Lung Transplantation In Patients With Systemic Sclerosis

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The identification and management of interstitial lung

Patients with systemic sclerosis are at high risk of developing interstitial lung disease (ILD). 50% of patients with systemic sclerosis have ILD when first assessed by high-resolution CT (HRCT),1 although a lesser proportion of patients have a severe reduction in pulmonary function. 2 Early diagnosis, severity assessment, prediction of pro-

Autopsy versus clinical findings in patients with systemic

Autopsy in EUSTAR patients B. Sandmeier et al. ed by the clinician, whereas only five deaths were classified as SSc-related by the pathologist after autopsy. In two cases, no statement of pathologists was given and in one case there was a dis-sent. In the patient, who underwent prior lung transplantation, the clinician


lung transplantation. Gastro-esophageal reflux disease (GERD), or esophageal dysmotility occurs frequently in scleroderma, and may be a reason not to attempt lung transplantation due to the risk of aspiration. putting it all together Pulmonary hypertension is not the only type of lung disease that can occur in patients with scleroderma.

Effectiveness and safety of tocilizumab for the treatment of

Oct 22, 2018 patients with systemic sclerosis (SSc) treated with tocilizumab (TCZ) compared with those receiving placebo. Initial investi-gations with TCZ in patients with SSc demonstrated improve-ments in skin sclerosis and polyarthritis.2 3 Scleroderma-associated interstitial lung disease (SSc-ILD) is a

Survival of Adults With Systemic Sclerosis Following Lung

Lung transplantation is a potentially life-saving treatment for adults with systemic sclerosis (SSc) who have developed end-stage lung disease due to interstitial lung disease (ILD) or pulmonary hy pertension. Despite a pau-city of data, many transplant p rograms are hesitant to offer lung transplantation to patients with SSc due to concerns

Skin Gene Expression Correlates of Severity of Interstitial

Additionally, lung tissue samples from patients with SSc and idiopathic pulmonary fibrosis (IPF) were investigated. These samples were obtained from patients who underwent lung transplantation at the University of Pittsburgh Medical Center under a protocol approved by the institutional review board. Also, normal lung tissue specimens were

Hematopoietic Stem Cell Transplantation for Scleroderma

of the lung (9). In a systematic review of 20 publications comprising 1,524 patients, DLCO (diffusion capacity of the lung for carbon monoxide) was the most consistent predictor of mortality in scleroderma lung disease while the extent of disease on HRCT imaging was an independent predictor for overall mortality and ILD progression (10).

Current and future perspectives on management of systemic

Group, National Heart and Lung Institute, Imperial College, London, UK ABSTRACT Introduction: Systemic sclerosis (SSc) is a rare and complex connective tissue disease characterized by fibrosis of the skin and internal organs. Interstitial lung disease (ILD) is a common complication of SSc and the leading cause of SSc-related death.

Commentary: The return of peristalsis after lung transplant

Mar 19, 2020 lung disease, lung allocation score, type of lung transplant, mean pulmonary artery pressure, and graft ischemic time. In the aperistalsis group, after lung transplant, 65% (19/ 29) of patients had improved esophageal motility, and approximately half of these patients (10/19) had what was determined to be effective esophageal motility (defined as

Commentary: Esophageal aperistalsis should not preclude lung

Mar 19, 2020 cohort of patients with systemic sclerosis, 60% of whom exclude the possibility of lung transplantation in carefully selected patients. High-risk fea-

Is heart transplantation a valuable option in patients with

Systemic sclerosis (SSc) is a multisystemic disorder mani-fested by autoimmune and fibrotic changes involving the skin and internal organs. Whereas lung transplantation has been widely reported in SSc, heart transplantation in an adult pa-tient has been rarely described. We report a case of a female

A Multipronged Approach to Inflammatory Disorders

identify scleroderma patients with pulmonary hypertension. In other research at Columbia, Dr. Bernstein is looking at outcomes in scleroderma patients who undergo lung transplantation. Lung transplantation is a potentially lifesaving procedure for scleroderma patients who develop end-stage lung disease due to pulmonary

Lung and Lobar Lung Transplant - 4/28/2017

A lung transplant consists of replacing all or part of diseased lungs with healthy lung(s). Transplantation is an option for patients with end-stage lung disease. The literature on lung and lobar lung transplantation, which consists of case series and registry data, demonstrates that lung and lobar lung transplantation provides a survival

