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Acta Bio-medica : Atenei Parmensis Dec 2019The mesenteric panniculitis is a rare form of inflammation that mainly involves the mesenteric adipose tissue. The etiology remains unknown and the disease has been... (Review)
Review
The mesenteric panniculitis is a rare form of inflammation that mainly involves the mesenteric adipose tissue. The etiology remains unknown and the disease has been associated with various conditions such as cancer, abdominal trauma, previous surgery, autoimmune diseases and obesity. Mesenteric panniculitis can be divided into two main groups: the mesenteric panniculitis with only the inflammation and degeneration of the mesenteric fat, and the retractile panniculitis, mainly fibrotic, with retraction of the surrounding structures. From a radiological point of view, there are two main signs: the fat ring sign, which is the presence of normal fat around vessels and lymph nodes, and the pseudocapsula around the lesion. In this paper, we present the imaging and clinical features of mesenteric panniculits with particular reference to the differential diagnosis and the possible etiological associations. (www.actabiomedica.it).
Topics: Humans; Magnetic Resonance Imaging; Panniculitis, Peritoneal; Tomography, X-Ray Computed
PubMed: 31910164
DOI: 10.23750/abm.v90i4.7696 -
Orphanet Journal of Rare Diseases Jul 2018Alpha-1 antitrypsin deficiency (AATD) is a rare hereditary condition that leads to decreased circulating alpha-1 antitrypsin (AAT) levels, significantly increasing the... (Review)
Review
BACKGROUND
Alpha-1 antitrypsin deficiency (AATD) is a rare hereditary condition that leads to decreased circulating alpha-1 antitrypsin (AAT) levels, significantly increasing the risk of serious lung and/or liver disease in children and adults, in which some aspects remain unresolved.
METHODS
In this review, we summarise and update current knowledge on alpha-1 antitrypsin deficiency in order to identify and discuss areas of controversy and formulate questions that need further research.
RESULTS
1) AATD is a highly underdiagnosed condition. Over 120,000 European individuals are estimated to have severe AATD and more than 90% of them are underdiagnosed.
CONCLUSIONS
2) Several clinical and etiological aspects of the disease are yet to be resolved. New strategies for early detection and biomarkers for patient outcome prediction are needed to reduce morbidity and mortality in these patients; 3) Augmentation therapy is the only specific approved therapy that has shown clinical efficacy in delaying the progression of emphysema. Regrettably, some countries reject registration and reimbursement for this treatment because of the lack of larger randomised, placebo-controlled trials. 4) Alternative strategies are currently being investigated, including the use of gene therapy or induced pluripotent stem cells, and non-augmentation strategies to prevent AAT polymerisation inside hepatocytes.
Topics: Animals; Fibrosis; Humans; Panniculitis; Pulmonary Disease, Chronic Obstructive; Vasculitis; alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency
PubMed: 29996870
DOI: 10.1186/s13023-018-0856-9 -
The Journal of Nutritional Biochemistry Aug 2018Strategies to reduce obesity have become public health priorities as the prevalence of obesity has risen in the United States and around the world. While the... (Review)
Review
Strategies to reduce obesity have become public health priorities as the prevalence of obesity has risen in the United States and around the world. While the anti-inflammatory and hypotriglyceridemic properties of long-chain omega-3 polyunsaturated fatty acids (n-3 PUFAs) are well known, their antiobesity effects and efficacy against metabolic syndrome, especially in humans, are still under debate. In animal models, evidence consistently suggests a role for n-3 PUFAs in reducing fat mass, particularly in the retroperitoneal and epididymal regions. In humans, however, published research suggests that though n-3 PUFAs may not aid weight loss, they may attenuate further weight gain and could be useful in the diet or as a supplement to help maintain weight loss. Proposed mechanisms by which n-3 PUFAs may work to improve body composition and counteract obesity-related metabolic changes include modulating lipid metabolism; regulating adipokines, such as adiponectin and leptin; alleviating adipose tissue inflammation; promoting adipogenesis and altering epigenetic mechanisms.
