Isolated acute lupus pneumonitis as the initial presentation of systemic lupus erythematosus in an 8-year-old girl

AbstractSystemic lupus erythematosus (SLE) is a systemic autoimmune disease which has broad pleuropulmonary manifestations. One of the rare and mortal complications is acute lupus pneumonitis, which is reported very rarely, especially in childhood. Herein, we report an 8-year-old girl with isolated acute lupus pneumonitis as the initial presentation that required a lung biopsy for diagnosis. Although she had improvement with the administration of steroids, steroid treatment was reduced due to the drug ’s side effects resulting in the addition of azathioprine and mycophenolate mofetil to the treatment regimen. After the new regimen failed to result in clinical improvement, hydroxychloroquine treatment was started and a significant improvement was observed. Acute lupus pneumonitis is an uncommon manifestation of SLE and diagnosis may be difficult in patients without other organ involvement.
Source: Autoimmunity Highlights - Category: Allergy & Immunology Source Type: research

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Authors: Zhang R, Li Y, Pan B, Li Y, Liu A, Li X Abstract Systemic lupus erythematosus (SLE) is a prototypical autoimmune disease, characterized by overactive inflammation and aberrant activation of lymphocytes. Chemokine C-X-C motif ligand 10 (CXCL10) has an important role in the initiation and deterioration of SLE. However, the expression levels of CXCL10 mRNA in T-helper (Th) cells and B lymphocytes from SLE patients have remained elusive. In the present study, a Bioinformatics analysis of differentially expressed gene (DEG) profiles obtained from RNA sequencing data for three matched samples was performed to ex...
Source: Experimental and Therapeutic Medicine - Category: General Medicine Tags: Exp Ther Med Source Type: research
ConclusionsThe mutant A20 may play a role in weakening the tight junction of the blood-brain barrier to cause neurologic symptoms. We report a rare variant ofTNFAIP3 in a patient with NPSLE and reveal its autoimmune disease –causing mechanism in both peripheral tissues and the central nervous system.
Source: Journal of Clinical Immunology - Category: Allergy & Immunology Source Type: research
Abstract Several reports have highlighted the abnormal increments of serum immunoglobulin free light chains (FLC) in the course of systemic autoimmune rheumatic diseases (SARD) but a comparative analysis among different conditions is still lacking. A strong association between elevated FLC and HCV-related mixed cryoglobulinaemia (HCVMC) has been well established. Here, we aimed to analyze serum FLC levels in patients with four different SARDs in comparison with HCVMC. Using a turbidimetric assay, free k and λ chains were quantified in sera from 198 SARD patients (37 Rheumatoid Arthritis, RA; 47 Systemic Lup...
Source: Clinical and Developmental Immunology - Category: Allergy & Immunology Authors: Tags: Clin Exp Immunol Source Type: research
Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by increased production of autoantibodies, which commonly target nuclear antigens, and concomitant deposition of immune complexes that cause inflammation in tissues. SLE is often associated with increased systemic expression of type I interferons, in some cases due to dysregulation in nucleic acid-sensing innate pathways. There is strong genetic evidence for a link between cytoplasmic RNA sensing pathways (RIG-I/MDA5) and SLE, both in human patients and murine models, however questions still remain regarding pathway initiation, cell types involved a...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
AbstractAryl hydrocarbon receptor (AhR), a type of transcriptional factor, is widely expressed in immune cells. The activation of AhR signaling pathway depends on its ligands, which exist in environment and can also be produced by metabolism. Normal expressions of AhR and AhR-mediated signaling may be essential for immune responses, and effects of AhR signaling on the development and function of innate and adaptive immune cells have also been revealed in previous studies. Recent studies also indicate that aberrant AhR signaling may be related to autoimmune diseases, including rheumatoid arthritis (RA), systemic lupus eryth...
Source: Inflammopharmacology - Category: Drugs & Pharmacology Source Type: research
FcγRIIb is the only inhibitory Fc receptor and controls many aspects of immune and inflammatory responses. The observation 19 years ago that FcγRIIb−/− mice generated by gene targeting in 129 derived ES cells developed severe lupus like disease when backcrossed more than 7 generations into C57BL/6 background initiated extensive research on the functional understanding of this strong autoimmune phenotype. The genomic region in the distal part of Chr1 both in human and mice in which the FcγR gene cluster is located shows a high level of complexity in relation to the susceptibility to SLE. Specif...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Abstract Inflammasomes are a specialized group of intracellular sensors that are key components of the host innate immune system. Autoinflammatory diseases are disorders of the innate immune system that are characterized by recurrent inflammation and serious complications. Dysregulation of the inflammasome is associated with the onset and progression of several autoinflammatory and autoimmune diseases, including cryopyrin-associated periodic fever syndrome, familial Mediterranean fever, rheumatoid arthritis, and systemic lupus erythematosus. In this review, we discuss the involvement of various inflammasome compon...
Source: Journal of Leukocyte Biology - Category: Hematology Authors: Tags: J Leukoc Biol Source Type: research
In conclusion, a polypharmacology approach of combining established, prolongevity drug inhibitors of specific nodes may be the most effective way to target the nutrient-sensing network to improve late-life health. Deletion of p38α in Neurons Slows Neural Stem Cell Decline and Loss of Cognitive Function in Mice https://www.fightaging.org/archives/2019/10/deletion-of-p38%ce%b1-in-neurons-slows-neural-stem-cell-decline-and-loss-of-cognitive-function-in-mice/ Researchers here provide evidence for p38α to be involved in the regulation of diminished neural stem cell activity with age. It is thought...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Sterile inflammation arises without external cause, such as infection or injury, and chronic sterile inflammation is a characteristic of aging. Inflammatory signaling becomes constant and pronounced in tissues, and the immune system is constantly roused to action. Processes, such as regeneration from injury, that depend upon a clear cycle of inflammation that starts, progresses, and resolves are significantly disrupted. It is no exaggeration to say that the downstream consequences of chronic inflammation accelerate the progression of all of the common age-related conditions. It is of great importance in atherosclerosis and...
Source: Fight Aging! - Category: Research Authors: Tags: Medicine, Biotech, Research Source Type: blogs
ConclusionsThis study is first to demonstrate that X-chromosome-linked IRAK1 polymorphisms are associated with the risk of NMOSD and provide novel insights into the underlying mechanisms of this disease. Further studies are needed to elucidate the function of IRAK1 variants in the pathogenesis of NMOSD and the underlying molecular mechanisms.
Source: Multiple Sclerosis and Related Disorders - Category: Neurology Source Type: research
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