The Experts below are selected from a list of 360 Experts worldwide ranked by ideXlab platform
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...
Masako Hara - One of the best experts on this subject based on the ideXlab platform.
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a case of Relapsing Polychondritis with bilateral sensorineural hearing loss and perforation of the nasal septum at the onset
Modern Rheumatology, 2007Co-Authors: Tokutaro Tsuda, Ayako Nakajima, Sayumi Baba, Kiyoko Tanohara, Ikuko Masuda, Toru Yamada, Kae Takagi, Takuya Yamakawa, Naoyuki Kamatani, Masako HaraAbstract:A 33-year-old woman suffered from epistaxis and perforation of the nasal septum. Based on a biopsy of nasal mucosa, Wegener's granulomatosis was suspected initially. Her nasal symptoms improved spontaneously, but tinnitus, hearing loss, and dizziness appeared within 3 months. Laboratory analyses revealed no inflammation, and antineutrophil cytoplasmic antibodies were negative. Audiometry revealed bilateral sensorineural hearing loss. A second biopsy of the nasal septum showed an inflammatory change in the cartilage. Thus we diagnosed early-stage Relapsing Polychondritis.
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...
Huijun Zhou - One of the best experts on this subject based on the ideXlab platform.
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18f fdg pet ct imaging of Relapsing Polychondritis a case report
Medicine, 2016Co-Authors: Huijun ZhouAbstract:BACKGROUND Relapsing Polychondritis (RP) is an uncommon autoimmune inflammatory disease that may affect cartilage throughout the body. CASE REPORT We report on a case of fever of unknown origin in which 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) was performed to make a diagnosis of RP. CONCLUSION Our case demonstrates that the use of 18F-FDG PET/CT is a useful diagnostic tool to accurately determine the extent of inflammation throughout the body which can be identified by an increased 18F-FDG uptake.
David Dcruz - One of the best experts on this subject based on the ideXlab platform.
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Relapsing Polychondritis and large vessel vasculitis
The Journal of Rheumatology, 2020Co-Authors: David Dcruz, Marcela A FerradaAbstract:Relapsing Polychondritis (RP) is a rare and in some cases fatal systemic inflammatory rheumatic disorder characterized by episodic inflammation of cartilage1,2,3. Common clinical features include chondritis of the nasal bridge, auricular cartilage, ocular and inner ear inflammation, arthritis, and involvement of the tracheobronchial tree. Destruction of the laryngeal and tracheal cartilage rings may lead to collapse of the airways and is associated with a high risk of morbidity and mortality4. Its rarity often leads to considerable delay in establishing a diagnosis1,2,3. RP may present with similar clinical features to other autoimmune rheumatic diseases such as granulomatosis with polyangiitis (GPA) and eosinophilic GPA (eGPA). Treatment for RP is usually with corticosteroids and immunosuppressive drugs but there are no randomized trials or specific guidelines for management, so treatment remains empirical and based on expert opinion5. Vascular involvement in RP ranges from 5% to 25%. The disease can affect small, medium, and large vessels. Although aortic involvement is particularly rare, it is associated with significant morbidity and mortality. The largest study in the literature evaluating aortic involvement by Le Besnerais, et al of 172 patients with RP found a prevalence of nonatheromatous aortic disease in 11 patients (6.4%)6. The pattern of … Address correspondence to Prof. D. D’Cruz, Consultant Rheumatologist, Louise Coote Lupus Unit, Guy’s Hospital, London SE1 9RT, UK. Email: david.d’cruz{at}kcl.ac.uk.
