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Last update 23 Jan 2025

Polychondritis, Relapsing

Last update 23 Jan 2025

Basic Info

Synonyms

Atrophic Polychondritides, Chronic, Atrophic Polychondritis, Chronic, CHONDROMALACIA, SYSTEMIC

+ [52]

Introduction

An acquired disease of unknown etiology, chronic course, and tendency to recur. It is characterized by inflammation and degeneration of cartilage and can result in deformities such as floppy ear and saddle nose. Loss of cartilage in the respiratory tract can lead to respiratory obstruction.

Drugs associated with Polychondritis, Relapsing

Abatacept

Target

CD80 x CD86

Mechanism

CD80 modulators [+1]

Active Org.

Bristol Myers Squibb Co. [+3]

Originator Org.

Bristol Myers Squibb Co.

Active Indication

Acute Graft Versus Host Disease [+19]

Inactive Indication

Alopecia Areata [+30]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date23 Dec 2005

Prednisone

Target

Mechanism

GR antagonists

Active Org.

Horizon Therapeutics USA, Inc. [+8]

Originator Org.

Merck Sharp & Dohme Corp.

Active Indication

Adrenal Hyperplasia, Congenital [+61]

Inactive Indication

acute leukemia [+16]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date21 Feb 1955

Clinical Trials associated with Polychondritis, Relapsing

NCT06561568

/ Active, not recruitingNot ApplicableIIT

Additional Effects of Kinesio Taping on Knee Joint Proprioception and Spatiotemporal Gait Parameters in Patient With Chrondromalacia Patellae

Chondromalacia patellae (CMP), also known as runner's knee, is characterized by anterior knee pain (AKP) and typically occurs in youngsters. Causes of chondromalacia patellae include micro trauma wear and tear, post traumatic injuries and muscular imbalance. In Chondromalacia patella pain is behind or around the patella caused by stress in the patellofemoral joint that usually provoked by climbing stairs, squatting, and sitting with flexed knees for longer time periods. Patients of CMP presents with patellar mal-tracking, muscular weakness, proprioceptive deficit. Exercise training has been proven beneficial for CMP patients but additive effect of kinesio taping along with exercise have not been seen yet in these patients. The study will help people with chondromalacia patellae to improve their quality of life. The findings will contribute to future research in chondromalacia patellae management.

Start Date10 Jun 2024

Sponsor / Collaborator

Foundation University Islamabad

NCT06019221

/ RecruitingNot ApplicableIIT

Pediatric Relapsing Polychondritis : Diagnosis and Management in a French Retrospective Study

The incidence in pediatrics is very low (about 3.5 per million per year according to a 2015 study) and therefore the data on the pathology very poor, especially on the therapeutic level.
Without appropriate treatment, the disabling sequelae, even involving the vital prognosis, are significant. However, in paediatrics, therapeutic habits have been extrapolated from adult data and lack precision.
Existing treatments are almost composed of immunomodulatory and/or immunosuppressive treatments. Different therapeutic lines have been introduced over the years and a better understanding of the pathology. More recently, biotherapies have been introduced in this pathology, but data on their effectiveness remain limited. Data on the evolution under therapy in children are thus still poor.
Complications related to the pathology that can jeopardize the vital prognosis and the response to treatment for this pathology deserve to be studied in order to be known and if possible avoided.
The aim of the study is to describe French practices and compare the lines of treatment proposed for juvenile atrophic polychondritis.

