Uveitis associated with relapsing polychondritis

Introduction to uveitis associated with recurrent polychondritis Relapsing polychondritis is a rare autoimmune disease, mainly characterized by inflammation of the auricle, nose, throat, tracheal cartilage, prone to arthritis, scleral inflammation, scleritis and uveitis. . basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: optic neuritis optic disc edema

Causes of uveitis associated with recurrent polychondritis

Pathology (30%):

The disease may be an autoimmune disease, and the antigens associated with the disease may be type II, type IV and type VI collagen, which may be mediated by immune complexes. Delayed allergic reactions may also play a role in their onset. Certainly works.

Epidemiology (30%):

Uveitis associated with relapsing polychondritis is rare, with more than 600 cases reported worldwide. Most reports have similar rates of male to female, but there are also reports that female patients are three times as many as male patients. Can be seen at any age, but more common in adults 40 to 59 years old. We have not seen uveitis caused by this disease. It is reported that this disease and its associated uveitis have been found in Taiwan.

Low autoimmunity (20%):

Due to the low immunity of the body, it is easy to cause this disease.

Uveitis prevention associated with relapsing polychondritis

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.

Uveitis complications associated with recurrent polychondritis Complications optic neuritis optic disc edema

Complications: Optic neuritis and optic disc edema are more common.

Recurrent multi-chondritis associated with uveitis symptoms Common symptoms Nasal bleeding Scleral perforation Deafness Tired night sweats conduction block Nasal weight loss Protein urinary auricle redness

Eye performance

Ocular manifestations may be the initial manifestations of the disease, but more often after systemic lesions, the incidence of eye lesions is about 50%, mainly manifested as conjunctivitis, scleritis, scleral inflammation, peripheral ulcerative keratitis, iris ciliary body Inflammation, in addition to choroiditis, chorioretinitis, neuro-ophthalmology abnormalities, optic neuritis, optic disc edema, ocular protrusion, extraocular muscle paralysis, orbital edema, dry keratitis (Table 1).

(1) scleritis: the incidence of scleritis is about 10%, can be diffuse, nodular or necrotizing, involving the anterior sclera, scleral thinning, scleral softening or even scleral perforation, necrotizing scleritis It is easy to combine with systemic vasculitis. Diffuse scleritis generally does not occur in patients with systemic vasculitis. Nodular scleritis has nothing to do with systemic vasculitis.

(2) uveitis: iridocyclitis is a common manifestation that can occur in 30% of patients, iridocyclitis has recurrence, easy to cause complicated cataract, often accompanied by scleritis, keratitis And retinopathy, sometimes uveitis can be the initial manifestation of the disease, in addition, there may be inflammation of the posterior segment of the eye such as posterior uveitis and vitreitis.

(3) keratitis: the keratitis caused by this disease is mostly peripheral ulcerative keratitis. In severe cases, the cornea can be perforated, which can cause corneal vasospasm and thinning of the surrounding cornea.

2. Non-specific whole body performance

Patients may have non-specific systemic manifestations such as fever, weight loss, fatigue, night sweats, and swollen lymph nodes.

(1) auricular cartilage inflammation: auricular cartilage inflammation is the most common, accounting for 80% to 90% of the total number of patients, sudden onset, manifested as unilateral or bilateral auricle swelling and pain, inflammation is prone to recurrence or chronic, resulting in cartilage Destruction, resulting in a sagging of the auricle, or a broccoli-like appearance, due to the deformed auricle of the outer ear, can cause hearing loss, serous otitis media, neurosensory deafness and vestibular dysfunction.

(2) nasal chondritis: patients may have redness and tenderness in the affected area, often accompanied by nasal congestion, runny nose, nose bleeding, and later saddle-shaped nasal deformity, the incidence rate is 30% to 60%.

(3) joint involvement: about 30% of patients with joint involvement, typically manifested as peripheral polyarthritis, may involve large joints, may also involve small joints, may appear narrow joint space, but does not cause erosional changes, Does not cause joint deformation, arthritis is often an acute recurrent episode, but also accompanied by rheumatoid arthritis.

(4) Laryngeal, tracheal, bronchial involvement: patients complained of hoarseness, cough, difficulty breathing, wheezing, and sometimes due to cartilage damage can cause atrophy of the upper respiratory tract and cause death.

(5) Other systemic manifestations: patients may have aortitis, involving large, medium and small blood vessel vasculitis, myocarditis, bundle branch block, skin purpura, nodular erythema, MAGIC syndrome (oral, genital ulcer) And chondritis), glomerulonephritis, proteinuria, microscopic hematuria, cranial neuropathy, stroke, meningitis, etc.

Examination of uveitis associated with recurrent polychondritis

The diagnosis of this disease is mainly based on typical clinical manifestations. At present, there is no positive laboratory examination for this disease. In some patients, erythrocyte sedimentation rate, mild normal erythrocyte and erythrocyte pigmentary anemia, leukocytosis, thrombocytosis, and more may occur. Clonal globulinemia.

Biopsy

It was found that the disappearance of basophilic staining substances revealed infiltration of focal or diffuse polymorphonuclear leukocytes and monocytes (mainly CD4 lymphocytes and macrophages), destruction of collagen matrix, fibrous granulation tissue proliferation and focal calcification. .

2. Imaging examination

X-ray of the neck and chest, lateral examination can help find throat, trachea, bronchial lesions, CT and magnetic resonance examinations can also provide valuable information.

Diagnosis and diagnosis of uveitis associated with recurrent polychondritis

Diagnostic criteria

The disease is mainly diagnosed according to typical clinical manifestations. There is no uniform diagnostic standard for this disease. McAdam et al. developed a diagnostic standard in 1976, which was revised by Hochberg. This standard includes 6 (Table 2), such as If there are 3 or more of the patients, and the histological examination finds a positive result, the diagnosis of recurrent polychondritis can be made. If the clinical manifestation of the patient is very clear, the histological examination is not necessary at the time of diagnosis.

Differential diagnosis

Recurrent polychondritis should be differentiated from infectious chondritis, vestibular or cochlear vasculitis, inflammatory diseases of the larynx and trachea, recurrent scleritis or scleral inflammation, and uveitis should be associated with vertebral joints. Inflammation (lesion), rheumatoid arthritis, Sjögren syndrome, Reiter syndrome, sarcoma-like disease, nodular arteritis, Behcet's disease, juvenile chronic arthritis, Cogan's syndrome, Wegner's granulomatosis, etc. Scleritis, scleral inflammation or uveitis.

About 25% of patients with relapsing polychondritis have collagen vascular disease, such as systemic lupus erythematosus, rheumatoid arthritis, Behcet's disease, Sjögren's syndrome, mixed connective tissue disease, and should pay attention to them in diagnosis and differential diagnosis. The overlap between them.

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