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Armin Ernst - One of the best experts on this subject based on the ideXlab platform.
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pulmonary manifestations of relapsing Polychondritis
Clinics in Chest Medicine, 2010Co-Authors: Samaan Rafeq, David E Trentham, Armin ErnstAbstract:Relapsing Polychondritis (RP) is a chronic multisystemic disease characterized by recurrent episodes of cartilage inflammation throughout the body. The lower respiratory tract is involved in 20% to 50% of patients and results in significant morbidity. Effective medical therapies and airway interventions are available in experienced centers; however, no single treatment is curative, and the prognosis of RP with airway disease remains overall guarded.
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relapsing Polychondritis and airway involvement
Chest, 2008Co-Authors: Armin Ernst, Samaan Rafeq, Phillip M Boiselle, Arthur Sung, Chakravarthy Reddy, Gaetane Michaud, Adnan Majid, Felix J F Herth, David E TrenthamAbstract:Objective To assess the prevalence and characteristics of airway involvement in relapsing Polychondritis (RP). Methods Retrospective chart review and data analysis of RP patients seen in the Rheumatology Clinic and the Complex Airway Center at Beth Israel Deaconess Medical Center from January 2004 through February 2008. Results RP was diagnosed in 145 patients. Thirty-one patients had airway involvement, a prevalence of 21%. Twenty-two patients were women (70%), and they were between 11 and 61 years of age (median age, 42 years) at the time of first symptoms. Airway symptoms were the first manifestation of disease in 17 patients (54%). Dyspnea was the most common symptom in 20 patients (64%), followed by cough, stridor, and hoarseness. Airway problems included the following: subglottic stenosis (n = 8; 26%); focal and diffuse malacia (n = 15; 48%); and focal stenosis in different areas of the bronchial tree in the rest of the patients. Twelve patients (40%) required and underwent intervention including balloon dilatation, stent placement, tracheotomy, or a combination of the above with good success. The majority of patients experienced improvement in airway symptoms after intervention. One patient died during the follow-up period from the progression of airway disease. The rest of the patients continue to undergo periodic evaluation and intervention. Conclusion In this largest cohort described in the English language literature, we found symptomatic airway involvement in RP to be common and at times severe. The nature of airway problems is diverse, with tracheomalacia being the most common. Airway intervention is frequently required and in experienced hands results in symptom improvement.
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relapsing Polychondritis prevalence of expiratory ct airway abnormalities
Radiology, 2006Co-Authors: Karen S Lee, David E Trentham, Armin Ernst, William Lunn, David Fellerkopman, Phillip M BoiselleAbstract:Purpose: To retrospectively determine the prevalence of expiratory computed tomographic (CT) abnormalities, including malacia and air trapping, in patients with relapsing Polychondritis and to retrospectively determine the frequency with which expiratory abnormalities are accompanied by inspiratory abnormalities on CT scans. Materials and Methods: Institutional review board approval was obtained, and informed consent was not required for this retrospective HIPAA-compliant study. A computerized hospital information system was used to identify all patients with clinically diagnosed or biopsy-proved relapsing Polychondritis who were referred for CT airway imaging during a 17-month period. The study cohort comprised 18 patients (15 women, three men; mean age, 47 years; age range, 20–71 years). Multidetector helical CT was performed in all patients by using a standard protocol, which included end-inspiratory and dynamic expiratory volumetric imaging. Two observers who were blinded to the original scan interpre...
David E Trentham - One of the best experts on this subject based on the ideXlab platform.
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pulmonary manifestations of relapsing Polychondritis
Clinics in Chest Medicine, 2010Co-Authors: Samaan Rafeq, David E Trentham, Armin ErnstAbstract:Relapsing Polychondritis (RP) is a chronic multisystemic disease characterized by recurrent episodes of cartilage inflammation throughout the body. The lower respiratory tract is involved in 20% to 50% of patients and results in significant morbidity. Effective medical therapies and airway interventions are available in experienced centers; however, no single treatment is curative, and the prognosis of RP with airway disease remains overall guarded.
