OVERVIEW
Recurrent polychondritis is a rare autoimmune disease characterized by inflammation of the cartilage of the ear, nose, throat, and trachea, predisposing to arthritis, scleral epiphora, scleritis, and uveitis. The disease has been referred to in the literature as polychondritis chondritis. Ocular manifestations can be the first manifestation of the disease but tend to follow systemic lesions. Ocular lesions occur in approximately 50% of cases and are characterized by conjunctivitis, scleritis, scleral episcleritis, peripheral ulcerative keratitis, and iridocyclitis, in addition to choroiditis, choroidal retinitis, neuro-ophthalmologic anomalies, optic neuritis, optic disc edema, proptosis, extraocular muscle paralysis, eyelid edema, and desiccating keratitis.
Etiology.
The etiology is unknown.
Symptoms
1. Ocular manifestations
Ocular manifestations can be the first manifestation of the disease but most often occur after systemic lesions. The incidence of ocular lesions is about 50%, and the main manifestations are conjunctivitis, scleritis, scleral episcleritis, peripheral ulcerative keratitis, iridocyclitis, and choroiditis, choroidal retinitis, optic neuritis, optic disc edema, proptosis, paralysis of the extraocular muscles, eyelid edema, and desiccating keratitis.
(1) Scleritis can be manifested as diffuse nodular or necrotizing mostly involving the anterior sclera, and scleral thinning and scleral softening or even scleral perforation can occur. Necrotizing scleritis is easy to be combined with systemic vasculitis, diffuse scleritis usually does not occur in patients with systemic vasculitis, nodular scleritis has no relationship with systemic vasculitis.
(2) Uveitis Iridocyclitis is a common manifestation, with recurrence, easy to cause concomitant cataract, often accompanied by scleritis, keratitis and retinopathy, and sometimes uveitis can be the initial manifestation of this disease. Sometimes uveitis can be the first manifestation of the disease. In addition, posterior uveitis, vitritis and other inflammation of the posterior segment of the eye can also occur.
(3) Keratitis The keratitis caused by this disease is mostly peripheral ulcerative keratitis, and in severe cases, it can lead to corneal perforation or corneal opacification and peripheral corneal thinning.
2. Non-specific systemic manifestations
Patients may have fever, weight loss, fatigue, night sweats, lymph node enlargement and other non-specific systemic manifestations.
(1) Chondritis of the ear is the most common. The onset of the disease is sudden, manifested as unilateral or bilateral redness, swelling and pain in the ear canal, and the inflammation is easy to recur or chronic, resulting in the destruction of cartilage, leading to the drooping of the ear canal, or a cauliflower-like appearance. As the outer ear is obscured by the deformed auricle, it may cause hearing loss, plasma otitis media, neurosensory deafness and vestibular dysfunction.
(2) Nasal chondritis Patients may have redness, swelling and pressure pain in the affected area, often accompanied by nasal congestion, runny nose, nosebleed, and saddle-shaped nasal deformity in the later stage.
(3) Joint involvement Patients may have joint involvement, manifesting as peripheral polyarthritis, which may involve the large joints or small joints, and may result in narrowing of the joint space, but it does not cause invasive changes or deformation of the joints. Arthritis is mostly acute and recurrent, and may be accompanied by rheumatoid arthritis.
(4) Involvement of larynx, trachea and bronchial tubes Patients often complain of hoarseness, cough, dyspnea, wheezing, and sometimes the destruction of cartilage can lead to the atrophy of the upper respiratory tract and cause death.
(5) Other systemic manifestations Patients may also present with aortitis, vasculitis involving small, medium and large blood vessels, myocarditis, bundle branch block, skin purpura, erythema nodosum, MAGIC syndrome (oral and genital ulcers and chondritis), glomerulonephritis, proteinuria, microscopic hematuria, encephalomyelopathy, stroke and meningitis.
Examination
1. Laboratory examination
The diagnosis of the disease is mainly based on the typical clinical manifestations. In some patients, there may be rapid blood sedimentation, mild normocytic and normocytic anemia, leukocytosis, thrombocytosis, polyclonal globulinemia.
2. Other auxiliary examinations
(1) Biopsy reveals the disappearance of basophilic staining material, focal or diffuse infiltration of polymorphonuclear leukocytes and monocytes (mainly CD4+ lymphocytes and macrophages), disruption of collagen matrix, fibrous granulomatous tissue hyperplasia and focal calcification.
(2) Imaging examinations Frontal and lateral neck and chest X-rays are helpful in detecting laryngotracheobronchial lesions, and examinations such as CT and magnetic resonance may also provide valuable information.
Diagnosis
The disease is diagnosed mainly on the basis of typical clinical manifestations. There are no standardized diagnostic criteria for this disease. If the patient’s clinical presentation is very clear, histologic examination may not be necessary for diagnosis.
Differential diagnosis
Recurrent polychondritis should be differentiated from infectious chondritis, vestibular or cochlear vasculitis, and inflammatory diseases of the larynx and trachea. The presence of recurrent scleritis or scleral epitheliitis uveitis should be differentiated from scleritis, scleral epitheliitis or uveitis associated with vertebral arthritis (lesions), rheumatoid arthritis, SjÖgren’s syndrome, Reiter’s syndrome, sarcoidosis, arteritis nodosa, Behcet’s disease, juvenile-onset chronic arthritis, Cogan’s syndrome, Wegner’s granulomatosis, and so on.
Recurrent polychondritis should be differentiated from systemic lupus erythematosus, rheumatoid arthritis, Behcet’s disease, SjÖgren’s syndrome, and mixed connective tissue disease.
Treatment
Prednisone can be used to treat patients with mild or moderate inflammation, and other immunosuppressive agents or combinations of other immunosuppressive agents can be given for severe cases.
Aminosulfone has a better effect on the systemic lesions of recurrent polychondritis, and azathioprine methotrexate phenylbutyrate nitrogen mustard and cyclophosphamide can also be tried in some patients with persistent inflammation.
For the ocular inflammation associated with this disease, in addition to local spot glucocorticosteroids, glucocorticosteroids (such as prednisone oral treatment) should generally be applied systemically; for some patients with intractable inflammation of the uvea, immunosuppressant treatments such as cyclophosphamide, azathioprine benzoate, azathioprine and other immunosuppressant treatments can be given or combined; for severe necrotizing sclero, aminoglycoside combined with glucocorticosteroids has a better effect on the treatment of severe necrotizing scleritis.
Prognosis
Patients tend to have recurrent episodes of chondritis, and long-term high-dose application of immunosuppressants can increase the chance of infection and the likelihood of tumorigenesis. Systemic vasculitis can lead to death in patients. Scleritis, ulcerative keratitis, and uveitis can all lead to severe vision loss or loss of vision.