Relapsing polychondritis

Introduction to recurrent polychondritis Recurrent polychondritis is a rare disease involving multiple systemic diseases. It has progressive inflammatory destructive lesions with recurrent and remission, involving cartilage and other systemic connective tissues, including the ear, nose, eyes, joints, respiratory tract and heart. Vascular system, etc. Clinical manifestations include ear, nose, and respiratory chondritis, accompanied by symptoms of organs such as the eyes and vestibule of the ear, and polyarthritis and vascular involvement are also common. In 1923, Jaksch-Wartenhorst first described the clinical manifestations of this disease and named it polychondropathia. In 1960, Pearson analyzed 12 patients, named relapsing polychondritis (RP), its etiology and incidence. The mechanism is still unclear, but there is growing evidence that the disease has autoimmune mechanisms involved. basic knowledge The proportion of illness: 0.0003% Susceptible people: no special people Mode of infection: non-infectious Complications: scleritis leukemia aneurysm anemia

Cause of recurrent polychondritis

(1) Causes of the disease

The cause is still unknown, and may be related to trauma, infection, allergy, alcoholism, taking hydralazine hydrochloride, etc. Some people think that it is related to mesoderm synthesis disorder or proteolytic enzyme abnormality, but through clinical features, laboratory tests and pathology for many years. Research, more and more data suggest that it is an immune-mediated disease, including humoral and cellular immunity.

(two) pathogenesis

1. The pathogenesis of this disease is still not clear. Some people think that it is related to mesoderm synthesis disorder or abnormal proteolytic enzymes. Studies have shown that immune mediation may be the key to the disease, and 25% to 30% of cases have other autoimmune diseases. Such as rheumatoid arthritis, nodular polyarteritis, Sjogren's syndrome, systemic lupus erythematosus, Behcet's disease, Wright syndrome, Wegener's granulomatosis, ankylosing spondylitis, vasculitis, etc., pathology It shows that the diseased tissue has mononuclear cell infiltration, especially lymphocytes and plasma cells of CD4. Serological tests can find antibodies of type II collagen. In a few cases, antibodies of IX and XI collagen are also found, and some cases have anti-nuclear antibodies and rheumatoid factor. Or positive for circulating immune complexes, inflammatory changes in auricular cartilage and polyarticular cartilage can be observed with type II collagen-immunized rodents, and cell-mediated specific immune enhancement of cartilage antigens is observed by direct Immunofluorescence was observed, and immunoglobulin and complement deposition were observed in the affected cartilage. RP was associated with HLA-DR4 and was not associated with type I HLA. Called Matrilin-1 antigen may be involved in the pathogenesis of RP, which is a cartilage matrix protein, adult tracheal, nasal and ear cartilage specific glucocorticoid or immunosuppressive therapy effective.

In summary, RP is the body's autoimmune response to type II collagen, causing cartilage damage, in addition to cartilage glycoprotein, elastin and other collagen can also induce autoimmune reactions, cartilage glycoprotein antigen is widely present in the sclera, Iris ciliary body, trachea, optic nerve, endothelial cells, aortic vascular connective tissue, heart valve, myocardial fibrous membrane, glomerular basement membrane, synovial membrane, etc., to prove that cartilage glycoprotein antibody can induce cartilage degeneration, synovitis And perichondritis, cartilage glycoprotein can also inhibit the synthesis of chondrocyte glycoprotein, its significance in RP needs to be further clarified.

2. RP has no specific pathological changes, its histopathological features are cartilage dissolution with perichondritis, and various acute and chronic inflammatory cell infiltration can be seen at the junction of the initial cartilage and perichondrion, including mononuclear cells, multinucleated cells, and fibers. Mother cells, vascular endothelial cells, etc., then the acid mucopolysaccharide in the cartilage matrix is reduced or disappeared, the cartilage matrix becomes loose, the chondrocytes are destroyed, the disease is further developed, the cartilage matrix is necrotic, dissolved, liquefied, and granulation tissue appears, and finally the remaining cartilage tissue Disappeared, granulation tissue fibrosis, scar formation contraction, tissue collapse deformation.