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relapsing Polychondritis and airway involvement
Chest, 2008Co-Authors: Armin Ernst, Samaan Rafeq, Phillip M Boiselle, Arthur Sung, Chakravarthy Reddy, Gaetane Michaud, Adnan Majid, Felix J F Herth, David E TrenthamAbstract:Objective To assess the prevalence and characteristics of airway involvement in relapsing Polychondritis (RP). Methods Retrospective chart review and data analysis of RP patients seen in the Rheumatology Clinic and the Complex Airway Center at Beth Israel Deaconess Medical Center from January 2004 through February 2008. Results RP was diagnosed in 145 patients. Thirty-one patients had airway involvement, a prevalence of 21%. Twenty-two patients were women (70%), and they were between 11 and 61 years of age (median age, 42 years) at the time of first symptoms. Airway symptoms were the first manifestation of disease in 17 patients (54%). Dyspnea was the most common symptom in 20 patients (64%), followed by cough, stridor, and hoarseness. Airway problems included the following: subglottic stenosis (n = 8; 26%); focal and diffuse malacia (n = 15; 48%); and focal stenosis in different areas of the bronchial tree in the rest of the patients. Twelve patients (40%) required and underwent intervention including balloon dilatation, stent placement, tracheotomy, or a combination of the above with good success. The majority of patients experienced improvement in airway symptoms after intervention. One patient died during the follow-up period from the progression of airway disease. The rest of the patients continue to undergo periodic evaluation and intervention. Conclusion In this largest cohort described in the English language literature, we found symptomatic airway involvement in RP to be common and at times severe. The nature of airway problems is diverse, with tracheomalacia being the most common. Airway intervention is frequently required and in experienced hands results in symptom improvement.
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relapsing Polychondritis prevalence of expiratory ct airway abnormalities
Radiology, 2006Co-Authors: Karen S Lee, David E Trentham, Armin Ernst, William Lunn, David Fellerkopman, Phillip M BoiselleAbstract:Purpose: To retrospectively determine the prevalence of expiratory computed tomographic (CT) abnormalities, including malacia and air trapping, in patients with relapsing Polychondritis and to retrospectively determine the frequency with which expiratory abnormalities are accompanied by inspiratory abnormalities on CT scans. Materials and Methods: Institutional review board approval was obtained, and informed consent was not required for this retrospective HIPAA-compliant study. A computerized hospital information system was used to identify all patients with clinically diagnosed or biopsy-proved relapsing Polychondritis who were referred for CT airway imaging during a 17-month period. The study cohort comprised 18 patients (15 women, three men; mean age, 47 years; age range, 20–71 years). Multidetector helical CT was performed in all patients by using a standard protocol, which included end-inspiratory and dynamic expiratory volumetric imaging. Two observers who were blinded to the original scan interpre...
Phillip M Boiselle - One of the best experts on this subject based on the ideXlab platform.
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relapsing Polychondritis and airway involvement
Chest, 2008Co-Authors: Armin Ernst, Samaan Rafeq, Phillip M Boiselle, Arthur Sung, Chakravarthy Reddy, Gaetane Michaud, Adnan Majid, Felix J F Herth, David E TrenthamAbstract:Objective To assess the prevalence and characteristics of airway involvement in relapsing Polychondritis (RP). Methods Retrospective chart review and data analysis of RP patients seen in the Rheumatology Clinic and the Complex Airway Center at Beth Israel Deaconess Medical Center from January 2004 through February 2008. Results RP was diagnosed in 145 patients. Thirty-one patients had airway involvement, a prevalence of 21%. Twenty-two patients were women (70%), and they were between 11 and 61 years of age (median age, 42 years) at the time of first symptoms. Airway symptoms were the first manifestation of disease in 17 patients (54%). Dyspnea was the most common symptom in 20 patients (64%), followed by cough, stridor, and hoarseness. Airway problems included the following: subglottic stenosis (n = 8; 26%); focal and diffuse malacia (n = 15; 48%); and focal stenosis in different areas of the bronchial tree in the rest of the patients. Twelve patients (40%) required and underwent intervention including balloon dilatation, stent placement, tracheotomy, or a combination of the above with good success. The majority of patients experienced improvement in airway symptoms after intervention. One patient died during the follow-up period from the progression of airway disease. The rest of the patients continue to undergo periodic evaluation and intervention. Conclusion In this largest cohort described in the English language literature, we found symptomatic airway involvement in RP to be common and at times severe. The nature of airway problems is diverse, with tracheomalacia being the most common. Airway intervention is frequently required and in experienced hands results in symptom improvement.
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relapsing Polychondritis prevalence of expiratory ct airway abnormalities
Radiology, 2006Co-Authors: Karen S Lee, David E Trentham, Armin Ernst, William Lunn, David Fellerkopman, Phillip M BoiselleAbstract:Purpose: To retrospectively determine the prevalence of expiratory computed tomographic (CT) abnormalities, including malacia and air trapping, in patients with relapsing Polychondritis and to retrospectively determine the frequency with which expiratory abnormalities are accompanied by inspiratory abnormalities on CT scans. Materials and Methods: Institutional review board approval was obtained, and informed consent was not required for this retrospective HIPAA-compliant study. A computerized hospital information system was used to identify all patients with clinically diagnosed or biopsy-proved relapsing Polychondritis who were referred for CT airway imaging during a 17-month period. The study cohort comprised 18 patients (15 women, three men; mean age, 47 years; age range, 20–71 years). Multidetector helical CT was performed in all patients by using a standard protocol, which included end-inspiratory and dynamic expiratory volumetric imaging. Two observers who were blinded to the original scan interpre...