Start Date18 Feb 2023

Sponsor / Collaborator

Les Hopitaux Universitaires de Strasbourg

JPRN-UMIN000047098

/ RecruitingNot ApplicableIIT

Assessment of pulmonary function by time-of-flight sensor in patients with respiratory disease - Time-of-flight sensor for respiratory disease

Start Date07 Mar 2022

Sponsor / Collaborator

STMARIANNA University School of Medicine

100 Clinical Results associated with Polychondritis, Relapsing

100 Translational Medicine associated with Polychondritis, Relapsing

0 Patents (Medical) associated with Polychondritis, Relapsing

1,796

Literatures (Medical) associated with Polychondritis, Relapsing

01 Feb 2025·Practical Neurology

Bright ears on MRI: an imaging clue to relapsing polychondritis-associated encephalopathy

Article

Author: Bhatnagar, Priya ; Griffiths, Timothy D ; Reynolds, Gary ; Bolton, Clare ; Devine, Helen ; Ledingham, David ; Chambers, Anne ; Rayner, Fiona

01 Jan 2025·International Journal of Medical Sciences

Investigating the Process of Autoimmune Inner Ear Disease: Unveiling the Intricacies of Pathogenesis and Therapeutic Strategies

Review

Author: Zhang, Ping ; Wang, Mengmeng ; Kong, Chunyu ; Li, Qiang

Autoimmune inner ear disease (AIED) is a rare condition characterized by immune-mediated damage to the inner ear, leading to progressive sensorineural hearing loss (SNHL) and vestibular symptoms such as vertigo and tinnitus. This study investigates the pathogenesis and therapeutic strategies for AIED through the analysis of three cases with different underlying autoimmune disorders: rheumatoid arthritis, relapsing polychondritis, and IgG4-related disease. The etiology of AIED involves complex immunopathological mechanisms, including molecular mimicry and the "bystander effect," with specific autoantibodies, such as those against heat shock protein 70 (HSP70), playing a potential role in cochlear damage. Diagnosis remains challenging due to nonspecific symptoms and the lack of distinct biomarkers, emphasizing the need for comprehensive clinical evaluation and exclusion of other hearing loss causes. Treatment primarily involves immunosuppressive therapies, with glucocorticoids as the first line, effective in 70% of cases. However, resistance or partial response necessitates the use of additional agents like methotrexate and biologics such as anti-TNF and IL-6 receptor antagonists. Early intervention is crucial for favorable outcomes, as demonstrated in the studied cases, where timely corticosteroid and immunosuppressive treatments led to significant hearing improvement. The study underscores the importance of personalized treatment strategies based on individual immunologic profiles and comorbidities. Our findings highlight the heterogeneity of AIED and the potential for biologic therapies in refractory cases.

01 Dec 2024·La Revue de Médecine Interne

Aortites

Review

Author: Espitia, Olivier ; Toquet, Claire ; Jamet, Bastien ; Serfaty, Jean-Michel ; Agard, Christian

Aortitis is a rare disease entity of unknown prevalence. Primary aortitis mainly affects the thoracic aorta. They are most often diagnosed on imaging by grade III 18-FDG uptake of the aortic wall on PET, or by circumferential thickening>2.2mm on CT or MRI with late-stage contrast. More rarely, aortitis is histologically proven, as in some cases of clinically isolated aortitis discovered after planned aortic aneurysm surgery or during aortic dissection surgery. The most common histological types are granulomatous/giant cell or lymphoplasmacytic. Clinical signs associated with aortitis are often non-specific: asthenia, fever, dry cough, chest, back, lumbar or abdominal pain. Aortitis can be divided into different etiological categories: primary aortitis, which includes vasculitis with a preferential or exclusive tropism for the aortic wall, aortitis secondary to systemic or iatrogenic diseases, and infectious aortitis. The main etiologies of primary aortitis are giant cell arteritis (GCA), Takayasu arteritis (TA) or clinically isolated aortitis. Aortitis secondary to systemic diseases is seen in atrophying polychondritis, systemic lupus and inflammatory rheumatic diseases such as spondyloarthropathy and rheumatoid arthritis. In both ACG and AT, aortitis is a negative factor, characterized by a higher risk of relapse, cardiovascular complications and increased mortality. The management of aortitis is insufficiently codified, and relies on the control of cardiovascular risk factors, with particular monitoring of blood pressure and LDL cholesterol, and on corticosteroid therapy and immunosuppressive drugs, the use of which will depend on the disease associated with the aortitis, the initial severity and comorbidities.

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Polychondritis, Relapsing

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