Recurrent polychondritis prevention

Every effort is made to avoid picking up the body's immune response and is a 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.

Recurrent polychondritis complications Complications Scleritis leukemia aneurysm anemia

Vasculitis is the most common disease that coexists with RP, including isolated leukocytic vasculitis and systemic vasculitis involving multiple organs (Table 3). RP can affect blood vessels of various sizes, most commonly for aortic involvement. , manifested as aortic dilatation or aneurysm formation; there are also microscopic arteritis under the microscope, in order to explain RP skin changes, kidney performance, scleritis, hearing and vestibular dysfunction, etc., RP can be associated with clear vasculitis Coexistence, including Wegener's granulomatosis, Behcet's disease, nodular polyarteritis.

RP coexists with various forms of connective tissue disease, and most connective tissue diseases precede months to years before the onset of RP, the most common of which are systemic lupus erythematosus, which is currently speculated to be part of connective tissue disease, in genetics It is considered that RP is a branch of connective tissue disease, and RP cases in our hospital have combined rheumatoid arthritis and Sjogren's syndrome.

RP and hematological diseases are combined, mainly for myelodysplastic syndrome, most of which are male, half of them have chromosomal abnormalities, acute and chronic myeloid leukemia and aplastic anemia have been reported. It has been reported that individual RP combined with Hodgkin's disease .

The association between RP and thyroid disease is 4%, such as combined with exophthalmia, non-toxic goiter, chronic lymphocytic thyroiditis (Hashimoto disease), and hypothyroidism.

There are also reports of RP with ulcerative colitis, primary biliary cirrhosis and sclerosing cholangitis.

Recurrent polychondritis symptoms Common symptoms Respiratory failure erythema nodules ataxia dyspnea

The disease can occur in all ages, and the peak incidence of 40 to 50 years old, both men and women can be affected, but women with more respiratory tract involvement and heavier, the clinical features of recurrent polychondritis are shown in Table 1.

The disease occurs in Caucasians, but the disease is reported in all parts of the world. The incidence rate is about 3.5/1 million. It can occur from any newborn to 90 years old. Most of the ages are 40-60 years old. There is no gender and familial morbidity, and the clinical process is diverse. Most cases are diagnosed with multiple systems, sudden onset, sudden onset of illness, or fulminant episodes with respiratory failure and cartilage distribution. Various tissues and organs throughout the body, usually characterized by multiple sites of chondritis, clinical manifestations vary depending on the site involved, but also due to combined connective tissue disease or vasculitis.

1. Auricular cartilage inflammation: Auricular cartilage inflammation is the most common symptom, in 39% of cases is the first symptom, sudden pain in the outer ear, swelling, redness, hot, characterized by inflammation can be resolved or treated by itself With retreat, the auricle becomes soft and collapses after repeated episodes. Due to the vestibular structure of the ear or the vasculitis of the inner ear, sudden hearing loss and dizziness can occur, 85% of the disease is involved, and the onset is sudden, usually symmetry, unilateral Rare, acute exacerbation is characterized by red, swollen, hot, painful, erythematous nodules in the outer ear. The lesions can be limited or diffuse. The severity of the lesions varies from a few days to a few weeks, and then can be relieved by itself. Repeated episodes of inflammation can lead to destruction of cartilage, relaxation of the outer auricle, collapse, deformity and local pigmentation, known as cauliflower ears (Fig. 1, 2). The lesion is confined to the cartilage portion and does not invade the earlobe.

2. Hearing and/or vestibular function involvement: lesions invade the external auditory canal or eustachian tube, resulting in stenosis or occlusion, causing hearing damage; lesions involving the middle and inner ear, which may be manifested as auditory and/or vestibular dysfunction; Auditory abnormalities and vestibular dysfunction can also occur when combined vasculitis involves the internal auditory arterial branch. These symptoms can be acute or occult, with a hearing test of 35 dB of neurological or mixed hearing impairment, often accompanied by a rotation. Sexual dizziness, ataxia, nausea and vomiting.

