Relapsing polychondritis scleritis

Introduction to recurrent polychondritis scleritis Recurrent polychondritis (RP) is a rare, unexplained multisystem disease. Cartilage and other tissues containing high concentrations of glycosaminoglycans are the main target organs, including the auricle, nasal cartilage, eustachian tube, larynx, trachea, main bronchus, joints, blood vessels and sclera. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: retinitis optic disc edema

Causes of recurrent polychondritis scleritis

Causes:

The etiology is unclear, but systemic factors are suggested by the disease site and often accompanied by systemic diseases. The presence of type II collagen antibodies on glycosaminoglycans and type II collagen is evidence of immune disorders.

Incidence machine:

It belongs to immune disorder diseases, and the specific pathogenesis is still unclear.

Recurrent polychondritis scleritis prevention Trying to avoid causing the body's immune response is the key to preventing autoimmune diseases. 1. Eliminate and reduce or avoid the disease factors, improve the living environment, develop good living habits, prevent infection, pay attention to food hygiene, and rational diet. 2. Insist on exercising, increase the body's ability to resist disease, do not over-fatigue, excessive consumption, quit smoking and alcohol. 3. Early detection and early diagnosis and early treatment, establish confidence in the fight against disease, adhere to treatment. Stay optimistic. 4. Prevention of infection, especially prevention of streptococcal infection is an important part of autoimmune rheumatism and co-morbidity.

Recurrent polychondritis scleritis complications Complications retinitis optic disc edema

The eyes showed retinitis, extraocular muscle paralysis, optic disc edema and so on.

Recurrent cartilage inflammation scleritis symptoms common symptoms saddle nose keratitis convulsion external auditory canal stenosis

1. Eye performance: The most common eye lesions are scleritis or scleral inflammation, but anterior uveitis, retinitis, extraocular muscle paralysis, optic neuritis, conjunctivitis, keratitis and exophthalmos can also occur. .

(1) Scleritis: The incidence of scleritis in RP patients is 14%, and the incidence of RP in patients with scleritis is 0.96% to 2.06%. Scleritis can be diffuse, nodular, necrotizing anterior scleritis and Scleritis, often associated with other active lesions such as nasal chondritis and arthritis, is often recurrent, associated with anterior uveitis and marginal ulcerative keratitis.

(2) Scleral outer inflammation: scleral inflammation is a common manifestation of RP. According to Foster et al, the incidence of scleral inflammation in RP patients is 39%, which may be simple or nodular, monocular or binocular, easy relapse.

(3) Keratitis: is a common ocular manifestation associated with RP scleritis.

(4) Non-granulomatous anterior uveitis: another common ocular manifestation associated with RP scleritis.

2. Non-eye manifestations: Auricular chondritis is the most common manifestation of RP. About 88.6% of patients have external ear cartilage inflammation, which may have sudden or severe pain in the unilateral or bilateral auricles, tenderness, redness and swelling, and long-term recurrence. Inflammation can cause cartilage absorption, loosening the ear shape, softening and sagging, about 50% of patients involve the middle ear, inner ear, causing hearing loss, vestibular dysfunction, eustachian tube obstruction, external auditory canal stenosis, conductive deafness Etc. 50% of patients develop nasal chondritis and can develop further to form saddle-nose malformations. 50% of patients develop laryngotracheitis. Severe laryngeal lesions can cause hoarseness, fatal respiratory collapse or suffocation, 10% of which The patient died of this disease, and the further development of bronchial inflammation can cause pneumonia and respiratory failure. 50% to 80% of patients may develop joint lesions, which are non-erosive, non-deformable and RF-negative monoarthritis or polyarticular inflammation. The most commonly affected are the costal cartilage joints, 25% of patients with cardiovascular manifestations of macrovascular inflammation, aortic inflammatory disease with dysfunction caused by thoracic and abdominal aortic aneurysm, aortic regurgitation and blood reflux, to Aortic root involvement and cardiac conduction system near the site of the lesion and electrocardiographic abnormalities.

Clinical manifestations of small and medium vascular inflammation, including skin lesions, kidney and nervous system disorders, skin lesions including urticaria purpura, nodular erythema, reticular bluish, angioedema, migratory thrombophlebitis, etc. Lesions can occur in the proliferative glomerulonephritis formed by the crescent, causing proteinuria or microscopic hematuria and IgA nephropathy, neurological disorders including neurological damage to the brain, II, VI, VII, VIII, and convulsions , hemiplegia, movement disorders and sensation - motor neuropathy, occasional fever, oral lesions and genital ulcers.

Examination of recurrent polychondritis scleritis

1. Blood routine examination: positive pigment or hypochromic anemia, increased white blood cells, eosinophilia, increased DR (ESR) and proteinuria.

2. Pathological examination: Under light microscopy, the changes of cartilage include the disappearance of matrix staining, in which neutrophils and lymphocytes are infiltrated in the shallow layer, and finally the normal tissue structure is destroyed, followed by fibrosis, and the surface layer of chondrocytes is displayed under electron microscope. Abnormal changes in matrix and elastic fibers.

3. X-ray examination: severe tracheal stenosis can be found, and slight airway obstruction can be found in CT scan and lung function tests.

Diagnosis and diagnosis of recurrent polychondritis scleritis

The clinical features of RP are recurrent ear, nasal cartilage, RF negative, inflammatory polyarthritis, inflammation of the ocular tissue, respiratory chondritis, cochlear and/or vestibular damage, and one of the following conditions in clinical practice, RP The diagnosis can be established:

1 The above 3 or more clinical features appear;

2 at least 1 clinical feature and biopsy tissue found vasculitis;

3 found chondritis in 2 or more sites, and sensitive to glucocorticoids and dapsone drugs.

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