Browsing by Subject "autoimmune"

Now showing 1 - 3 of 3
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    Differentiating IgG4-related sclerosing cholangiopathy from cholangiocarcinoma using CT and MRI: experience from a tertiary referring center
    (Springer, 2019-06) Swensson, Jordan; Tirkes, Temel; Tann, Mark; Cui, Enming; Sandrasegaran, Kumaresan; Radiology and Imaging Sciences, School of Medicine
    Purpose To compare the cross-sectional imaging findings of immunoglobulin G4-related sclerosing cholangiopathy (IgG4-SC) and cholangiocarcinoma (CCA). Methods Retrospective search of radiology and pathology databases identified 24 patients with IgG4-SC and over 500 patients with CCA from January 2009 to December 2016. Patients with no pre-treatment imaging studies available on PACS, non-contrasted imaging only, presence of mass lesions, metastatic disease or biliary stents were excluded. 17 patients with IgG4-SC and a selected group of 20 (age and gender matched) patients with CCA were obtained. Images were blinded and independently reviewed by two radiologists. Differences in proportions and means between groups were analyzed using Fishers and Mann–Whitney tests, respectively. Results Both readers identified a statistically significant difference in the presence of abrupt common bile duct narrowing between IgG4-SC and CCA (6.7% vs. 68.4%, p < 0.001; 33.3% vs. 75%, p = 0.019). No difference was seen in biliary wall thickening, wall enhancement, extrahepatic exclusive location of disease, or pancreatic duct dilation. Inter-observer variability was κ = 0.52. Total bilirubin and CA 19-9 were unable to differentiate between IgG4-SC and CCA. Serum IgG4 was positive in two of six IgG4-SC patients who were tested. Conclusion IgG4-SC and CCA share many clinical and imaging findings on CT and MRI. Abrupt bile duct cut sign strongly favors CCA. In the absence of this finding, IgG4-SC should be considered in the differential diagnosis in all cases of suspected extrahepatic CCA.
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    New Insights in the Pathogenesis of Multiple Sclerosis—Role of Acrolein in Neuronal and Myelin Damage
    (MDPI, 2013-10-09) Tully, Melissa; Shi, Riyi; Medicine, School of Medicine
    Multiple sclerosis (MS) is an autoimmune disease of the central nervous system (CNS) characterized by an inappropriate inflammatory reaction resulting in widespread myelin injury along white matter tracts. Neurological impairment as a result of the disease can be attributed to immune-mediated injury to myelin, axons and mitochondria, but the molecular mechanisms underlying the neuropathy remain incompletely understood. Incomplete mechanistic knowledge hinders the development of therapies capable of alleviating symptoms and slowing disease progression in the long-term. Recently, oxidative stress has been implicated as a key component of neural tissue damage prompting investigation of reactive oxygen species (ROS) scavengers as a potential therapeutic option. Despite the establishment of oxidative stress as a crucial process in MS development and progression, ROS scavengers have had limited success in animal studies which has prompted pursuit of an alternative target capable of curtailing oxidative stress. Acrolein, a toxic β-unsaturated aldehyde capable of initiating and perpetuating oxidative stress, has been suggested as a viable point of intervention to guide the development of new treatments. Sequestering acrolein using an FDA-approved compound, hydralazine, offers neuroprotection resulting in dampened symptom severity and slowed disease progression in experimental autoimmune encephalomyelitis (EAE) mice. These results provide promise for therapeutic development, indicating the possible utility of neutralizing acrolein to preserve and improve neurological function in MS patients.
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    PAS the Salt: A Case of Autoimmune Polyglandular Syndrome Type II
    (2021-03) d'Arnaud, Lindsey; Owusu, Raiven; Vinze, Sanjna; Vucescu, Raluca I.
    CASE DESCRIPTION: Here we present the case of a 71 year old female with a decades-long history of Hashimoto thyroiditis and vitiligo who developed Addison Disease (AD). Routine labs showed serum sodium of 124, and the patient reported fatigue and lightheadedness on follow-up with her primary care physician. Despite discontinuation of hydrochlorothiazide, subsequent labs showed Na of 112 and she was sent to the emergency department and found to have hypoosmolar hyponatremia consistent with syndrome of inappropriate antidiuretic hormone secretion. Fluid restriction, saline infusion, and later sodium-chloride tablets failed to improve sodium levels two days after admission. Further investigation revealed low morning cortisol that did not respond to adrenocorticotropic hormone (ACTH) stimulation, demonstrating adrenal insufficiency. Later workup revealed elevated serum ACTH and positive 21-hydroxylase antibodies consistent with autoimmune adrenalitis. CONCLUSION: AD, albeit rare, is important to consider in severely hyponatremic patients with established monoglandular endocrinopathy. CLINICAL SIGNIFICANCE: The patient has autoimmune polyglandular syndrome type II (PAS-II), defined by the presence of AD and either autoimmune thyroid diseases (AITDs) and/or type one diabetes mellitus; patients may also exhibit other autoimmune conditions such as pernicious anemia, premature ovarian failure, alopecia, vitiligo, celiac disease, or multiple sclerosis. PAS-II is a rare diagnosis with a prevalence of 1-2 per 100,000 and a male-to-female ratio of 1:3. It is usually not recommended to routinely screen for other autoimmune diseases in patients with existing AITDs. Autoimmune endocrinopathies pose potential harms to patients, such as life-threatening adrenal crisis, metabolic derangements, infertility, and worsened quality of life; these harms ought to be considered in deciding if and how often to screen for concomitant autoimmune disorders in patients with monoglandular autoimmune endocrinopathies.