3. Nasal cartilage: the incidence rate is 63% to 82%, often sudden onset, manifested as pain and redness, remission after a few days, such as repeated attacks can cause localized collapse of nasal cartilage, forming saddle nose deformity (Figure 3, 4 ), even some patients may suddenly sag in the nose within 1 to 2 days of onset, patients often accompanied by nasal congestion, nasal secretions and nasal induration.

4. Ocular inflammatory lesions : the incidence rate is 55%, mainly manifested as inflammation of the attachment of the eye, which may be unilateral or symmetrical, most commonly conjunctivitis, keratitis, iridocyclitis, scleritis and In uveitis, the severity of these symptoms is often parallel to other areas of inflammation, and retinopathy often occurs, such as omental microaneurysms, hemorrhage and exudation, omental venous occlusion, arterial embolism, retinal detachment, optic neuritis and Ischemic optic neuritis and the like.

5. Joint lesions: Polyarthritis is the second most common initial disease of this disease. It is typically characterized by migratory, asymmetrical, non-arthritic arthritis, which can affect the size of the surrounding or central axis, the respiratory tract. Chondritis can cause nasal cartilage atrophy and collapse, manifested as saddle nose deformity, laryngeal, tracheal and bronchial involvement can cause hoarseness, gas stagnation, tenderness on the thyroid cartilage, cough, wheezing or wheezing, atrophy and collapse of the main airway, often caused Respiratory obstruction, and has a high mortality rate, requiring urgent diagnosis and treatment, patients with severe airway involvement, often secondary to upper and lower respiratory tract infections, the incidence rate is 70%, can be a single asymmetry of large joint lesions, It can also be a persistent multiple sympathetic facet joint lesion. The most commonly involved joints are the metacarpophalangeal joint, the proximal interphalangeal joint and the knee joint, followed by the ankle joint, the wrist joint, the elbow joint, and the joint of the sternum. Such as costal cartilage, sternum stem and sternocleidal joint, etc., ankle joint and pubic symphysis can also be involved in RP, arthritis is often sudden, non-destructive and non-deformed, local pain and tenderness, With swelling, the onset of the disease will be relieved after several days to several weeks, or the anti-inflammatory treatment will improve. The involvement of the joint is not related to the activity of the disease. RP patients may also be accompanied by destructive joint disease, such as adult psoriatic arthritis, juvenile Rheumatoid arthritis, Wright syndrome, Sjogren's syndrome, ankylosing spondylitis, etc.

6. Laryngeal: tracheal and bronchial tree cartilage lesions incidence rate of 50% to 71%, 26% of the first symptoms, which are more common in women, and most patients complain of chronic cough, sputum, followed by shortness of breath, often diagnosed as chronic bronchus Inflammation, which lasted from 6 months to several decades, eventually caused dyspnea, repeated respiratory infections and wheezing, sometimes pre-tracheal and thyroid cartilage tenderness, hoarseness or aphasia, airway obstruction in the early stage was inflammatory edema; late Airway cartilage ring destruction, easy to collapse, resulting in airway elastic stenosis; advanced fibrosis and scar contraction, resulting in fixed stenosis of the airway; due to airway ciliated epithelial damage, clearance of secretions decreased, can also cause Blockage and infection; in addition, vocal cord paralysis can also cause inspiratory dyspnea.

7. Cardiovascular disease : recurrent polychondritis can also affect the cardiovascular system, the incidence rate is 30%, including aortic aneurysm, aortic valve vascular embolization, small blood vessel or macrovascular inflammation and heart valve damage, pericarditis and Myocardial ischemia can cause death. In addition, there are two fatal disasters in cardiovascular complications: one is complete block and acute aortic insufficiency caused by cardiovascular collapse; the other is the main Aortic valve rupture, large vessel involvement can lead to vascular aneurysms (aorta, subclavian artery), or thrombosis due to vasculitis or coagulopathy, small blood vessels involved in white blood cell fragmentation vasculitis, generally male The patient's aortic involvement is common, and the aortic ring and descending aorta are progressively dilated. In some cases, an aortic aneurysm, aneurysm of the thoracic abdomen, aorta, and subclavian artery may occur.

