Introduction And State Of The Art: Systemic lupus erythematosus (SLE) is an autoimmune disease that affects many organs throughout its course, most frequently the joints, skin and kidneys. Both the central (CNS) and peripheral (PNS) nervous systems are also often affected. T he involvement of the CNS has a negative prognosis in lupus patients. Neurological symptoms are diverse, from headaches and cognitive dysfunction to life-threatening seizures or stroke. Due to the great diversity of neurological presentations, diagnosing neuropsychiatric SLE (NPSLE, neurolupus) can be challenging and necessitates a careful differential diagnostic work-up. Furthermore, neurological symptoms can be one of the first signs of the disease, making the correct diagnosis even more challenging. White matter lesions in NPSLE may closely resemble lesions formed during multiple sclerosis (MS), which is a chronic autoimmune disease of the CNS resulting in neuroinflammatory damage to the myelin sheath, axonal impairment, and neurodegeneration. Based on imaging only, it is challenging to differentiate between the two diseases.
Clinical Implications: While both diseases have characteristic features, in their early stages they may mimic each other. The purpose of this literature review was to emphasise the differences in clinical, immunological and neuroimaging features between the two diseases in order to facilitate diagnosis, highlighting the most useful diagnostic tools.
Future Directions: Prompt and accurate diagnosis is crucial for implementing appropriate, disease-specific treatment and thereby improving the prognosis for the patient. Therefore, there is a need for novel imaging and laboratory biomarkers, possibly used as a multifactorial profile, to differentiate NPSLE from MS.
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http://dx.doi.org/10.5603/pjnns.103538 | DOI Listing |
Pediatr Allergy Immunol Pulmonol
January 2025
Clinical Immunology Unit, Faculty of Medicine and Health Sciences, Department of Paediatrics, Universiti Putra Malaysia, Selangor, Malaysia.
: RAS guanyl-releasing protein 1 (RASGRP1) deficiency is characterized by immune dysregulation and Epstein-Barr virus (EBV)-related lymphoproliferation. Diffuse mesangial sclerosis is one of the infrequent causes of infantile nephrotic syndrome. : Here, we described a 7-year-old girl who was diagnosed with diffuse mesangial sclerosis at 5 months old and subsequently developed chronic bilateral neck swelling at the age of 3 years.
View Article and Find Full Text PDFClin Rev Allergy Immunol
December 2024
Department of Otolaryngology-Head and Neck Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Olfactory dysfunction (OD) can have serious consequences as it hinders individuals from detecting important warning signals like smoke, spoiled food, and gas leaks. This can significantly impact their nutritional status, eating satisfaction, and overall quality of life. Allergic rhinitis (AR) is a common disease that greatly affects the quality of life and can lead to a decrease, distortion, or complete loss of olfactory ability.
View Article and Find Full Text PDFJ Clin Immunol
January 2025
Division of Allergy and Immunology, Children's Hospital of Philadelphia, Philadelphia, 19104, USA.
Major histocompatibility complex class I deficiency results from deleterious biallelic variants in TAP1, TAP2, TAPBP, and B2M genes. Only a few patients with variant-curated TAP1 deficiency (TAP1D) have been reported in the literature and the clinical phenotype has been variable with an emphasis on autoimmune and inflammatory complications. We report TAP1D in a Nepalese girl with a severe clinical phenotype with serious viral infections at a very young age.
View Article and Find Full Text PDFClin Rev Allergy Immunol
December 2024
Division of Allergy and Clinical Immunology, The Johns Hopkins Asthma & Allergy Center, Johns Hopkins University School of Medicine, 5501 Hopkins Bayview Circle, Room 3B.71, Baltimore, MD, 21224, USA.
Asthma is a chronic airway inflammatory disease that affects millions globally. Although glucocorticoids are a mainstay of asthma treatment, a subset of patients show resistance to these therapies, resulting in poor disease control and increased morbidity. The complex mechanisms underlying steroid-resistant asthma (SRA) involve Th1 and Th17 lymphocyte activity, neutrophil recruitment, and NLRP3 inflammasome activation.
View Article and Find Full Text PDFAnn Hematol
January 2025
Department of Hematology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, 325035, China.
Background: Autoimmune hemolytic anemia (AIHA) following allogeneic hematopoietic stem cell transplantation (allo-HSCT) is often refractory and relapsing, leading to increased mortality post-HSCT.
Methods: We retrospectively analyzed the cases of patients with transfusion-dependent β-thalassemia (TDT) who underwent allo-HSCT to study their clinical features, the occurrence of AIHA post-HSCT, and treatment response and to explore the possible pathogenesis of AIHA.
Result: A total of 113 patients were registered in the study, out of whom 14 developed AIHA following allo-HSCT, resulting in a cumulative incidence of 12.
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