Basics of onychopathology
Archana Singal, Shekhar Neema, Piyush Kumar in Nail Disorders, 2019
Scleroderma is characterized by progressive hardening of the skin. Of the several variants, acrosclerosis and CREST syndrome are important for nail pathology. The histologic alterations of diffuse scleroderma and morphea are virtually identical. Differences depend on the severity and depth of the changes. Early lesions exhibit a dense perivascular lymphocytic infiltrate. With time, the collagen bundles change to large, eosinophilic, hyaline, swollen ones. In nail pathology, proximal nail fold biopsies have been used for diagnostic purposes.206 They show perivascular lymphocytic infiltrates, splitting of the basal lamina, broadening of the perivascular connective tissue and immunoglobulin deposits,207 that are easily identified by PAS.208 Pterygium inversum unguis shows a hyperkeratosis of the hyponychium reaching farther distal and adhering to the nail plate. The isthmus appears to extend to the medial third of the nail bed.209–211
Altered Regulation of Fibrinolysis in Scleroderma and Potential for Thrombolytic Therapy
Pia Glas-Greenwalt in Fibrinolysis in Disease Molecular and Hemovascular Aspects of Fibrinolysis, 2019
One of the prevailing hypotheses for the pathogenesis of scleroderma is based on the possibility that the disease is initiated by repeated insults to the vascular endothelium3,5,39,50,58,59 (Figure 2). Factor Vm/von Willebrand factor has been reported to be increased in Raynaud’s phenomenon under basal conditions and more so after cold exposure.60-65 It has been suggested that the vascular abnormality may be caused by vasoconstriction leading to vascular ischemia and intravascular coagulation. After exposure to cold, decreased blood flow to organs and capillary “standstill” have been observed.66 In the presence of serum or plasma of scleroderma patients, erythrocytes demonstrate decreased deformability and enhanced binding to endothelial cells.67 Other indicators of vascular injury include evidence of platelet activation marked by decreased platelet serotonin,68 increased serotonin-induced platelet aggregation,69 circulating platelet aggregates, and increased plasma thromboglobulin.60,70-72 There has been unconfirmed evidence for an endothelial cytotoxic factor in scleroderma serum (reviewed in References 1 and 5).
Mobilization and Conditioning Regimens in Stem Cell Transplant for Autoimmune Diseases
Richard K. Burt, Alberto M. Marmont in Stem Cell Therapy for Autoimmune Disease, 2019
Noninfectious mobilization regimen related mortality has also been reported in systemic sclerosis patients receiving 4 g/m2 cyclophosphamide. The deaths occurred in patients with scleroderma associated with pulmonary artery hypertension. The cause of death was alveolar hemorrhage and/or myocardial infarction. Pulmonary artery hypertension should be considered a contraindication to either HSCT or cyclophosphamide stem cell mobilization. Patients with autoimmune cytopenias, such as ITP-related thrombocytopenia, have a compensatory marrow hyperplasia. In one patient, cyclophosphamide mobilization caused lethal bleeding by suppressing marrow production of megakarocytes, while antibody immune-mediated platelet destruction was still present.38 While no patient receiving only G-CSF died or became bacteremic, some patients mobilized with G-CSF experienced chest pain of unclear etiology, transient elevation of transaminases, headaches, joint pain, myalgias, and as mentioned above, disease exacerbation.36 Therefore, the symptoms and organ dysfunction due to the preexisting disease process must be taken into consideration in selecting candidates and designing the mobilization regimens. Mobilization regimens used in autoimmune transplants for autoimmune diseases are shown in Table 1.
Association between Skin Thickness Measurements with Corneal Biomechanical Properties and Dry Eye Tests in Systemic Sclerosis
Published in Ocular Immunology and Inflammation, 2019
Ziya Ayhan, Mahmut Kaya, Taylan Ozturk, Gul Arikan, Merih Birlik
Scleroderma is an autoimmune progressive chronic disorder of unknown aetiology; it is characterised by tightening and thickening of the skin associated with widespread microangiopathy and multi-organ fibrosis.1–3 It can be classified as limited cutaneous scleroderma (lcSSc) or diffuse cutaneous scleroderma (dcSSc) according to extent of skin involvement shown to influence the patient’s daily functions and survival.4,5 In lcSSc, the fibrosis is mainly restricted to the hands, arms and face, and Raynaud’s phenomenon is present for several years before fibrosis appears; pulmonary hypertension is frequent, and anti-centromere antibodies occur in 50–90% of those patients. Diffuse cutaneous scleroderma is a rapidly progressing disorder that affects large areas of the skin and compromises internal organs. Progressive fibrosis in the skin and lungs accounts for the significant morbidity and mortality of patients with SSc.6
“I'm still dad”: The Impact of Scleroderma on being a Father
Published in Occupational Therapy In Health Care, 2018
Janet L. Poole, Donna Haygood, Cindy Mendelson
Scleroderma is an autoimmune collagen disease that is manifested by hardening and fibrosis of the skin and internal organs. While the prevalence is 4.6 times greater in females than males (Meier et al., 2012), the disease is more severe in men resulting in a poorer prognosis and increased occurrence of lung and heart involvement (Elhai et al., 2016). The mean age of onset, between 30–50 years of age, is during the peak years when males are becoming or being fathers. Systemic scleroderma has two main subtypes: limited cutaneous scleroderma and diffuse cutaneous scleroderma. Limited systemic scleroderma (lSSc) has fibrotic skin involvement limited to the hands, fingers and face and later involvement of the internal organs (Connolly & Tuffanelli, 2004). In diffuse systemic scleroderma (dSSc), the more severe subtype, skin involvement occurs more proximally with early internal organ involvement.
Scleroderma-related interstitial lung disease: principles of management
Published in Expert Review of Respiratory Medicine, 2019
Aparna Das, Anupam Kumar, Andrea Valeria Arrossi, Subha Ghosh, Kristin B. Highland
Scleroderma is a disorder characterized by auto-antibody formation, vascular dysfunction and abnormal fibroblast function resulting in increased deposition of extracellular matrix. Interstitial lung disease (ILD) represents a common comorbidity in patients with scleroderma (systemic sclerosis, SSc). Evidence of interstitial changes in the lung maybe seen in up to 90% of patients and at least 50% have physiological impairment evident on pulmonary function tests [1]. Nonspecific interstitial pneumonia (NSIP) is the most common radiologic and histopathologic pattern seen, followed by usual interstitial pneumonia (UIP) [2]. ILD is more prevalent in diffuse cutaneous systemic sclerosis, but also occurs in limited SSc [3]. SSc-ILD typically occurs within the first few years of diagnosis of SSc, with the greatest decline in FVC occurring within the first four years of disease [3]. The prevalence of ILD increases with time from diagnosis and results in significant morbidity and mortality accounting for approximately 35% of SSc deaths [4]. Success with pharmacologic agents in slowing the progression of SSc-ILD remains modest. Nevertheless, several medications have demonstrated modest benefit and other treatments are under investigation.
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