8. Skin: 25% to 35% involving the skin, 10% of which are the first symptoms. Recurrent polychondritis can have multiple skin mucosal lesions, and the lesions are non-specific, such as nodular erythema, panniculitis, net Plaque, urticaria, cutaneous polyarteritis nodules and aphthous ulcers, biopsy pathology often shows leukocyte rupture vasculitis, the incidence of skin lesions are not related to age, gender, etc., with bone marrow dysplasia lesions The incidence rate is 90%.

9. Nervous system: Acute or subacute lesions of the cranial nerves of II, III, IV, VI, VII and VIII can cause ophthalmoplegia, optic neuritis, facial paralysis, hearing loss and dizziness, other neurological complications and hemiplegia , chronic headache, ataxia, seizures, insanity, dementia and meningoencephalitis, etc., a few patients have involved, such as manifestations II, VI, VII, VIII cranial nerve palsy, cerebellar ataxia, epilepsy, organic Sexual encephalopathy and dementia, a few reports of intracranial aneurysms.

10. Kidney: Involved and rare, about 8%, the most common pathological tissue types are mild mesangial proliferative and focal segmental glomerulonephritis, other glomerular sclerosis, IgA nephropathy Interstitial renal tubular nephritis, etc., there was 1 case of intermittent gross hematuria in our hospital for 8 months, accompanied by a decrease in proteinuria and creatinine clearance. Most authors believe that patients with renal disease often have other systemic vasculitis. disease.

11. Others : Anemia and weight loss are the most common systemic symptoms, often accompanied by fever during acute attacks, muscle pain and liver damage.

Generally speaking, laboratory abnormalities are non-specific. For example, most patients have chronic anemia, mild white blood cells or thrombocytopenia, often with increased erythrocyte sedimentation rate, hypergammaglobulinemia, and proteinuria and cellular urine in addition to kidney involvement. Conventional is generally normal, common serological abnormalities are RF (10% to 20%) positive, ANA (15% to 25%), anti-natural DNA antibody positive patients can coexist with SLE, serum complement levels are generally normal or slightly elevated High, a small number of patients can be found in cold globulin, there have been reports of anti-II, IX, XI collagen autoantibodies.

The diagnosis of recurrent polychondritis is generally based on clinical features, not necessarily biopsy, McAdam et al. proposed the following diagnostic criteria: symmetric ear cartilage, non-destructive, seronegative polyarthritis, nasal chondritis, ophthalmia, Respiratory chondritis, cochlear or vestibular dysfunction, in accordance with at least 3 of them can be established diagnosis, if the clinical manifestations are uncertain, must be excluded from other causes of chondritis, especially in addition to infectious diseases, must be biopsy and culture or other necessary Test to exclude syphilis, leprosy, fungus or other bacterial infections.

The narrowing of the trachea and bronchi can be determined by radiography and CT techniques. CT scan is the safe, fast, and accurate preferred procedure. Typical performance can be seen in the thickening and collapse of the supporting cartilage.

Other tests, such as pulmonary function tests (especially with cough and dyspnea), echocardiography, cardiac catheterization, angiography, etc., should be considered in the presence of cardiovascular symptoms and signs.

Examination of recurrent polychondritis

1. RP laboratory examination: no specific performance, mainly manifested as positive cell angiochromatosis , significant increase in white blood cells, elevated platelets, increased eosinophils, increased erythrocyte sedimentation rate, hypoalbuminemia, high gamma globulin Hyperemia and hypocomplementemia, among which erythrocyte sedimentation rate is the most common, and is related to the activity of the disease, the degree of anemia is mild to moderate, serum iron and serum iron saturation are reduced, but the reserves of bone marrow iron are generally normal.

