Maxillofacial Implications of Scleroderma and Systemic Sclerosis – A Case Report and Literature Review

Scleroderma is a group of autoimmune diseases characterized by increased collagen synthesis and deposition within connective tissue. The skin disease can be classified into two major groups: localized and systemic scleroderma. Localized scleroderma differs from systemic scleroderma in that it involves only the skin. Systemic scleroderma, also known as systemic sclerosis, may affect any organ system.1 Systemic sclerosis is often differentiated from localized scleroderma early by the development of Raynaud ’s phenomenon, or paroxysmal vasospasm induced by cold or stress, which is the first sign in the vast majority of systemic sclerosis cases.
Source: Journal of Oral and Maxillofacial Surgery - Category: ENT & OMF Authors: Tags: Case Report Source Type: research

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Authors: Bobeică C, Laurențiu Tatu A, Crăescu M, Solovăstru L Abstract Systemic sclerosis (SSc) is a collagenosis with insufficiently known etiopathogenesis, characterized by microvasculopathy and excessive fibrosis in the context of an autoimmune disorder. The incompletely elucidated pathogenesis and limited therapeutic options, disabling aspects, skin lesions and pain determine important functional and psychological deficiencies which affect the quality of life. It is imperative to observe and correlate individual clinical and paraclinical data to optimize disease management. A group of 22 patients diagnosed ...
Source: Experimental and Therapeutic Medicine - Category: General Medicine Tags: Exp Ther Med Source Type: research
Authors: Rezus E, Burlui AM, Gafton B, Stratulat TA, Zota GR, Cardoneanu A, Rezus C Abstract Systemic sclerosis (SSc) is a rare and complex autoimmune disease associated with poor vital and functional outcomes. The functional hindrance in patients derives from various disease-specific manifestations, including Raynaud's phenomenon and digital ulcers (DUs). Bosentan is an endothelin receptor antagonist capable of preventing the appearance of new DUs in patients with scleroderma. The present study aimed to evaluate the effects of Bosentan on the severity of Raynaud's phenomenon, DU-related symptoms and functional imp...
Source: Experimental and Therapeutic Medicine - Category: General Medicine Tags: Exp Ther Med Source Type: research
[Influence of time to diagnosis of patients with systemic sclerosis on lung function and comorbidities: a preclinical and clinical analysis]. Z Rheumatol. 2019 Dec 17;: Authors: Janto A, Triantafyllias K, Schwarting A Abstract BACKGROUND: Scleroderma or systemic sclerosis (SSc) is a rare autoimmune rheumatic connective tissue disease. The clinical picture is manifold and symptoms can vary greatly between different patients. All manifestations are possible ranging from isolated skin involvement up to systemic disease with multiple organ manifestations. Due to this inhomogeneous clinical pictu...
Source: Zeitschrift fur Rheumatologie - Category: Rheumatology Authors: Tags: Z Rheumatol Source Type: research
Abstract Raynaud's phenomenon is a common vasospastic condition which carries a significant burden of pain and hand-related disability (Hughes and Herrick, 2016). The prevalence of Raynaud's phenomenon in the general population has been reported to be approximately 5% (Garner et al, 2015). Raynaud's phenomenon can occur either as a primary ('idiopathic') phenomenon or secondary to a wide range of underlying medical conditions and drug causes. Therefore, hospital-based specialists are frequently involved in the care of patients with Raynaud's phenomenon and need to be aware of associated conditions and prescribed m...
Source: British Journal of Hospital Medicine - Category: Internal Medicine Authors: Tags: Br J Hosp Med (Lond) Source Type: research
Purpose of review To summarize recent advances in the understanding of the pathogenesis of autoimmune fibrotic diseases. These diseases include IgG4-related disease, systemic sclerosis and lupus nephritis. Recent findings Recent studies indicate that a poorly studied subset of helper T cells, cytotoxic CD4+ T cells and sub-populations of disease-specific activated B cells infiltrate inflamed tissues and collaborate to induce tissue fibrosis in autoimmune fibrotic diseases. Cycles of apoptosis induced by antigen-specific cytotoxic CD4+ T cells followed by macrophage-mediated clearing of apoptotic cells and finally tiss...
Source: Current Opinion in Rheumatology - Category: Rheumatology Tags: RAYNAUD PHENOMENON, SCLERODERMA, OVERLAP SYNDROMES AND OTHER FIBROSING SYNDROMES: Edited by John Varga Source Type: research
Abstract Systemic sclerosis is a complex autoimmune connective tissue disease which carries a significant burden of disease-related morbidity including potentially life-threatening complications. Systemic sclerosis can affect all the major organs and therefore, although the disease is uncommon, many hospital-based specialists are involved in patient care. Vascular disease (e.g. Raynaud's phenomenon) is an almost universal symptom in patients with systemic sclerosis and is often the earliest manifestation of the disease. Systemic sclerosis not uncommonly can overlap with other rheumatological conditions (e.g. rheum...
Source: British Journal of Hospital Medicine - Category: Internal Medicine Authors: Tags: Br J Hosp Med (Lond) Source Type: research
AbstractRaynaud ’s phenomenon (RP) is used to describe a symptom complex caused by digital vascular compromise. RP is a clinical diagnosis. The typically episodic nature of RP has resulted in a reliance upon patient self-report for diagnosis. The term ‘primary RP’ is generally applied when no underlying patho logy can be demonstrated. Whilst ‘primary RP’ is currently considered a distinct disorder, there is evidence that the term may comprise several entities that include a functional vasospastic disorder, a physiologically appropriate thermoregulatory response, subclinical atherosclerosis and...
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
This report has basically focused on scleroderma renal crisis (SRC), which is the most serious renal manifestation of SSc, characterized by renal failure and sudden onset of hypertension. A 44-year-old man was hospitalized with hypertension, headache, vertigo, nausea, rhinorrhea, reflux, dysphagia, dyspnea (Fc II), visual impairment, mechanical arthralgia, and edema (+3) accompanied by a rare skin lesion. Raynaud's phenomenon was also remarkable in fingers and toes. According to signs and symptoms, SSc diagnosed and the proper treatment was applied. It is of great importance that in the case of malignant hypertension in pa...
Source: Iranian Journal of Kidney Diseases - Category: Urology & Nephrology Tags: Iran J Kidney Dis Source Type: research
Authors: Cutolo M, Soldano S, Smith V Abstract INTRODUCTION: Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease characterized by chronic and progressive tissue and organ fibrosis with broad patient-to-patient variability. Some risk factors are known and include combination of persistent Raynaud's phenomenon, steroid hormone imbalance, selected chemicals, thermal or other injuries. Endogenous and/or exogenous environmental trigger/risk factors promote epigenetic mechanisms in genetically primed subjects. Disease pathogenesis presents early microvascular changes with endothelial cell dysfuncti...
Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research
Osman K. Yilmaz1, Stefanie Haeberle1, Meifeng Zhang1, Marvin J. Fritzler2, Alexander H. Enk1 and Eva N. Hadaschik1,3* 1Department of Dermatology, University of Heidelberg, Heidelberg, Germany 2Mitogen Advanced Diagnostics Laboratory, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada 3Department of Dermatology, University Hospital of Essen, Essen, Germany Due to a missense mutation in the Foxp3 gene, scurfy mice are deficient in functional regulatory T cells (Treg). The consequent loss of peripheral tolerance manifests itself by fatal autoimmune mediated multi-organ disease. Previous studies...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
More News: Autoimmune Disease | ENT & OMF | Raynaud's Phenomenom | Scleroderma | Skin