Relapsing Polychondritis: An Updated Review

Abstract

Relapsing polychondritis is an immune-mediated systemic disease characterized by recurrent episodes of inflammation of cartilaginous and proteoglycan-rich tissues, resulting in progressive anatomical deformation and functional impairment of the involved structures. Auricular and nasal chondritis and/or polyarthritis represent the most common clinical features, but potentially all types of cartilage may be involved. Because of the pleomorphic nature of the disease, with non-specific symptoms at the onset, the diagnosis of relapsing polychondritis is often delayed. In this review article we provide a comprehensive look into clinical presentation, laboratory and instrumental investigations, diagnostic criteria, and therapeutic options.

A Case of Undifferentiated Connective Tissue Disease with Bilateral Auricular Polychondritis Manifestations: A Rare Clinical Association

“…In the present case, the polychondritis-like involvement was successfully managed with an escalation in corticosteroid therapy, highlighting the responsiveness of these symptoms to immunosuppression. This observation is consistent with prior reports suggesting that corticosteroids are effective in mitigating inflammatory symptoms in cases of relapsing polychondritis associated with connective tissue diseases [ 8 , 9 ].…”

A Case of Undifferentiated Connective Tissue Disease with Bilateral Auricular Polychondritis Manifestations: A Rare Clinical Association

“…In the present case, the polychondritis-like involvement was successfully managed with an escalation in corticosteroid therapy, highlighting the responsiveness of these symptoms to immunosuppression. This observation is consistent with prior reports suggesting that corticosteroids are effective in mitigating inflammatory symptoms in cases of relapsing polychondritis associated with connective tissue diseases [ 8 , 9 ].…”

Bilateral pyogranulomatous otitis externa with putative cartilage destruction in a dog: A severe case of auricular chondritis?

“…Finally, the resolution of pain and lesions with an immunosuppressive dose of prednisolone is in agreement with the previously reported case of canine auricular chondritis, yet the pinnal deformity and ear canal stenosis were unexpected sequelae 3 . Deformity of the ear and closure of the external auditory meatus have been described in severe cases of human auricular chondritis 1 . Different hypotheses can be formulated to explain the lack of response to ciclosporin, including the possibility that a longer treatment period might be required to see a more significant response.…”

“…In the present case, a definitive diagnosis could not be established as a consequence of the lack of cartilage tissue in the histopathological sections. However, the pyogranulomatous inflammation and multifocal fibrosis were similar to those reported in canine, feline and human auricular chondritis 1,2,9 . A surgical biopsy sample may have been more likely to identify chondritis.…”

Relapsing polychondritis – analysis of symptoms and criteria

“…Auricular chondritis was present in 90% of our patients in the course of the disease, similarly to data from the literature (85–95%) [ 1 , 3 ] and was the most frequent manifestation of RP. Onset of inflammation is abrupt, mono- or bilateral.…”

A Case of Undifferentiated Connective Tissue Disease with Bilateral Auricular Polychondritis Manifestations: A Rare Clinical Association

“…In the present case, the polychondritis-like involvement was successfully managed with an escalation in corticosteroid therapy, highlighting the responsiveness of these symptoms to immunosuppression. This observation is consistent with prior reports suggesting that corticosteroids are effective in mitigating inflammatory symptoms in cases of relapsing polychondritis associated with connective tissue diseases [ 8 , 9 ].…”

Bilateral pyogranulomatous otitis externa with putative cartilage destruction in a dog: A severe case of auricular chondritis?

“…Finally, the resolution of pain and lesions with an immunosuppressive dose of prednisolone is in agreement with the previously reported case of canine auricular chondritis, yet the pinnal deformity and ear canal stenosis were unexpected sequelae 3 . Deformity of the ear and closure of the external auditory meatus have been described in severe cases of human auricular chondritis 1 . Different hypotheses can be formulated to explain the lack of response to ciclosporin, including the possibility that a longer treatment period might be required to see a more significant response.…”

“…In the present case, a definitive diagnosis could not be established as a consequence of the lack of cartilage tissue in the histopathological sections. However, the pyogranulomatous inflammation and multifocal fibrosis were similar to those reported in canine, feline and human auricular chondritis 1,2,9 . A surgical biopsy sample may have been more likely to identify chondritis.…”

Relapsing polychondritis – analysis of symptoms and criteria

“…Auricular chondritis was present in 90% of our patients in the course of the disease, similarly to data from the literature (85–95%) [ 1 , 3 ] and was the most frequent manifestation of RP. Onset of inflammation is abrupt, mono- or bilateral.…”

A Case of Undifferentiated Connective Tissue Disease with Bilateral Auricular Polychondritis Manifestations: A Rare Clinical Association

“…In the present case, the polychondritis-like involvement was successfully managed with an escalation in corticosteroid therapy, highlighting the responsiveness of these symptoms to immunosuppression. This observation is consistent with prior reports suggesting that corticosteroids are effective in mitigating inflammatory symptoms in cases of relapsing polychondritis associated with connective tissue diseases [ 8 , 9 ].…”

Bilateral pyogranulomatous otitis externa with putative cartilage destruction in a dog: A severe case of auricular chondritis?

“…Finally, the resolution of pain and lesions with an immunosuppressive dose of prednisolone is in agreement with the previously reported case of canine auricular chondritis, yet the pinnal deformity and ear canal stenosis were unexpected sequelae 3 . Deformity of the ear and closure of the external auditory meatus have been described in severe cases of human auricular chondritis 1 . Different hypotheses can be formulated to explain the lack of response to ciclosporin, including the possibility that a longer treatment period might be required to see a more significant response.…”

“…In the present case, a definitive diagnosis could not be established as a consequence of the lack of cartilage tissue in the histopathological sections. However, the pyogranulomatous inflammation and multifocal fibrosis were similar to those reported in canine, feline and human auricular chondritis 1,2,9 . A surgical biopsy sample may have been more likely to identify chondritis.…”

Relapsing polychondritis – analysis of symptoms and criteria

“…Auricular chondritis was present in 90% of our patients in the course of the disease, similarly to data from the literature (85–95%) [ 1 , 3 ] and was the most frequent manifestation of RP. Onset of inflammation is abrupt, mono- or bilateral.…”