2. Serological examination : rheumatoid factor and anti-nuclear antibody positive, syphilis serological reaction false positive, blood circulation immune complex is also often positive, indirect fluorescent immunoassay shows that anti-chuck antibody and anti-natural collagen type II antibody are generally active during the active period Positive, can be negative after treatment with hormones, therefore, anti-natural collagen type II antibody positive may be helpful in the diagnosis of RP, uric acid mucopolysaccharide positive, in the attack period can be greater than the normal value of 4.21 times, which can indicate cartilage destruction degree.

3. X-ray examination : chest radiograph shows atelectasis and pneumonia, tracheal bronchial tomography can be seen in the trachea, bronchial general stenosis, especially the extension of the thoracic side of the two arms can show the collapse of the trachea, but also can show the aortic arch Sexual enlargement, ascending and descending aorta, auricle, nose, trachea and larynx calcification, X-rays of the surrounding joints show decreased bone density near the joints, occasionally the joint cavity is gradually narrow, but there is no erosive damage, the spine is generally normal, A few reports have severe kyphosis, narrow joint space, erosion and fusion of the lumbar spine and intervertebral disc, partial occlusion and irregular erosion of the pubic and ankle joints.

4.CT: The extent and extent of trachea and bronchial tree can be found. Thickening calcification of trachea and bronchial wall, stenosis of the lumen and enlarged mediastinal lymph nodes, and end-expiratory CT scan can be used to observe the degree of collapse of the airway. High-resolution CT can show inflammation of the sub-bronchus and pulmonary lobule, and the three-dimensional reconstruction of the airway can suggest more information.

Cardiac ultrasonography can be found in ascending aortic aneurysm or descending aortic aneurysm, pericarditis, impaired myocardial contraction, mitral or tricuspid regurgitation, atrial thrombus, etc., ECG may show i degree or complete atrioventricular block.

Fiberoptic bronchoscopy can directly observe the affected airway, can show inflammation, deformation, collapse, etc. of the tracheobronchial tree, further confirm the diagnosis and observation of the disease process, mucosa visible erythema, edema, granuloma-like changes or atrophy, cartilage ring Destructors can see the corresponding airway collapse when exhaling, can take biopsy under the microscope, help to confirm the diagnosis, but more bleeding, and in the evaluation of airway obstruction is not as good as lung function, and may induce airway collapse and suffocation death.

Pulmonary function shows that the exhalation and exhalation flow curves show that both exhalation and inspiratory obstruction, and the flow rate-volume curve is analyzed to obtain the maximum expiratory flow rate and the maximum inspiratory flow rate at 50% of the vital capacity, so that the fixed stenosis can be distinguished. And the proportion of variable stenosis in dyspnea, and the location of the stenosis.

Biopsy can provide further diagnostic evidence, but if the clinical symptoms are typical, biopsy is not necessary. The biopsy can be nasal cartilage, airway cartilage, auricular cartilage, etc., but biopsy may stimulate recurrent multi-cartilage. The onset of inflammation causes new deformities, so special care should be taken to take the auricle cartilage from the back of the ear.

Diagnosis and diagnosis of recurrent polychondritis

Diagnostic criteria

1. 1975 McAdam diagnostic criteria for RP

3 or more of the following 6 articles, without the need for histological confirmation, can be diagnosed as RP. If the clinical diagnosis is very obvious, it can be confirmed without the histology of cartilage.

(1) Recurrent multi-chondritis of both ears.

(2) Non-erosive seronegative polyarthritis.

(3) nasal chondritis.

(4) eye inflammation, conjunctivitis, keratitis, scleritis, external scleritis and uveitis.

(5) Laryngeal and/or tracheal chondritis.

(6) Damage to the cochlea and/or vestibule.

2. Diagnosis: Given the rare clinical manifestations, complicated clinical manifestations and no special laboratory tests, the diagnosis is difficult. The diagnosis of RP should be considered through the above clinical and laboratory data.

