Hearing Loss Secondary to Systemic Sclerosis Vasculopathy: Case Study with a Short Review

Bobeică, Carmen; Niculeț, Elena; Crăescu, Mihaela; Parapiru, Elena-Laura; Mușat, Carmina Liana; Dinu, Ciprian; Nechita, Luiza; Ştefănescu, Victoriţa; Ștefanopol, Ioana Anca; Pelin, Ana-Maria; Nechifor, Alexandru; Bălan, Gabriela; Tatu, Alin Laurențiu

doi:10.2147/ccid.s356818

Cited by 3 publications

(8 citation statements)

References 21 publications

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“…But just as with other clinical features of SSc patients, the results are inconclusive. Studies by Silva et al ( 5 ), Bobeica et al ( 26 ), Turan et al ( 4 ), Santarelli et al ( 15 ) report progression of hearing loss over the course of the disease while other authors found no such dependency in their research ( 3 , 8 – 10 ). Indirectly, the relationship between disease duration and hearing impairment may be suggested by the fact that patients with lcSSc have unexpectedly worse hearing test results than those with dcSSc ( 3 , 6 ).…”

Section: Discussionmentioning

confidence: 90%

Hearing impairment in systemic sclerosis patients—what do we really know?

Sieśkiewicz,

Rębacz,

Sieśkiewicz

2024

Front. Med.

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BackgroundSystemic sclerosis (SSc) is a disease of a very heterogeneous clinical picture and immunological profile with progression rate that varies between individuals. Although hearing deterioration is not a complaint that comes to the fore in SSc patients, as it is not life-threatening compared to many other more severe symptoms of this disease, it can significantly impair the quality of life. Medical literature concerning this problem is rather scarce.Materials and methodsIn this article we systematically reviewed the medical publications concerning hearing impairment in patients with systemic sclerosis to evaluate current understanding of this complex problem. Following PRISMA guidelines a total of 19 papers were found and analysed including 11 original studies and 8 case reports.ResultsAlthough it seems that hearing impairment in SSc patients is relatively more common than in the general population, based on the analysis of available literature, no firm conclusions regarding its frequency and pathomechanism can be drawn yet. Microangiopathy leading to damage to the sensory cells of the inner ear is suspected to be the main mechanism of hearing loss, although damage to the higher levels of the auditory pathway appears to be underestimated due to incomplete audiological diagnosis.ConclusionUndoubtedly, the reason for the difficulty in such an evaluation are the complex and still not fully elucidated pathomechanism of SSc, the individually variable dynamics of the disease and the unique heterogeneity of symptoms. Nevertheless, further studies in larger and appropriately selected groups of patients, focused more on the dynamics of microangiopathy and not solely on clinical symptoms could provide answers to many key questions in this regard.

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Section: Discussionmentioning

confidence: 90%

Hearing impairment in systemic sclerosis patients—what do we really know?

Sieśkiewicz,

Rębacz,

Sieśkiewicz

2024

Front. Med.

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“…Endothelial activation triggers an inflammatory cascade that accumulates proinflammatory cytokines and chemokines, as well as growth factors with fibrogenetic potential (IL1, IL4, IL6, IL8, TNFα, TGF-β). Transforming growth factor β (TGF-β) induces fibroblast hyperactivation which exhibits aberrant profibrotic activity ( Figure 1 ) ( 108 – 112 ).…”

Section: Discussionmentioning

confidence: 99%

Immunologic and nonimmunologic sclerodermal skin conditions - review

Bobeica,

Niculet,

Craescu

et al. 2023

Front. Immunol.

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Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.

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“…Vascular endothelial growth factor (VEGF) is responsible for altering angiogenesis. 1 , 12 , 26 , 27 …”

Section: Introductionmentioning

confidence: 99%

“… 1 , 28 , 29 Some authors found low level of VEGF in patients with active digital ulcers and increased levels of VEGF in those with healed ulcers. 1 , 12 , 30 …”

Section: Introductionmentioning

confidence: 99%

“…Immune cells release cytokines with pro-inflammatory potential, especially IL-6, which is an indicator for disease severity. 4,[9][10][11][12] The abundance of immune cells determines increased serum levels of cytokines and chemokines which are involved in fibrogenesis. 4,[13][14][15][16] The role of Ly Th2 is essential in the pathogenesis of SSc.…”

Section: Introductionmentioning

confidence: 99%

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The Association of Telangiectasias with Other Peripheral Vascular Lesions of Systemic Sclerosis

Bobeica,

Niculet,

Musat

et al. 2024

CCID

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Purpose Systemic sclerosis (SSc) is a relatively rare collagenosis manifested as microvasculopathy, excessive cutaneous and visceral fibrosis in a background of autoimmune alteration. Autoimmune vasculopathy in SSc occurs early and begins with endothelial cell activation followed by blood vessel intimal proliferation in a context of defective angiogenesis. The alteration of peripheral micro and macrocirculation in SSc is evident through vascular lesions, such as Raynaud’s phenomenon, telangiectasias, acrocyanosis, digital ulcers, gangrene, peripheral pulse deficiency. Our paper details the results of the study on the association between telangiectasias and other types of immune-mediated peripheral vascular lesions that can be identified in SSc. The presence of these peripheral vascular lesions can provide information about the magnitude of the peripheral vasculopathy. Patients and Methods A total of 37 patients diagnosed with SSc, recruited from a university clinic in Bucharest between February 2019 and March 2020, were enrolled in an observational study. We evaluated the presence of telangiectasias, as a stigma of autoimmune microvasculopathy, and their association with other immune-mediated peripheral vascular lesions that may be present in SSc. Results The presence of telangiectasias was identified in the absence, but especially in the presence of acrocyanosis and digital ulcerations, and patients with peripheral pulse deficiency almost always had telangiectasias. Less than a quarter of the patients with digital ulcers progressed unfavorably to gangrene, and only one required amputation, telangiectasias being present not only in the patient with amputation but in all patients with gangrene. Conclusion We appreciate that telangiectasias may be the clinical expression of peripheral vasculopathy characteristic of SSc, they can often be present in association with other peripheral vascular lesions and may represent a valuable indicator for the gangrene risk of digital ulcerations in SSc.

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Hearing Loss Secondary to Systemic Sclerosis Vasculopathy: Case Study with a Short Review

Cited by 3 publications

References 21 publications

Hearing impairment in systemic sclerosis patients—what do we really know?

Hearing impairment in systemic sclerosis patients—what do we really know?

Immunologic and nonimmunologic sclerodermal skin conditions - review

The Association of Telangiectasias with Other Peripheral Vascular Lesions of Systemic Sclerosis

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