What Is Relapsing Polychondritis?

Relapsing polychondritis (RP) is a rare disease that affects the entire system and multiple systems. It has recurrent and recurrent progressive inflammatory destructive lesions that involve cartilage and other systemic connective tissues, including ears, nose, eyes, joints, Respiratory and cardiovascular systems. The clinical manifestations are osteochondritis of the ear, nose, and respiratory tract, accompanied by symptoms of organ involvement such as eyes and ear vestibules. Polyarthritis and vascular involvement are also more common.

Basic Information

English name: relapsing polychondritis
Visiting department: Division of Rheumatology

Common locations: Ear, nose, eyes, joints, respiratory tract, cardiovascular
Common symptoms: Sudden pain, swelling, saddle nose deformity, joint pain and tenderness in the outer ear wheel

Causes of recurrent polychondritis

The cause is unknown until now, and may be related to trauma, infection, allergy, alcohol abuse, taking hydralazine hydrochloride, etc. It is also believed to be related to mesoderm synthesis disorders or abnormal proteolytic enzymes, but through years of clinical characteristics, laboratory tests and pathology Research is increasingly suggesting that it is an immune-mediated disease, including humoral and cellular immunity.

Clinical manifestations of relapsing polychondritis

Osteochondritis

Ottochondritis is the most common symptom. It is the first symptom in 39% of cases. It is characterized by sudden pain, swelling, redness, and hotness in the outer ear wheel. The inflammation can resolve on its own or through treatment. After repeated attacks, the outer ear Guo became soft and collapsed. Sudden deafness and dizziness due to the structure of the ear vestibule or inner ear arterial vasculitis. Involved in 85% of the disease course. Onset is relatively sudden, often symmetry, and rarely unilateral. Acute episodes include Guo Hong, swelling, heat, pain, and erythema nodules. Lesions can be localized or diffuse.

2. Hearing and / or vestibular function involvement

Lesions invade the external auditory canal or eustachian tube, leading to stenosis or occlusion, causing hearing damage; lesions involving the middle and inner ears can manifest as hearing and / or vestibular function impairment; when combined vasculitis affects the branches of the inner auditory artery, Hearing abnormalities and impaired vestibular function may also occur. It is often accompanied by rotational dizziness, ataxia, nausea and vomiting.

3. Rhin chondritis

Sudden onset, with pain and redness, which resolved after a few days. Such as repeated attacks can cause localized collapse of nasal cartilage, forming saddle nose deformities. Even some patients may suddenly collapse the nose bridge within 1-2 days of onset. Patients are often accompanied by nasal congestion, nasal secretions, and scleroderma.

4. Eye inflammatory lesions

Mainly manifested as inflammation of the attachment of the eye, which can be unilateral or symmetrical. The most common are conjunctivitis, keratitis, iridocyclitis, scleritis, and uveitis. Retinopathy often occurs, such as omental microaneurysms, bleeding and exudation, omental vein occlusion, arterial embolism, retinal detachment, optic neuritis and ischemic optic neuritis.

5. Joint disease

Polyarthritis is the second common first-onset disease of this disease, which is typically manifested by migratory, asymmetric, non-deformable arthritis, which can affect the large and small joints of the periphery or axis. Respiratory chondritis can cause atrophy and collapse of nasal cartilage, manifested as saddle nose deformities. Throat, trachea, and bronchial involvement can cause hoarseness, trachea, tenderness on the thyroid cartilage, cough, wheezing, or wheezing. The most commonly involved joints are the metacarpophalangeal, proximal interphalangeal, and knee joints, followed by the ankle, wrist, and elbow joints. It can also involve joints near the sternum, such as costal cartilage, sternal stem, and sternoclavicular joints. The sacroiliac joint and the pubic bone can also be involved in RP. Local pain and tenderness may be associated with swelling, and RP patients may also be accompanied by destructive joint disease such as adult psoriatic arthritis, juvenile rheumatoid arthritis, Wright syndrome, Sjögren's syndrome, ankylosing spondylitis Wait.