Nathalie Costedoatchalumeau - One of the best experts on this subject based on the ideXlab platform.
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tracheobronchial involvement of relapsing Polychondritis
Autoimmunity Reviews, 2019Co-Authors: Nina De Montmollin, Nathalie Costedoatchalumeau, Daniel Dusser, C Lorut, Jeremie Dion, Luc Mouthon, Guillaume Chassagnon, Mariepierre Revel, Xavier PuechalAbstract:Recent studies show that relapsing Polychondritis patients with tracheobronchial involvement are distinct from others in terms of clinical characteristics, therapeutic management, and disease evolution. Tracheobronchial involvement affects 20 to 50% of patients and may reveal the disease. It should be sought at the time of diagnosis and at each follow-up visit. Respiratory impairment is confirmed by computed tomography (CT) of the chest, including the cervical portion of the trachea, with end-inspiratory and dynamic expiratory scans, and pulmonary function tests. These investigations should be performed, even in asymptomatic patients, at the time of diagnosis, and repeated as necessary during follow-up. Bronchoscopy and a fortiori endoscopic intervention should be considered with caution and performed only by expert endoscopists after careful evaluation of the risks and benefits of such procedures, which can lead to damage or perforation of the airways and bronchospasm. Early detection and management of tracheobronchial involvement in relapsing Polychondritis has significantly improved the prognosis of patients, especially with the development of interventional fiberoptic bronchoscopy. However, relapsing Polychondritis-related morbidity and mortality are still elevated, particularly in tracheobronchial disease.
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the relapsing Polychondritis disease activity index development of a disease activity score for relapsing Polychondritis
Autoimmunity Reviews, 2012Co-Authors: Laurent Arnaud, H Devilliers, Stanford L Peng, Alexis Mathian, Nathalie Costedoatchalumeau, Jane H Buckner, Lorenzo Dagna, Clement J Michet, Aman Sharma, Ricard CerveraAbstract:Abstract Objective The rarity of relapsing Polychondritis (RP) has hindered the development of standardized tools for clinical assessment. Here, we describe the development of a preliminary score for disease assessing activity in RP, the Relapsing Polychondritis Disease Activity Index (RPDAI). Methods Twenty-seven RP experts participated in an international collaboration. Selection and definition of items for disease activity were established by consensus during a 4-round internet-based Delphi survey. Twenty-six experts assessed the Physician's Global Assessment (PGA) of disease activity on 43 test cases on a 0–100 scale, yielding a total of 1118 PGA ratings. The weight of each item was estimated by multivariate regression models with generalized estimating equation, using PGA as the dependent variable. Results Experts decided in consensus that the RPDAI should consider the 28-day period before each RPDAI assessment. Inter-rater reliability assessed by the intra-class correlation coefficient for the 1118 PGA ratings was 0.51 (CI95%: 0.41–0.64). The final RPDAI score comprised 27 items with individual weights ranging from 1 to 24 and a maximum theoretical RPDAI score of 265. Correlation between the RPDAI scores calculated based on the weights derived from the final multivariate model, and the 1118 PGA ratings was good (r = 0.56, p Conclusion We have developed the first consensus scoring system to measure disease activity in relapsing Polychondritis (see www.RPDAI.org for online scoring). This tool will be valuable for improving the care of patients with this rare disease.
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pediatric onset relapsing Polychondritis case series and systematic review
The Journal of Pediatrics, 2010Co-Authors: Alexandre Belot, Nathalie Costedoatchalumeau, Agnes Duquesne, Chantal Jobdeslandre, Sabaa Boudjemaa, Bertrand Wechsler, Pierre Cochat, Jeancharles Piette, Rolando CimazAbstract:Objectives To study the pediatric presentation and evolution of relapsing Polychondritis (RP), a rare inflammatory disease characterized by recurrent inflammation of cartilage. Study design We retrospectively collected data from 10 patients observed in 3 French hospitals for relapsing Polychondritis, with an age at onset Results The mean age at first symptoms was 8.6 years, and the sex ratio was 6 male patients and 4 female patients. Children came to medical attention with joint pain, ocular inflammation, and chondritis. Outcomes included severe visual impairment, chronic destructive chondritis, and 1 death caused by aortic dilatation. Treatment mainly consisted of non-steroidal-anti-inflammatory drugs, corticosteroids, and immunosuppressants. Growth was normal in 7 examined patients. Systematic literature review also suggested a high number of tracheostomy in pediatric cases, but this was not confirmed in our series. Conclusion RP in childhood shares the main clinical features of its adult counterpart, including destructive chondritis and systemic symptoms, but unlike adults, children frequently have a family history of autoimmunity and infrequently have other associated autoimmune diseases. RP can be fatal; close screening for complications is mandatory. Growth does not appear to be impaired by cartilage inflammation.
