Heart Transplantation in Systemic Sclerosis: New Impulses for Conventional Scleroderma Transplantation Regimen and Scleroderma Diagnostic Monitoring: 2 Case Reports

2019 ◽  
Vol 51 (3) ◽  
pp. 865-870 ◽  
Author(s):  
I. Faust ◽  
J. Weile ◽  
B. Fujita ◽  
R. Kandolf ◽  
D. Hendig ◽  
...  
2011 ◽  
Vol 2011 ◽  
pp. 1-12 ◽  
Author(s):  
Cristina Rigamonti ◽  
Dimitrios P. Bogdanos ◽  
Maria G. Mytilinaiou ◽  
Daniel S. Smyk ◽  
Eirini I. Rigopoulou ◽  
...  

Patients with primary biliary cirrhosis (PBC) often have concurrent limited systemic sclerosis (SSc). Conversely, up to one-fourth of SSc patients are positive for PBC-specific antimitochondrial antibodies (AMA). The mechanisms responsible for the co-occurrence of these diseases are largely unknown. Genetic, epigenetic, environmental, and infectious factors appear to be important for the pathogenesis of the disease, but the hierarchy of events are not well defined. Patients with SSc and PBC have an increased morbidity and mortality compared with the general population, but whether the presence of both diseases in an affected individual worsens the prognosis and/or outcome of either disease is not clear. Some case reports suggested that the presence of SSc in PBC patents is associated with a more favorable prognosis of the liver disease, whereas others report an increased mortality in patients with PBC and SSc compared to patients with PBC alone. This paper discusses the features of patients with PBC-associated SSc. Our aims are to clarify some of the pathogenetic, diagnostic, and clinical challenges that are currently faced in the routine management of these patients. We also intend to provide some practical hints for practitioners that will assist in the early identification of patients with PBC-associated SSc.


2015 ◽  
Vol 2 ◽  
pp. 176-180 ◽  
Author(s):  
Edyta Machura ◽  
Beata Chodór ◽  
Magdalena Kleszyk ◽  
Małgorzata Pindycka-Piaszczyńska ◽  
Ewelina Chrobak ◽  
...  

Revista Dor ◽  
2016 ◽  
Vol 17 (2) ◽  
Author(s):  
Matheus Santos Gomes Jorge ◽  
Lia Mara Wibelinger ◽  
Bruna Knob ◽  
Caroline Zanin

2019 ◽  
Vol 08 (01) ◽  
pp. e27-e29
Author(s):  
Jamila Kremer ◽  
Sameer Saeed Ahmed Al-Maisary ◽  
Gábor Szabó

A 50-year-old female presented with progressive heart failure due to obstruction of the mitral valve after heart transplantation in 2008. Through the occlusion catheter (IntraClude Intra-Aortic Occlusion Device), aortic cross-clamping, antegrade cardioplegia, and aortic root venting were performed.Our case reports the first published study on minimally invasive mitral valve replacement for valve stenosis through intraluminal aortic clamping. The hazard of traumatic injuries can be reduced by avoiding resternotomy and circumventing adhesions between the aorta and the pulmonary artery after heart transplantation. Minimally invasive valve surgery is a safe and effective method with regard to short- and long-term results, especially in redo operations.


2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Bumsoo Park ◽  
Raghavendra C. Vemulapalli ◽  
Amit Gupta ◽  
Maria E. Shreve ◽  
Della A. Rees

Systemic sclerosis, or scleroderma, is a complex medical disorder characterized by limited or diffuse skin thickening with frequent involvement of internal organs such as lungs, gastrointestinal tract, or kidneys. Docetaxel is a chemotherapeutic agent which has been associated with cutaneous side effects. An uncommon cutaneous side effect of docetaxel is scleroderma-like skin changes that extend from limited to diffuse cutaneous systemic sclerosis. Several case reports have been published regarding the association of docetaxel and systemic sclerosis. However, those reports demonstrated the association between docetaxel and scleroderma-like skin changes without internal organ involvement. Here, we report a case of systemic sclerosis with pulmonary arterial hypertension and a microangiopathic kidney involvement induced by docetaxel chemotherapy. After an exhaustive literature review, this could be the first case of docetaxel-induced systemic sclerosis involving internal organs.