Topics: Adipose Tissue; Animals; Anti-Obesity Agents; Body Composition; Exercise; Fatty Acids, Omega-3; Humans; Insulin Resistance; Metabolic Syndrome; Obesity; Panniculitis; Vegetables
PubMed: 29621669
DOI: 10.1016/j.jnutbio.2018.02.012 -
The Journal of Clinical Investigation Apr 2020BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine... (Clinical Trial)
Clinical Trial
BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.RESULTSThirty-six USAID patients (55%) had elevated IRG-S. Neutrophilic panniculitis (40% vs. 0%), basal ganglia calcifications (46% vs. 0%), interstitial lung disease (47% vs. 5%), and myositis (60% vs. 10%) were more prevalent in patients with elevated IRG-S. Moderate IRG-S elevation and highly elevated serum IL-18 distinguished 8 patients with pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS). Among patients with panniculitis and progressive cytopenias, 2 patients were compound heterozygous for potentially novel LRBA mutations, 4 patients harbored potentially novel splice variants in IKBKG (which encodes NF-κB essential modulator [NEMO]), and 6 patients had de novo frameshift mutations in SAMD9L. Of additional 12 patients with elevated IRG-S and CANDLE-, SAVI- or Aicardi-Goutières syndrome-like (AGS-like) phenotypes, 5 patients carried mutations in either SAMHD1, TREX1, PSMB8, or PSMG2. Two patients had anti-MDA5 autoantibody-positive juvenile dermatomyositis, and 7 could not be classified. Patients with LRBA, IKBKG, and SAMD9L mutations showed a pattern of IRG elevation that suggests prominent NF-κB activation different from the canonical interferonopathies CANDLE, SAVI, and AGS.CONCLUSIONSIn patients with elevated IRG-S, we identified characteristic clinical features and 3 additional autoinflammatory diseases: IL-18-mediated PAP and recurrent MAS (IL-18PAP-MAS), NEMO deleted exon 5-autoinflammatory syndrome (NEMO-NDAS), and SAMD9L-associated autoinflammatory disease (SAMD9L-SAAD). The IRG-S expands the diagnostic armamentarium in evaluating USAIDs and points to different pathways regulating IRG expression.TRIAL REGISTRATIONClinicalTrials.gov NCT02974595.FUNDINGThe Intramural Research Program of the NIH, NIAID, NIAMS, and the Clinical Center.
Topics: Autoimmune Diseases; Female; Humans; Interferon Type I; Interleukin-18; Macrophage Activation Syndrome; Male; Mutation; Panniculitis; Pulmonary Alveolar Proteinosis
PubMed: 31874111
DOI: 10.1172/JCI129301 -
Journal of the American Academy of... Oct 2022Panniculitis represents a rare and potentially lethal manifestation of alpha-1 antitrypsin deficiency (AATD). Evidence regarding management is limited to case reports... (Review)
Review
BACKGROUND
Panniculitis represents a rare and potentially lethal manifestation of alpha-1 antitrypsin deficiency (AATD). Evidence regarding management is limited to case reports and small case series. We sought to clarify typical features and investigation of AATD-associated panniculitis and assess the evidence regarding therapeutic options.
SEARCH METHODOLOGY
Articles and abstracts published between 1970 and 2020 were identified by searches of MEDLINE, PubMed, and secondary searches of references from relevant articles using the search terms "panniculitis," "alpha-1," "antitrypsin," "deficiency," and "Weber-Christian."
FINDINGS
We identified 117 cases of AATD-associated panniculitis. In 1 series, AATD was present in 15% of all cases of biopsy-proven panniculitis. Failure to achieve clinical response was seen in all instances of systemic steroid use. Dapsone, although effective and accessible, is frequently associated with failure to achieve remission. In these instances, intravenous AAT augmentation therapy generally resulted in response.
CONCLUSIONS
AATD may be more prevalent among patients presenting with panniculitis than previously thought. Patients presenting with panniculitis and systemic illness show high mortality risk. Although most cases are associated with the severe ZZ-genotype, moderate genotypes may also predispose to panniculitis. Dapsone remains the most cost-effective therapeutic option, whereas intravenous AAT augmentation remains the most efficacious. Finally, glucocorticoids appear ineffective in this setting.
Topics: Dapsone; Glucocorticoids; Humans; Panniculitis; alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency
PubMed: 33516773
DOI: 10.1016/j.jaad.2021.01.074 -
JAMA Dermatology Nov 2020This case series characterizes the initial presentation and treatment of lupus erythematosus panniculitis/profundus and its association with systemic lupus erythematosus...
This case series characterizes the initial presentation and treatment of lupus erythematosus panniculitis/profundus and its association with systemic lupus erythematosus and discoid lupus erythematosus.