Recommendations for the Treatment of Systemic Sclerosis

nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCISTM). Clin Exp Rheumatol 2017;35 Suppl 106:75 81. 14. Distler O, Highland KB, Gahlemman M, Azuma A, Fischer A, Mayes MD, et al; SENSCIS Trial Investigators. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 2019;380

Augmented concentrations of CX3CL1 are associated with

We collected lung tissue at the time of lung transplantation at UCLA from SSc-ILD patients concentrations of CX3CL1 are associated with interstitial lung disease in systemic sclerosis


Rationale: Patients with advanced lung disease due to systemic sclerosis have long been considered suboptimal and often unacceptable candidates for lung transplant. Objectives: To examine post lung transplant survival of patients with systemic sclerosis compared with patients with pulmonary fibrosis and to identify risk factors for 1-year

The potential of fecal microbiota transplantation in systemic

of interstitial lung disease in systemic sclerosis in a complete nationwide cohort. Am J Respir Crit Care Med. 2019;200:1258 1266. Epub ahead of print 2. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390 (10103):1685 1699. 3. Elhai M, Meune C, Boubaya M, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis

Patient and Physician Perspectives on Systemic Sclerosis

Systemic sclerosis (SSc), also known as scleroderma, is a sys-temic autoimmune disease that affects the skin and internal organs, particularly the lungs, kidney, heart, and gastrointestinal tract.1,2 Systemic sclerosis reduces quality of life and causes sig-nificant morbidity and mortality. It has a major financial impact,

Exercise pulmonary haemodynamics predict outcome in patients

tissue disease [1, 2]. In particular, PAH is most frequent in systemic sclerosis (SSc) with an estimated prevalence of 7.5 12% [3 5] and it is the leading cause of death in SSc with almost one-third of SSc-related deaths [6 9]. The gold standard for diagnosis of PAH in SSc patients is right heart catheterisation (RHC) and PAH is

Rituximab in the Treatment of Interstitial Lung Disease

Sep 23, 2020 Twenty-six patients (13 women and 13 men; mean age at AD and ILD diagnosis of 55.5 12.1 and 58.3 11.1years,respectively)fromacohortof34patientswithAD-ILD,treatedwithRTX,wereassessed in the present study. The remaining eight patients were excluded due to lung transplantation (n = 3),

Systemic sclerosis-associated interstitial lung disease

All patients with newly diagnosed systemic sclerosis should be screened for interstitial lung disease. Systemic sclerosis-associated interstitial lung disease is the end result of the interplay between fibrosis, autoimmunity, inflammation, and vascular injury. With the recent completion of the SENSCIS study, there is

IL-1 receptor blockade skews inflammation towards Th2 in a

Systemic sclerosis (SSc) is a rare progressive fibrotic disorder, which affects the skin and internal organs [1]. Lung involvement occurs in about 50% of patients and can manifest in both the parenchymal or vascular compartments as fibrosis and vascular remodelling,

Interstitial lung disease associated with systemic sclerosis

Background: Systemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death. Main body: All patients newly diagnosed with SSc should receive a comprehensive clinical evaluation, including

Hemodynamics and Survival in Patients With Pulmonary Arterial

Measurements and results: Patients with SScPH had somewhat lower percentage of predicted lung volumes than patients with PPH (total lung capacity, 80% vs 92%; p 0.06) and had lower percentage of predicted diffusion capacity of the lung for carbon monoxide (42% vs 68%; p 0.0002).

Lung Transplantation in Scleroderma

to lung transplantation in most centers. In this month s issue of the Annals, two centers report their experience with lung transplantation in SSc. Crespo and colleagues from the University of Pittsburgh Medical Center (UPMC) Lung Transplantation Program (pp. 784 792) Table 1. Outcomes in lung transplantation in systemic sclerosis Study Cohort

0597 Heart-Lung Transplantation (1)

Furthermore, heart-lung transplantation results in survival comparable to that reported for single- or double-lung transplantation for patients with primary pulmonary hypertension. Obliterative bronchiolitis, a form of chronic rejection, is a significant cause of late death. The frequency of heart-lung transplantation is partly limited by

The Treatment of Lung Involvement in Systemic Sclerosis

Feb 13, 2021 Abstract: Systemic sclerosis (SSc) patients are often affected by interstitial lung disease (ILD) and, although there have been recent treatment advances, it remains the leading cause of death among SSc, with a 10-year mortality up to 40%.