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treatment of relapsing Polychondritis with rituximab a retrospective study of nine patients
Arthritis & Rheumatism, 2009Co-Authors: Gaelle Leroux, Nathalie Costedoatchalumeau, Benoit Brihaye, Judith Cohenbittan, Zahir Amoura, Julien Haroche, Nicolas Limal, O Bletry, Jeancharles PietteAbstract:Objective Rituximab, a chimeric monoclonal antibody directed against the CD20 antigen expressed by B cells, is now considered an effective second-line therapy in various systemic diseases. We describe here the effects of rituximab in patients with relapsing Polychondritis. Methods This was a retrospective study of 9 patients with relapsing Polychondritis who received different regimens of rituximab in addition to their ongoing therapies. Clinical, laboratory, physiologic, and radiologic indicators were used to assess disease activity. We also examined their corticosteroid doses and any change in immunosuppressive agents. We then compared disease activity in the 6 months preceding rituximab administration and at 6 and 12 months after. Results At 6 months, 2 patients showed partial improvement, 4 were stable, and 3 had worsened disease; however, no patient had complete remission. At 12 months (after exclusion of the 3 patients whose disease had worsened at 6 months), 2 patients remained stable and 4 had worsened disease; however, there were no partial or complete remissions. B cells were counted in 8 patients during the first 6 months after treatment, and B cell depletion was observed in all of the patients. Conclusion Although we cannot rule out the possibility that rituximab had a small effect, our patients' clinical courses did not improve significantly with this treatment.
P Arlet - One of the best experts on this subject based on the ideXlab platform.
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biologics in relapsing Polychondritis a case series
Clinical and Experimental Rheumatology, 2013Co-Authors: Guillaume Moulis, L Sailler, G Pugnet, L Astudillo, P ArletAbstract:Objectives To describe the effects of biologics in an unbiased series of relapsing Polychondritis cases. Methods We extracted all the cases encoded 'Polychondritis' from the computerized medical files of our department. The relapsing Polychondritis diagnosis was confirmed using Damiani's criteria. Patients treated with biologics were evaluated for efficacy and adverse drugs reactions until October 2012. Results Nine patients were exposed to 22 biologics as corticosteroid-sparing drugs. Biologics were used at the same doses as in rheumatoid arthritis. Mean duration of exposure to biologics was 28 months. A TNF-antagonist was most frequently used as first-line biologic therapy (7/9), leading to partial or complete efficacy in six cases (85.7%). Loss of efficacy occurred in 5 cases. Abatacept (n=3) and tocilizumab (n=2) were effective as second-line biologic therapy while anakinra (n=2) and certolizumab (n=1) were not. Seven serious adverse drug reactions occurred, including 5 infections. Conclusions TNF-α antagonists may be proposed earlier in relapsing Polychondritis to spare corticosteroids. Switching to another biologic can be proposed in case of loss of efficacy. Tocilizumab or abatacept can be proposed as third-line therapy. The benefit-to-risk ratio of biologics in relapsing Polychondritis should be evaluated prospectively.
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Abatacept in relapsing Polychondritis
Annals of the rheumatic diseases, 2013Co-Authors: Guillaume Moulis, L Sailler, G Pugnet, L Astudillo, P ArletAbstract:Dear Editor, We read with great interest the open clinical trial of four relapsing Polychondritis (RP) patients treated with abatacept by Peng and Rodriguez recently published in the Annals of the Rheumatic Diseases .1 Indeed, as the authors pointed out, there is rational to block T-cell pathway in this disease, though the biologic agents most used as second-line therapy after corticosteroids (CS) are proinflammatory cytokines blockers as tumor necrosis factor (TNF) inhibitors or tocilizumab.2 Of note, rituximab seems not efficient in this disease.3 Despite this rational, the study by Peng and Rodriguez is the first report of abatacept use in RP since we reported a first case in 2010.4 Results from this nice small clinical trial using abatacept subcutaneously at the dose of 125 mg weekly are mitigated: three patients experienced a considerable improvement on ear, nose and throat (ENT) …