Damiani and Levine believe that to achieve early diagnosis, McAdam's diagnostic criteria should be expanded, as long as one of the following is available:

1 meet 3 McAdam sign or more;

21 McAdam signs, plus pathology confirmed, such as ear, nose, respiratory cartilage biopsy;

3 lesions involving 2 or more anatomical sites, effective for hormone or dapsone treatment.

We believe that one of the following conditions should be suspected of this disease:

1 one or both sides of the external ear cartilage, with external ear malformation;

2 nasal chondritis or an unidentified saddle nose deformity;

3 repeated episodes of scleritis;

4 Unexplained tracheal and bronchial extensive stenosis, cartilage ring is unclear, or limited wall collapse, combined with laboratory tests, such as increased levels of urinary acid mucopolysaccharide and anti-collagen type II antibodies, will help diagnosis.

Differential diagnosis

In the early stages of the disease, it should be differentiated from many clinically similar diseases:

1. Auricular lesions are often the first symptoms of RP. They should be differentiated from other isolated auricle inflammations, including acute and chronic infections of the auricle, followed by trauma, frostbite, chemical stimulation, insect bites, sun exposure, etc. It should also be differentiated from cartilage dermatitis. The disease has small nodules around the ear, and the lesions also involve the edge of the cartilage. The onset is caused by dysfunction of the blood vessels. The lesions can be recurrent, similar to RP, and cystic cartilage of the auricle. Similar to RP, it has cavities in the central area of cartilage, but it is clinically painless and may be accompanied by swelling, often occurring in the upper part of the auricle, with localized serous exudation.

2. Hearing and vestibular dysfunction as the first symptom of RP should be differentiated from cerebral basilar artery disease and stroke, especially in sudden onset cases, with keratitis should be identified with Cogan's syndrome, the latter more common in young people, occasionally The onset of the elderly, often sudden on the unilateral or bilateral blurred vision, eye pain, tears, convulsions, tinnitus, dizziness, nausea, vomiting, bilateral progressive deafness, conjunctival hyperemia and hemorrhage, corneal plaque particles Sexual infiltration, etc., the lesion repeatedly or alternately invades both eyes, but it generally does not have chondritis.

3. The RP with nasal chondritis as the first symptom needs to be differentiated from nasal chronic infection, Wegener's granulomatosis, congenital syphilis, fatal midline granuloma, lymphoma, tuberculosis, etc., multiple biopsies and pathogens. Culture can help identify, and RP is mainly inflammation of cartilage, and does not invade soft tissue.

4. Ophthalmitis: due to the manifestations of this disease, the symptoms should be noted, such as necrotizing scleritis, keratitis, arthritis, otitis media with combined clinical manifestations of hearing and vestibular dysfunction, in Wegener's granulomatosis and Polyarteritis can also occur. When RP involves both eyes, joints and heart valves, and myocardium, it should be differentiated from rheumatoid arthritis, Behcet's disease, sarcoidosis and seronegative spondyloarthropathy.

5. Tracheal bronchial diffuse stenosis should be associated with infectious granulomatosis, induration, external pressure stenosis of the trachea, sarcoidosis, tumor, scabbard-like bronchial disease of chronic obstructive pulmonary disease, amyloidosis, congenital Identification of diseases such as trachea and bronchial softening, and the above-mentioned diseases can be clearly diagnosed by biopsy.

6. Aortic inflammation and aortic disease should be differentiated from syphilis, equine syndrome, Ehlers-Danlos syndrome, and atherosclerosis.

7. Costal cartilage lesions need to be differentiated from benign thoracic syndrome, such as idiopathic, traumatic costal cartilage, Tietze's syndrome, rib cage chondritis, xiphoid cartilage syndrome, etc., these diseases are not systematic Clinical manifestations, with the identification of RP.

8. RP joint lesions are diverse, with multiple peripheral small joints involved in the identification of rheumatoid arthritis; with a single large joint involvement with joint bacterial infection, reactive arthritis, etc.; Sexual joint pain is the main manifestation of sometimes being a pseudo-disease, and RP can be clinically combined with connective tissue disease to make the diagnosis more clear.

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