6. Laryngeal, tracheal and bronchial tree cartilage lesions

Most patients complain of chronic cough and sputum, followed by shortness of breath, often diagnosed as chronic bronchitis, which lasts for 6 months to decades, and eventually develops dyspnea, recurrent respiratory infections and wheezing, and sometimes pretracheal and thyroid cartilage Tenderness, hoarseness, or aphasia.

7. Cardiovascular disease

Recurrent polychondritis can also affect the cardiovascular system, with a rate of 30%, including aortic aneurysms, aortic valve embolization, small or large vessel inflammation, and heart valve damage, pericarditis, and myocardial ischemia. Can cause death. Cardiovascular complications: cardiovascular collapse caused by complete conduction block and acute aortic insufficiency; aortic valve rupture. Involvement of large blood vessels can cause vascular aneurysms (aorta, subclavian artery), or thrombosis due to vasculitis or coagulopathy. Ascending aortic aneurysms can occur in some cases, and aneurysms in the chest, abdomen, aorta, and subclavian arteries.

8. Skin

10% are the first symptoms. Recurrent polychondritis can have a variety of skin and mucosal lesions, and the lesions are non-specific, such as nodular erythema, panniculitis, reticular plaques, urticaria, cutaneous polyarteritis nodules, and aphthous ulcers. .

9. Nervous system

Acute or subacute lesions of the brain nerves by , , , , and can cause ophthalmoplegia, optic neuritis, facial paralysis, hearing loss and dizziness. Other neurological complications include hemiplegia, chronic headaches, ataxia, seizures, insanity, dementia, and meningoencephalitis.

10. Kidney

The most common types of pathological tissues are mild mesangial proliferative and focal segmental crescent glomerulonephritis. Others include glomerulosclerosis, IgA nephropathy, and interstitial tubular nephritis.

11. Other

Anemia and weight loss are the most common systemic symptoms, often accompanied by fever during the acute episode. Muscle pain and liver damage can also occur. In general, laboratory abnormalities are non-specific, such as most patients with chronic anemia.

Recurrent polychondritis examination

1.RP laboratory inspection

The main manifestations are positive cell angiochromic anemia, significantly increased white blood cell count, increased platelets, increased eosinophils, increased erythrocyte sedimentation, hypoalbuminemia, hypergammaglobulinemia, and hypocomplementemia.

2. Serological examination

Rheumatoid factor and antinuclear antibodies were positive. False positive for syphilis serology. Blood circulation immune complexes are also often positive. Indirect fluorescence immunoassay showed that anti-cartilage antibodies and anti-natural collagen type antibodies were generally positive during the active phase, and could turn negative after treatment with hormones. Therefore, a positive anti-natural collagen type II antibody may be helpful in the diagnosis of RP. Urinary acidic mucopolysaccharide is positive and can be 4.21 times greater than the normal value during the onset of the disease, which can indicate the degree of cartilage destruction.

3. Auxiliary inspection

(1) X-ray examination of the chest radiograph showed atelectasis and pneumonia. Tracheobronchial tomography showed that the trachea and bronchi were generally narrow, especially the thoracic phase of the two arms extended posteriorly could show the limited collapse of the trachea. It can also show progressive enlargement of the aortic arch with calcifications in the ascending and descending aorta, ears, nose, trachea, and throat. The X-rays of the surrounding joints showed a decrease in bone mineral density near the joints, with occasional narrowing of the joint cavity, but without invasive destruction. The spine is generally normal, with a few reports of severe kyphosis, stenosis of the joint cavity, and invasion and fusion changes of the lumbar spine and intervertebral disc. Partial occlusion of the pubic bone and sacroiliac joints and irregular invasion.

(2) CT examination can find the stenosis and scope of the trachea and bronchial tree, thickening and calcification of the trachea and bronchial wall, stenosis of the lumen, and enlarged mediastinal lymph nodes. End-expiratory CT scan can observe the degree of airway collapse. High-resolution CT can show inflammation of subsegmental bronchi and lung lobules.

(3) Cardiac ultrasound may reveal ascending or descending aortic aneurysms, pericarditis, impaired myocardial contraction, mitral or tricuspid regurgitation, and atrial thrombosis. The ECG may show first-degree or complete atrioventricular block.

