ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (2)
Relapsing polychondritis - External and Internal Eye
See also in: Skin,Cellulitis DDx
Print
Other Resources UpToDate PubMed

Relapsing polychondritis - External and Internal Eye

See also in: Skin,Cellulitis DDx
Print Images (2)
Contributors: Brandon D. Ayres MD, Christopher Rapuano MD, Harvey A. Brown MD, Sunir J. Garg MD, Lauren Patty Daskivich MD, MSHS
Other Resources UpToDate PubMed

Synopsis

Relapsing polychondritis is an episodic, progressive inflammatory autoimmune disorder involving cartilaginous structures such as the ears, nose, larynx, and tracheobronchial tree. It often affects multiple other organ systems as well. Signs and symptoms of relapsing polychondritis include auricular, nasal, costal and laryngotracheobronchial chondritis, arthropathy, ocular inflammation (eg, scleritis), audiovestibular damage, cardiovascular disease (aortitis, aortic aneurysm, or regurgitation), cutaneous vasculitis, CNS disturbances (eg, cranial neuropathies, seizures), and renal involvement. Flares are accompanied by fever, asthenia, weight loss, and sometimes liver or lymph node enlargement. Relapsing polychondritis is frequently associated with other autoimmune diseases, Behçet disease, and myelodysplastic syndromes. Complications include saddle-nose deformity/joint deformity, vertigo, tinnitus, pulmonary infection, laryngotracheobronchial stricture, scleritis or iritis, epiglottitis, blindness, valve regurgitation, aortic dissection, renal failure, and death. Relapsing polychondritis is more common in whites and the middle-aged.

Ocular manifestations of relapsing polychondritis are frequent (59% of cases) and include conjunctivitis, keratitis, scleritis, episcleritis, uveitis, retinopathy (cotton wool spots and intraretinal hemorrhages), and in those with associated vasculitis one can see optic nerve involvement and cranial nerve palsies.

Nasal pain, hoarseness, throat pain, and difficulty talking are common presenting symptoms.

Several clinical criteria have been developed for establishing the diagnosis of relapsing polychondritis (see Diagnostic Pearls).

Codes

ICD10CM:
M94.1 – Relapsing polychondritis

SNOMEDCT:
72275000 – Relapsing polychondritis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Updated: 10/26/2017
Copyright © 2020 VisualDx®. All rights reserved.
Relapsing polychondritis - External and Internal Eye
See also in: Skin,Cellulitis DDx
Print 2 Images
View all Images (2)
(with subscription)
Relapsing polychondritis : Fever, Ear, Hoarseness, Nose, Painful skin lesions, Arthralgia, Dysphagia, Otalgia, Wheezing
Clinical image of Relapsing polychondritis
Copyright © 2020 VisualDx®. All rights reserved.