present a case of a 34-year-old woman with a history of Systemic Lupus Erythematosus and Antiphospholipid Syndrome, who started postpartum back pain. After an ineffective response to analgesic escalation, she performed imaging exams with evidence of multiple dorsal and lumbar vertebral fractures.

Predictive Factors of Severe Behçet's disease: A Longitudinal, Prospective Cohort Followed Between 1981-2020.

Introduction: Behçet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described.

Prevalence of Primary Biliary Cholangitis in a Cohort of Primary Sjögren's Syndrome Patients.

Objective To analyze the prevalence and clinical progression of primary biliary cholangitis (PBC) in patients with primary Sjögren's syndrome (pSS) and possible associations between biochemical and immunological features and the development of PBC. Methods We retrospectively reviewed a cohort of 115 pSS patients followed up in an outpatient clinic from 1987 to 2020, without a history of liver disease, and looked for the presence of PBC through analysis of several biochemical, immunological, and histologic characteristics. Results Twenty patients (17.

Refractory Orbital Myositis in Systemic Lupus Erythematosus A Role for Rituximab.

Unlabelled: Orbital myositis in systemic lupus erythematosus (SLE) is a rare entity with risk of serious complications. Timely treatment with effective immunosuppressors is desirable. We report a case of a 32-year-old female patient with SLE who presented with an acute ocular pain and extraorbital muscle thickening, consistent with orbital myositis.

Systemic lupus erythematosus and pregnancy: A retrospective single-center study of 215 pregnancies from Portugal.

Objective: Systemic lupus erythematosus (SLE) is a life-threatening disorder that affects women at reproductive age. We evaluate the clinical impact of pregnancy in a cohort of Portuguese SLE patients and the risk factors associated with maternal and fetal adverse outcomes.

Methods: A retrospective observational study that included all pregnant women with SLE managed at a Portuguese tertiary hospital, between January 1993 and December 2019.

Systemic Lupus Erythematosus and Pregnancy: a Portuguese Case-Control Study.

Pregnancy in systemic lupus erythematosus (SLE) patients is associated with an increased risk of adverse outcomes. During pregnancy, SLE patients have a higher rate of miscarriage, stillbirth, preterm delivery, fetal growth restriction, or hypertensive disorders of pregnancy. To date, only a few case-control studies were published with the purpose to evaluate the magnitude of risk associated with pregnancy in lupus patients.

Predictive Factors of Severe Behçet's disease: A Longitudinal, Prospective Cohort Followed Between 1981-2020.

Introduction: Behçet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described.

Evaluating the potential of adaptive comfort approach using historic data to reduce energy consumption in buildings in southern Spain.

The application of adaptive comfort models is among the determinant factors to reduce greenhouse gas emissions in the building sector. This research studies the region of Andalusia (south of Spain). A cluster analysis is applied to 786 Andalusian municipalities, and 4 groups are established according to the potential of adaptive strategies.

Eosinophilic fasciitis (Shulman's disease): review and comparative evaluation of seven patients.

Objectives: Eosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and express fibrogenic cytokines including the transforming factor of tumor growth a and b and interleukins 1 and 6. The purpose of this study was to summarize seven cases of EF in a central hospital.

Tuberculous osteomyelitis of the wrist presenting as a monoarthritis syndrome.

Osteoarticular tuberculosis is the third most frequent location of tuberculosis after the lung and lymph nodes, accounting for approximately 10%-20% of all cases of extrapulmonary disease. Tuberculosis of the hand and wrist is the rarest osteoarticular location after the shoulder. The authors report the case of a 50-year-old woman without medical history who was diagnosed with isolated tuberculosis of the wrist presenting as monarthritis.

Vitamin D supplementation effects on FoxP3 expression in T cells and FoxP3/IL-17A ratio and clinical course in systemic lupus erythematosus patients: a study in a Portuguese cohort.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with multi-organ inflammation, linked to loss of immune tolerance to self-antigens and the production of a diversity of autoantibodies, with a negative impact on the patients' quality of life. Regulatory T cells have been reported as deficient in number and function in SLE patients. However, some authors also described an enrichment of this cell type.

The Protective Role of HLA-DRB1(∗)13 in Autoimmune Diseases.

Autoimmune diseases (AIDs) are characterized by a multifactorial aetiology and a complex genetic background, with the MHC region playing a major role. We genotyped for HLA-DRB1 locus 1228 patients with AIDs-213 with Systemic Lupus Erythematosus (SLE), 166 with Psoriasis or Psoriatic Arthritis (Ps + PsA), 153 with Rheumatoid Arthritis (RA), 67 with Systemic Sclerosis (SSc), 536 with Multiple Sclerosis (MS), and 93 with Myasthenia Gravis (MG) and 282 unrelated controls. We confirmed previously established associations of HLA-DRB1(∗)15 (OR = 2.

Frequent involvement of central nervous system in primary Sjögren syndrome.

Primary Sjögren syndrome (pSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the salivary and tear glands, and autoantibody secretion, in the absence of other systemic autoimmune disorder. Among autoimmune diseases, it is a relatively common disease, but the burden of central nervous system (CNS) involvement is controversial. This retrospective study evaluates the prevalence, clinical patterns and outcomes of CNS involvement in a cohort of patients with primary Sjögren syndrome.

Neurological involvement in Primary Sjögren Syndrome.

Objectives: To perform an observational retrospective cross-sectional case-control study to evaluate prevalence, clinical patterns and outcomes of neurological involvement in a cohort of Primary Sjögren Syndrome (pSS) patients followed up in a single center.

Material And Methods: From a total of 93 pSS patients, diagnosed according to the 2002 criteria proposed by the American-European Consensus Group, we reviewed the clinical data of those with neurological complaints that were referred to observation by Neuroimmunology doctors. Demographic, clinical, seroimmunological data were compared between patients with and without neurological involvement.

Systemic lupus erythematosus, progressive multifocal leukoencephalopathy, and T-CD4+ lymphopenia.

Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection caused by the reactivation of JC virus and occurs in patients with severe primary or secondary immunosuppression. Recently, PML is becoming relevant in autoimmune disorders, particularly in patients treated with biologic agents. However, systemic lupus erythematosus (SLE) appears to be associated with susceptibility to PML that cannot be entirely explained by the immunosuppressive therapy.