New therapeutic strategies for systemic sclerosis a critical

Keywords: Systemic sclerosis, bosentan, epoprostenol, IVIg, ACE inhibitors, stem cell transplantation Introduction Systemic sclerosis (SSc) is a multi-system disease characterized by skin fibrosis and visceral disease. The pathophysiology includes vascular damage, fibroblast proliferation, collagen production and activation of the immune system.

Hematopoietic stem cell transplantation for systemic

alternative for patients with severe and progressive systemic sclerosis. Keywords: Systemic sclerosis, Hematopoietic stem cell transplantation, Stem cells, Progenitor cells, Transplantation Background Autologous hematopoietic stem cell transplantation (HSCT) has been investigated as treatment for systemic sclerosis

Lung Function and Survival in Systemic Sclerosis Interstitial

Maher TM, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 2008; 177:1248-54. 3. Simeon C, Armadans L, Fonollosa V, Solans R, Selva A, Villar M, et al. Mortality and prognostic factors in Spanish patients with systemic sclerosis. Rheumatology 2003;42:71-5. 4. Steen VD, Medsger TA.

Radiological pleuroparenchymal fibroelastosis associated to

conditions like bone marrow transplantation, lung transplantation, chemotherapy, radiotherapy, but also immune diseases (spondylarthropathy, hemorrhagic recto-colitis) [7, 8]. PPFE may also be associated to heterozygous mutations in telomere-related genes like TERT, TERC,or RTEL1. In these cases, PPFE affects predominantly fe-males [3, 4].

Haematopoietic stem cell transplantation in systemic sclerosis

Systemic sclerosis Key messages Autologous haematopoietic stem cell transplanta-tion (HSCT) is currently the only disease modifying strategy that demonstrated grade A evidence for improving long-term survival, prevention of organ worsening as well as improvement of skin and pul-monary function in systemic sclerosis (SSc).

Lung and Lobar Lung Transplant - 9/28/2018

A lung transplant consists of replacing all or part of diseased lungs with healthy lung(s) or lobes. Transplantation is an option for patients with end-stage lung disease. For individuals who have end-stage pulmonary disease who receive a lung transplant, the evidence includes case series and registry studies.

Major lung complications of systemic sclerosis

Systemic sclerosis (SSc) is an uncommon disease characterized by fibrosis of the skin and internal organs and by vasculopathy. Amongst the autoimmune rheumatic diseases, systemic sclerosis has the highest mortality, which is mainly owing to the development of complications that affect the lungs [1,2]. The

The Lungs in Systemic Sclerosis

the functional changes in systemic sclerosis. They found reduction in lung volume, in­ creased minute ventilation and hypoxemia corrected by oxygen inhalation. Most au­ thors measuring pulmonary function in this disease, with or without x-ray changes, agree on the finding that the pattern of

Pulmonary complications: one of the most challenging

function testing. For patients with severe end-stage pulmonary fibrosis, lung transplantation may offer a viable alternative therapeutic option. KEY WORDS: Dyspnoea, Interstitial lung disease, Pulmonary hypertension, Systemic sclerosis. Introduction SSc is a clinically heterogeneous, multi-system autoimmune dis-order.

Pulmonary veno-occlusive disease is highly prevalent in

highly prevalent in scleroderma patients undergoing lung transplantation To the Editor: Pulmonary complications including pulmonary hypertension (PH) and interstitial lung disease (ILD) are leading causes of mortality in systemic sclerosis/scleroderma (SSc) [1]. PH associated with SSc (SSc-PH)

Long-term immune reconstitution and T cell repertoire

Keywords: Systemic sclerosis, T cell repertoire, Immune reconstitution, Hematopoietic stem cell transplantation Introduction Systemic sclerosis (SSc) is characterized by progressive fi-brosis in the skin and internal organs [1], with 5-year mor-tality rates up to 30% in rapidly progressive diffuse cutaneous SSc (dcSSc) according to the extent

EXTENDED REPORT Autologous stem cell transplantation in the

for severe systemic sclerosis (SSc) and determine whether the high transplant related mortality (TRM) improved with experience. This EBMT/EULAR report describes the longer outcome of patients originally described in addition to newly recruited cases. Methods: Only patients with SSc, treated by HSCT in European phase I II studies from 1996 up

Kidney failure after lung transplantation in systemic

Keywords: Arteriovenous fistula, Calcineurin inhibitor, Chronic kidney disease, Lung transplantation, Systemic sclerosis Introduction Systemic scleroderma (SSc) is known as a systemic disorder involving the kidney as a life-threatening scleroderma renal crisis that presents with the abrupt onset of severe hypertension accompanied by rapidly