Low dose intravenous immunoglobulin in addition to cyclophosphamide in systemic sclerosis : Single centre experience.

CONCLUSION: The results of the study suggest that IVIG may be an additional treatment option together with CYC for patients for whom other therapies have failed, but further studies on the exact role of IVIG in the treatment of severe SSc are required. PMID: 32451820 [PubMed - as supplied by publisher]
Source: Wiener Klinische Wochenschrift - Category: General Medicine Authors: Tags: Wien Klin Wochenschr Source Type: research

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Authors: Kleymann J, Schütz C, Körholz J, Taube F, Vogler M, Halank M, Kolditz M, Langner S, Geberzahn L, Holotiuk O, Roesler J, Koschel D Abstract Chronic granulomatous disease (CGD) should be considered as a differential diagnosis in children and adolescents with frequent infections, especially when caused by certain specific pathogens.This case report describes a 64-year-old female with multiple recurrent and complicated bronchopulmonary infections, caused by common, but also rare pathogens, autoimmune phenomena, malignancies and recurrent organizing pneumonia (OP) with granulomas. Finally, the patient...
Source: Pneumologie - Category: Respiratory Medicine Tags: Pneumologie Source Type: research
Publication date: Available online 9 July 2020Source: Joint Bone SpineAuthor(s): Amélie Leurs, Sylvain Dubucquoi, François Machuron, Maïté Balden, Florence Renaud, Stéphanie Rogeau, Benjamin Lopez, Marc Lambert, Sandrine Morell-Dubois, Hélène Maillard, Hélène Béhal, Eric Hachulla, David Launay, Vincent Sobanski
Source: Joint Bone Spine - Category: Orthopaedics Source Type: research
Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc. There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Review 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
This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/). PMID: 31841044 [PubMed - as supplied by publisher]
Source: American Journal of Respiratory and Critical Care Medicine - Category: Respiratory Medicine Authors: Tags: Am J Respir Crit Care Med Source Type: research
AbstractObjectiveTo describe the phenotypic characteristics and natural history of patients with autoimmune forms of interstitial lung disease (ILD).MethodsRetrospective, descriptive, single-center study of patients with autoimmune forms of ILD evaluated between February 2008 and August 2014. All data were extracted from the electronic medical record. Longitudinal changes in forced vital capacity (FVC%) and diffusion capacity for carbon monoxide (DLco%) in percent predicted were analyzed and time-to-event analyses for death were performed using Cox regression.ResultsOf the entire cohort (n = 243), systemic sc...
Source: Lung - Category: Respiratory Medicine Source Type: research
ConclusionsThe lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respir...
Source: Multidisciplinary Respiratory Medicine - Category: Respiratory Medicine Source Type: research
Authors: Rizzi M, Radovanovic D, Airoldi A, Cristiano A, Frassanito F, Gaboardi P, Saad M, Atzeni F, Sarzi-Puttini P, Santus P Abstract Systemic sclerosis (SSc) is an autoimmune disease characterised by tissue fibrosis leading to vascular injury. Nitric oxide (NO) has been implicated in the pathogenesis of autoimmune diseases. A deficiency in basal NO production by the constitutive endothelial isoform of nitric oxide synthase may promote vasoconstriction and vascular wall thickening. In January 2017, we searched the PubMed/Medline, Cochrane Library and Enbase/Medline databases for studies analysing physio-pathologi...
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
Introduction: Systemic sclerosis (SSc) is a chronic autoimmune disease with ongoing high disease related mortality. Three randomized controlled trials proofed efficacy of autologous stem cell transplantation (aHSCT) in patients with severe SSc and superiority over the standard cyclophosphamide pulse therapy. Treatment related mortality is a major point of criticism and different treatment regimen are used in different centers.Methods: This prospective, open, multi-center, non-interventional study of the European Group for Blood and Marrow Transplantation (EBMT) analyzed data for consecutive aHSCT performed in SSc. Every ce...
Source: Blood - Category: Hematology Authors: Tags: 731. Clinical Autologous Transplantation: Results: Poster II Source Type: research
CONCLUSIONS: This review, which is based on studies of varying methodological quality, demonstrates that overall, in this population, small benefit may be derived from the use of cyclophosphamide in terms of mean difference in % FVC when compared with placebo, but not of the difference in % DLCO, or when compared with mycophenolate. Modest clinical improvement in dyspnoea may be noted with the use of cyclophosphamide. Clinical practice guidelines should advise clinicians to consider individual patient characteristics and to expect only modest benefit at best in preserving FVC. Clinicians should carefully monitor for advers...
Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
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