Relapsing Polychondritis

Quigley, Maureen; Hansell, David M.

doi:10.1016/b978-1-4160-4048-4.50055-6

Cited by 1 publication

(2 citation statements)

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“…Its prevalence is estimated to be 2/million person-years in Hungary [96] and 3.5/million person-years in the United States [97]. The early symptoms of RP include one or more of the following: external ear pain that does not affect the non-cartilaginous lobule, temporary pain in one or more joints, nasal pain, throat pain, hoarse voice, eye involvement in the form of scleritis or episcleritis, vasculitis, skin symptoms, hearing impairment, dizziness and systemic symptoms such as fever and weight loss [98] [99]. These symptom(s) can appear as intermittent acute flares, and the disease follows a progressive course that can lead to complete destruction of the affected tissue [98].…”

Section: Relapsing Polychondritismentioning

confidence: 99%

“…However, this process leads to frequent misdiagnosis of RP in the initial stages, especially in cases where there is a single presenting symptom. The mean delay between the time a patient first presents with symptoms and the time a diagnosis for RP is made is 2.9 years [98]. The first clinical algorithm for confirming a diagnosis was published in 1976, in which 3 of the following 6 criteria were considered to be sufficient: (1) bilateral chondritis in the ears, (2) polyarthritis that is inflammatory and non-erosive, (3) nasal chondritis, (4) inflammation in the eyes, (5) chondritis involving cartilage in the larynx and/or the trachea and (6) damage to the cochlea or the vestibules in the ears [101].…”

Section: Relapsing Polychondritismentioning

confidence: 99%

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Cartilage diseases

2018

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Hyaline cartilages, fibrocartilages and elastic cartilages play multiple roles in the human body including bearing loads in articular joints and intervertebral discs, providing joint lubrication, forming the external ears and nose, supporting the trachea, and forming the long bones during development and growth. The structure and organization of cartilage's extracellular matrix (ECM) are the primary determinants of normal function. Most diseases involving cartilage lead to dramatic changes in the ECM which can govern disease progression (e.g., in osteoarthritis), cause the main symptoms of the disease (e.g., dwarfism caused by genetically inherited mutations) or occur as collateral damage in pathological processes occurring in other nearby tissues (e.g., osteochondritis dissecans and inflammatory arthropathies). Challenges associated with cartilage diseases include poor understanding of the etiology and pathogenesis, delayed diagnoses due to the aneural nature of the tissue and drug delivery challenges due to the avascular nature of adult cartilages. This narrative review provides an overview of the clinical and pathological features as well as current treatment options available for various cartilage diseases. Late breaking advances are also described in the quest for development and delivery of effective disease modifying drugs for cartilage diseases including osteoarthritis, the most common form of arthritis that affects hundreds of millions of people worldwide.

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