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the Relapsing Polychondritis damage index rpdam development of a disease specific damage score for Relapsing Polychondritis
Joint Bone Spine, 2019Co-Authors: Philippe Mertz, Lorenzo Dagna, Ricard Cervera, Alexandre Belot, Tyng Yu Chuah, Laura Damian, Debashish Danda, David Dcruz, Gerard Espinosa, Camille FrancesAbstract:Abstract Objectives Relapsing Polychondritis is a rare, multi-systemic and inflammatory condition of unknown origin. We currently lack a core set of measures to assess and follow damage in patients suffering from this condition. Our primary aim was to derive a disease-specific damage measuring tool for Relapsing Polychondritis, the Relapsing Polychondritis Damage Index (RPDAM). Methods We performed an international 4-round multicenter Delphi study during which experts were asked to rate the relevance of potential damage items for Relapsing Polychondritis (141 items were obtained from a literature review and 12 from expert suggestion), using a Likert Scale. The selection of items for each subsequent round was based on the median rating of each item. Results Twenty-four experts from 11 nationalities participated in round 1 and 22 in rounds 2, 3 and 4. From the initial 153 potential damage items, 44 items were selected during round 1, 30 items during round 2 and 16 during round 3. During round 4, we refined the index to a total of 17 items referring to ear nose and throat, eye, respiratory, cardiovascular and hematological systems as well as to treatment-related specific damage items. Conclusion We have developed by international consensus a scoring system to assess damage in patients with Relapsing Polychondritis. Following its validation, the RPDAM may contribute to improve the care of patients suffering from this rare condition as well as to standardize data collection for future clinical trials.
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Relapsing Polychondritis a clinical review for rheumatologists
Rheumatology, 2018Co-Authors: Jack Kingdon, Joseph Roscamp, Shirish Sangle, David DcruzAbstract:Relapsing Polychondritis (RPC) is a rare autoimmune rheumatic disorder that is traditionally classified as a systemic vasculitis. It is characterized by inflammation of cartilage, and typical presenting features include chondritis of the nasal bridge, auricular chondritis, ocular inflammation and involvement of the bronchial tree. Its rarity often leads to considerable delay in establishing a diagnosis and poses a significant management challenge to clinicians, as no conventional guidelines exist. This review summarizes the clinical features of RPC and provides guidance for rheumatologists on making the diagnosis and assessing organ involvement. The current state of RPC management is reviewed, with a focus on the use of the anti-TNF-α agents in patients with pulmonary involvement, the leading cause of mortality and morbidity in RPC.
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incidence and mortality of Relapsing Polychondritis in the uk a population based cohort study
Rheumatology, 2015Co-Authors: Nisha C Hazra, Alexandru Dregan, Judith Charlton, Martin Gulliford, David DcruzAbstract:OBJECTIVE Relapsing Polychondritis is a rare disease characterized by cartilage inflammation. Our aim was to estimate the incidence, prevalence and mortality of Relapsing Polychondritis and describe the clinical features of Relapsing Polychondritis in a large population. METHODS All participants diagnosed with Relapsing Polychondritis were sampled from the Clinical Practice Research Datalink. Prevalence and incidence rates for 1990-2012 were estimated. Relative mortality rates were estimated in a time-to-event framework using reference UK life tables. A questionnaire validation study assessed diagnostic accuracy. RESULTS There were 117 participants with Relapsing Polychondritis ever recorded. Fifty (82%) of 61 cases were validated by a physician and unconfirmed cases were excluded. The analysis included 106 participants (42 men, 64 women) diagnosed with Relapsing Polychondritis. The mean age (range) at diagnosis in men was 55 (range 17-81) years and in women 51 (range 11-79) years. The median interval from first symptom to diagnosis was 1.9 years. The incidence of Relapsing Polychondritis between 1990 and 2012 was 0.71 (95% CI 0.55, 0.91) per million population per year. There were 19 deaths from any cause. There were 16 observed deaths eligible for survival analysis and 7.4 deaths expected for the UK population of the same age, sex and period. The standardized mortality ratio was 2.16 (95% CI 1.24, 3.51), P < 0.01. Respiratory disease, cardiac conditions and cancer were the most frequent causes of death. CONCLUSION The incidence of Relapsing Polychondritis may be lower than previously estimated, and diagnostic misclassification and delay are common. Mortality in Relapsing Polychondritis is more than twice that of the general population.