Topics: Adolescent; Adult; Aged; Antirheumatic Agents; Child; Child, Preschool; Comorbidity; Delayed Diagnosis; Female; Humans; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Male; Middle Aged; Panniculitis, Lupus Erythematosus; Retrospective Studies; Young Adult
PubMed: 32876656
DOI: 10.1001/jamadermatol.2020.2797 -
BMJ Case Reports Sep 2017A 53-year-old black woman presented with a 3-day history of abdominal pain. Ultrasound of the abdomen showed a gall bladder packed with small stones. She gave a history...
A 53-year-old black woman presented with a 3-day history of abdominal pain. Ultrasound of the abdomen showed a gall bladder packed with small stones. She gave a history of abdominal surgery for a gynaecological condition. She had a cholecystectomy done, but her symptoms continued after cholecystectomy. She then had anendoscopic retrograde cholangiopancreatogram (ERCP) and sphincterotomy done, again her symptoms remained the same. A CT scan of the abdomen was done, and mesenteric panniculitis was suspected. A laparoscopic biopsy of the mesentery was performed, and it confirmed mesenteric panniculitis. She was started on a 2-week course of steroids to which she responded very well. Three months after the initial presentation, she was still asymptomatic.
Topics: Abdominal Pain; Diagnosis, Differential; Female; Glucocorticoids; Humans; Laparoscopy; Mesentery; Middle Aged; Panniculitis, Peritoneal; Prednisone; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 28870885
DOI: 10.1136/bcr-2017-220910 -
The Journal of Clinical Investigation Feb 2019Persistent, unresolved inflammation in adipose tissue is a major contributor to obesity-associated metabolic complications. However, the molecular links between...
Persistent, unresolved inflammation in adipose tissue is a major contributor to obesity-associated metabolic complications. However, the molecular links between lipid-overloaded adipocytes and inflammatory immune cells in obese adipose tissues remain elusive. Here we identified adipocyte-secreted microRNA-34a (miR-34a) as a key mediator through its paracrine actions on adipose-resident macrophages. The expression of miR-34a in adipose tissues was progressively increased with the development of dietary obesity. Adipose-selective or adipocyte-specific miR-34a-KO mice were resistant to obesity-induced glucose intolerance, insulin resistance, and systemic inflammation, and this was accompanied by a significant shift in polarization of adipose-resident macrophages from proinflammatory M1 to antiinflammatory M2 phenotype. Mechanistically, mature adipocyte-secreted exosomes transported miR-34a into macrophages, thereby suppressing M2 polarization by repressing the expression of Krüppel-like factor 4 (Klf4). The suppressive effects of miR-34a on M2 polarization and its stimulation of inflammatory responses were reversed by ectopic expression of Klf4 in both bone marrow-derived macrophages and adipose depots of obese mice. Furthermore, increased miR-34a expression in visceral fat of overweight/obese subjects correlated negatively with reduced Klf4 expression, but positively with the parameters of insulin resistance and metabolic inflammation. In summary, miR-34a was a key component of adipocyte-secreted exosomal vesicles that transmitted the signal of nutrient overload to the adipose-resident macrophages for exacerbation of obesity-induced systemic inflammation and metabolic dysregulation.
Topics: Adipocytes; Animals; Exosomes; Inflammation; Intra-Abdominal Fat; Kruppel-Like Factor 4; Kruppel-Like Transcription Factors; Macrophages; Mice; Mice, Knockout; MicroRNAs; Obesity; Panniculitis
PubMed: 30667374
DOI: 10.1172/JCI123069 -
Internal Medicine (Tokyo, Japan) Sep 2020A 44-year-old woman presented to our hospital with abdominal pain. Abdominal ultrasonography and computed tomography showed a mass-like change in the lesser omentum...
A 44-year-old woman presented to our hospital with abdominal pain. Abdominal ultrasonography and computed tomography showed a mass-like change in the lesser omentum between the liver and stomach. Esophagogastroduodenoscopy revealed a submucosal tumor-like change, and endoscopic ultrasonography (EUS) revealed that the mass was located outside of the stomach wall. We performed EUS fine-needle aspiration and diagnosed panniculitis of the lesser omentum. Based on these findings, we suggest that mass-like lesions in the lesser omentum and submucosal tumor-like changes in the anterior wall on the lesser curvature side of the stomach be evaluated for the possibility of panniculitis of the lesser omentum.
Topics: Adult; Biopsy, Fine-Needle; Endosonography; Female; Humans; Omentum; Panniculitis, Peritoneal; Tomography, X-Ray Computed
PubMed: 32461523
DOI: 10.2169/internalmedicine.4300-19 -
Medicina 2021
Topics: Humans; Pancreas; Pancreatic Neoplasms; Panniculitis
PubMed: 34633973
DOI: No ID Found