Relapsing polychondritis

Introduction to recurrent polychondritis Recurrent polychondritis is a rare disease that affects multiple systems and systems. It has progressive inflammatory destructive lesions with recurrent and remission, involving cartilage and other systemic connective tissues including the ear, nose, eyes, joint respiratory tract and cardiovascular system. Wait. Jaksch-Wartenhorst, 1923: Describes the clinical manifestations of this disease for the first time and named it polychondropathia. In 1960 Pearson analyzed 12 patients and named it recurrent polychondritis. The etiology and pathogenesis are still unclear, but more and more evidence indicates that the disease has autoimmune mechanisms involved. basic knowledge The proportion of illness: 0.02% Susceptible people: no special people Mode of infection: non-infectious Complications: optic disc edema retinitis

Recurrent polychondritis

(1) Causes of the disease

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.

(two) pathogenesis

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

Recurrent polychondritis prevention

This disease is an immune disorder, and attention should be paid to enhancing immunity.

Recurrent polychondritis complication Complications optic disc edema retinitis

The eyes showed retinitis, extraocular muscle paralysis, optic disc edema, and the like.

Recurrent multi-chondritis symptoms Common symptoms Scleral outer inflammation Auricular pain Saddle-nose conduction deafness 2nd costal cartilage pain Proteinuria twitching Eustachian tube blockage

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 posterior scleritis, often associated with other active lesions such as nasal chondritis and arthritis. Scleritis is mostly recurrent and is associated with anterior uveitis and marginal ulcerative keratitis.

(2) scleral outer inflammation: scleral outer 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, and easy to 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, accompanied by redness and swelling. Long-term repeated inflammation can cause cartilage absorption, making the shape of the ear loose, soft and drooping. About 50% of patients involve the middle ear and inner ear, causing hearing loss, vestibular dysfunction, eustachian tube obstruction, external auditory canal stenosis, and conductive deafness. Nasal cartilage inflammation occurs in 50% of patients and can be further developed to form saddle nose deformities. Laryngeal bronchitis occurs in 50% of patients, and severe laryngeal lesions can cause hoarseness, fatal airway collapse, or suffocation, with 10% of patients dying. Further development of bronchial inflammation can cause pneumonia and respiratory failure. 50% to 80% of patients can develop joint lesions, which are non-erosive, non-deformable and RF-negative monoarthritis or polyarticular inflammation, the most commonly affected are costal cartilage joints. 25% of patients have a cardiovascular manifestation of macrovascular inflammation. Aortic inflammatory disease with dysfunction caused by thoracic and abdominal aortic aneurysm, aortic regurgitation and blood reflux, as well as aortic root involvement and lesions in the vicinity of the cardiac conduction system, ECG abnormalities.

Clinical manifestations of small and medium vascular inflammation, including skin lesions, kidney and nervous system disorders. Skin lesions include urticaria purpura, nodular erythema, reticular leukoplakia, angioedema, and migratory thrombophlebitis. Renal lesions may present with focal proliferative glomerulonephritis formed by the crescent, causing proteinuria or microscopic hematuria and IgA nephropathy. Nervous system disorders include damage to the cranial nerves of II, VI, VII, and VIII, as well as convulsions, hemiplegia, ataxia, and sensory-motor neuropathy. Occasionally there are fever, oral lesions and genital ulcers.

Recurrent polychondritis

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

2. Pathological examination: Under the light microscope, the changes of cartilage include the disappearance of matrix staining, in which the neutrophils and lymphocytes are mainly infiltrated, and finally the normal tissue structure is destroyed, followed by fibrosis. Electron microscopy showed abnormal changes in the surface layer of chondrocytes and elastic fibers. X-ray examination: severe tracheal stenosis can be found, and mild airway obstruction can be found in CT scan and lung function tests.

Recurrent multi-chondritis diagnosis

Generally not confused with other diseases.

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