Susac’s Syndrome: An Updated Review

Pereira, Sara; Vieira, Bruna; Maio, Tiago; Moreira, Jorge; Sampaio, Filipa

doi:10.1080/01658107.2020.1748062

Cited by 36 publications

(28 citation statements)

References 32 publications

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“…Susac’s syndrome is a rare autoimmune disease that mainly affects young women. It is characterized by endotheliopathy, which presents as encephalopathy, retinal vasoocclusive disease, and hearing loss [ 46 ]. Figure 2 does not classify Susac’s syndrome with other cardiovascular AIDs, perhaps due to its relatively recent AID classification as attested by antinuclear antibodies, or to a different etiology.…”

Section: Resultsmentioning

confidence: 99%

Autoimmune Disease Classification Based on PubMed Text Mining

Samuels

Malov

Saha

et al. 2022

JCM

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Autoimmune diseases (AIDs) are often co-associated, and about 25% of patients with one AID tend to develop other comorbid AIDs. Here, we employ the power of datamining to predict the comorbidity of AIDs based on their normalized co-citation in PubMed. First, we validate our technique in a test dataset using earlier-reported comorbidities of seven knowns AIDs. Notably, the prediction correlates well with comorbidity (R = 0.91) and validates our methodology. Then, we predict the association of 100 AIDs and classify them using principal component analysis. Our results are helpful in classifying AIDs into one of the following systems: (1) gastrointestinal, (2) neuronal, (3) eye, (4) cutaneous, (5) musculoskeletal, (6) kidneys and lungs, (7) cardiovascular, (8) hematopoietic, (9) endocrine, and (10) multiple. Our classification agrees with experimentally based taxonomy and ranks AID according to affected systems and gender. Some AIDs are unclassified and do not associate well with other AIDs. Interestingly, Alzheimer’s disease correlates well with other AIDs such as multiple sclerosis. Finally, our results generate a network classification of autoimmune diseases based on PubMed text mining and help map this medical universe. Our results are expected to assist healthcare workers in diagnosing comorbidity in patients with an autoimmune disease, and to help researchers in identifying common genetic, environmental, and autoimmune mechanisms.

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Section: Resultsmentioning

confidence: 99%

Autoimmune Disease Classification Based on PubMed Text Mining

Samuels

Malov

Saha

et al. 2022

JCM

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“…Hearing loss, sometimes accompanied by vertigo and tinnitus, is usually bilateral and irreversible and is an indication for cochlear implantation [10]. BRAO can be one or two-sided, limited, or extensive and can cause black spots, photopsia or scintillating scotomas [4]. The disease may have monocyclic, polycyclic, or chronic-progressive course.…”

Section: Discussionmentioning

confidence: 99%

“…To diagnose SuS, a clinical picture and the results of additional tests such as brain MRI, FA and tonal audiometry are necessary [4]. MRI is the basic neuroimaging examination of choice for SuS diagnosing and it demonstrates numerous, multifocal, and different sized high signal intensity ischemic lesions on T2-weighted and FLAIR images in the white and grey matter.…”

Section: Discussionmentioning

confidence: 99%

“…The etiology of this disease is unknown, but most likely CD8 + T-cell-mediated endotheliopathy, leading to micro-infarcts of the precapillary arterioles of the brain, inner ear and retin, is a key disease mechanism in SuS [3]. Diagnosis is based on clinical symptoms and the results of additional tests, such as magnetic resonance imaging (MRI), audiometry and retinal fluorescein angiography (FA) [4]. However, the disease may be monocyclic, may follow a polycyclic or chronic-progressive course, and the symptoms may appear simultaneously or, more often, successively, which makes diagnosis difficult [5].…”

Section: Introductionmentioning

confidence: 99%

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Susac syndrome as a multidisciplinary diagnostic challenge

Sapko¹,

Jamróz-Wiśniewska²,

Woźniak³

et al. 2021

Ann Agric Environ Med.

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“…Susac’s syndrome (SS) is a rare, autoimmune-mediated vasculitis involving central nervous system (CNS) microvasculature [ 1 ]. This disease typically targets the brain, retina, and cochlea, giving the characteristic triad of encephalopathy, vision loss, and hearing loss.…”

Section: Introductionmentioning

confidence: 99%

A Rare Case of Susac's Syndrome Masquerading as Progressive-Relapsing Multiple Sclerosis

Darwish¹,

Bakillah²,

Rey³

et al. 2022

Cureus

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Susac’s syndrome (SS) is a rare, autoimmune-mediated vasculitis involving central nervous system (CNS) microvasculature, which typically targets the brain, retina, and cochlea. The disease pathology in these regions produces the characteristic triad of encephalopathy, visual loss, and hearing loss. Unfortunately, less than 20% of cases present as the full triad, often making diagnosis challenging. Diagnosis is also confounded by the similarity in the clinical presentation of multiple sclerosis (MS), with an overlap also seen in laboratory studies and radiographic imaging. In this report, we present a case of SS in a young and previously healthy adult male that was initially mistaken as MS. We review the characteristics of SS and highlight the key differences between the two diseases that can be used by diagnosing physicians. Lastly, we describe the treatment strategies involved in SS compared to MS.

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Susac’s Syndrome: An Updated Review

Cited by 36 publications

References 32 publications

Autoimmune Disease Classification Based on PubMed Text Mining

Autoimmune Disease Classification Based on PubMed Text Mining

Susac syndrome as a multidisciplinary diagnostic challenge

A Rare Case of Susac's Syndrome Masquerading as Progressive-Relapsing Multiple Sclerosis

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