Author(s):  
Jae Yoon Kim ◽  
Sang Yoon Do ◽  
Young Hoon Moon ◽  
Chul Gab Lee ◽  
Yun Sung Kim ◽  
...  

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Firdevs Ulutaş ◽  
Erdem Çomut ◽  
Veli Çobankara

Systemic sclerosis (SSc) is an uncommon rheumatic disease in which the underlying main histopathologic feature is a thickening of the skin due to excessive accumulation of collagen in the extracellular tissue. Fibrogenesis, chronic inflammation, and ulceration may eventually promote skin neoplasms. Although nonmelanoma skin cancer (NMSC) is the most frequent type, there have been restricted case reports and case series with skin cancers in SSc patients in the literature. Herein, we describe a 78-year-old woman diagnosed with diffuse cutaneous systemic sclerosis thirteen years ago and associated nonspecific interstitial pneumonia that was successfully treated with high cumulative doses of cyclophosphamide. She developed basal cell carcinoma and squamous cell carcinoma of the skin in the follow-up. She is still on rituximab treatment with stable interstitial lung disease as indicated by pulmonary function tests and high-resolution chest computed tomography. To our knowledge and a literature search, this is the first reported patient with SSc with two types of skin cancer. In this review, we also aimed to emphasize the relationship between SSc and skin cancer, and possible risk factors for SSc-related skin cancer.


2021 ◽  
Author(s):  
Nina Hofmann ◽  
◽  
Robert Ambühl ◽  
Suzana Jordan ◽  
Oliver Distler

Review question / Objective: To systematically review treatment effectiveness and adverse events of calcineurin inhibitors (CNIs) such as cyclosporine A (CsA) and tacrolimus in patients with systemic sclerosis (SSc). Condition being studied: Systemic sclerosis. Eligibility criteria: • Publications with SSc patients treated with CNIs and available information on the outcome of CNI therapy on SSc disease manifestation will be analysed.• Article types: case reports, clinical study, clinical trial, controlled clinical trial, historical article, randomized controlled trial.


2016 ◽  
Vol 73 (9) ◽  
pp. 868-872
Author(s):  
Jelena Karadzic ◽  
Aleksandra Radosavljevic ◽  
Igor Kovacevic

Introduction. Scleroderma (systemic sclerosis) is a severe chronic connective tissue disease, which results in involvement of numerous internal organs. Changes in the eye are the consequences of organ-specific manifestations of scleroderma or adverse effects of immunosuppressive treatment applied. Case report. We reported a 42-year-old woman with systemic sclerosis and acute deterioration of vision in the left eye, with visual acuity 0.9. After thorough clinical examination, including fluorescein angiography and optical coherence tomography, the diagnosis of nonischemic central retinal vein occlusion was made. Further biochemical, rheumatological and immunological investigation, apart from inactive systemic sclerosis, showed normal findings. Therefore, the cause of central retinal vein occlusion could only be attributed to the microvascular changes in systemic sclerosis. After three months, visual acuity deteriorated to 0.6 due to the development of cystoid macular edema. The patient received intravitreal injection of bevacizumab and after a single dose visual acuity improved to 0.9. After a 6- month follow-up, macular edema resolved and visual acuity stabilized. Conclusion. According to our knowledge and current data from the literature, central retinal vein occlusion is a rare vision threatening manifestation of scleroderma. There are only few published case reports on central vein occlusion in scleroderma patients. Examination of the ocular fundus is recommended for evaluation of vascular disease in patients with systemic sclerosis.


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