(4) Fiber bronchoscopy can directly observe the affected airways, and can show inflammation, deformation, collapse, etc. of the tracheobronchial tree, and further clarify the diagnosis and observe the progress of the disease.

(5) Pulmonary function tests show that both the expiratory and inspiratory flow are obstructed by measuring the inspiratory and expiratory flow curves. By analyzing the flow-volume curve, the maximum expiratory flow rate and the maximum inspiratory flow rate at 50% vital capacity can be obtained, so that the ratio of fixed stenosis and variable stenosis in dyspnea can be distinguished, and the location of the stenosis can be judged.

(6) A biopsy can provide further diagnostic evidence, but if the clinical symptoms are typical, a biopsy is not necessary. The biopsy site can be nasal cartilage, airway cartilage, auricular cartilage, etc., but after the biopsy may trigger the onset of recurrent polychondritis, causing new deformities. Therefore, special attention should be paid to taking ear cartilage from behind the ear.

Diagnosis of relapsing polychondritis

The diagnosis of relapsing polychondritis is generally based on clinical features and does not necessarily require a biopsy. McAdam et al. Proposed the following diagnostic criteria: symmetrical otochondritis, non-destructive, serum-negative polyarthritis, nasal chondritis, ophthalmitis, respiratory chondritis, cochlea or vestibular dysfunction. A diagnosis can be established by meeting at least three of them. If clinical manifestations are uncertain, chondritis due to other causes must be excluded, especially infectious diseases. Biopsy and culture or other necessary tests must be performed to exclude syphilis, leprosy, fungi or other bacterial infections.

Tracheal and bronchoconstriction can be determined using radiographic and CT techniques. A CT scan is the preferred test for safety, speed, and accuracy. Typical manifestations of narrowing of the airway lumen due to thickening and collapse of supportive cartilage structures can be seen.

Recurrent polychondritis treatment

Mild polychondritis, chondritis that is limited to the joints, nose, or ears, can be treated with non-steroidal anti-inflammatory drugs. More severe polychondritis, such as scleritis, uveitis, and those with systemic symptoms, must start glucocorticoid therapy, prednisone (or an equivalent amount of other preparations), and immunosuppressants such as azathioprine or cyclam Phosphamide. As soon as the effect appears, the dose of glucocorticoid should be gradually reduced until discontinuation. Cyclosporine has been used in refractory cases with good results. Patients with acute airway obstruction who have failed oral glucocorticoids have been successfully treated with methylprednisolone intravenous shock.

Traditional treatments include aspirin or other nonsteroidal anti-inflammatory drugs, dapsone, and hormones.

1. Patients with less severe illness

You can choose aspirin or other non-steroidal anti-inflammatory drugs and dapsone.

2. Moderate to severe patients

Choose glucocorticoids and immunosuppressants.

3. Other treatments

(1) Surgery For patients with severe epiglottis or inferior epiglottis obstruction that causes severe dyspnea, tracheostomy should be performed immediately, and even appropriate ventilation should be supplemented to obtain opportunities for further medical treatment. Generally, tracheal intubation is not used, because it can cause sudden airway occlusion and death. If unavoidable, choose a thinner intubation. For localized tracheal stenosis caused by chondritis, surgical resection can be performed, but the prognosis is not significantly improved. For heart valve disease or intractable heart failure due to valve insufficiency, valve repair or valve replacement can be used. Aortic aneurysms can also be removed surgically.

(2) Metal stent can narrow the trachea or bronchus in many places or under a wide range of bronchoscopy or X-ray guidance, which can significantly relieve breathing difficulties.

Recurrent polychondritis prognosis

Early diagnosis and timely treatment of RP patients may prolong the survival time of patients, and the 5-year survival rate and 10-year survival rate of relapsing polychondritis. Common causes of death are infections and cardiovascular diseases such as systemic vasculitis or ruptured hemangiomas. The prognosis of patients with RP is difficult to judge. Indicators of poor prognosis include: patients with older age at diagnosis, anemia, laryngotracheal involvement, saddle nose deformities, respiratory symptoms, microscopic hematuria, etc. Patients with vasculitis and poor response to oral hormones have a worse prognosis.