OVERVIEW
Recurrent polychondritis (RP) is a rare disease involving multiple systems throughout the body with recurrent and remitting progressive inflammatory destructive lesions involving cartilage and other connective tissues throughout the body, including the ear, nose, eye, joints, respiratory tract, and cardiovascular system. Clinical manifestations include chondritis of the ear, nose, and respiratory tract with symptoms of organ involvement of the eye and ear vestibule. Polyarthritis and vascular involvement are also relatively common.
Etiology
The etiology is still unknown and may be related to trauma, infection, allergy, alcoholism, and the use of hydralazine hydrochloride, etc. It is also thought to be related to mesangial synthesis disorders or protein hydrolase abnormalities. However, through years of research into the clinical features, laboratory tests, and pathology, more and more information suggests that it is an immune-mediated disease, including humoral immunity and cellular immunity.
Symptoms
1. Chondritis of the ear
Ossicular chondritis is the most common symptom, and is the first symptom in 39% of cases. It is characterized by pain, swelling, redness, and burning of the external auditory meatus, and the inflammation may subside on its own or with treatment. After repeated episodes, the external auditory meatus becomes soft and collapses. Sudden onset of deafness and vertigo may occur due to vasculitis of the vestibular structures or inner ear arteries. 85% of cases are affected during the course of the disease. The onset of the disease is sudden, usually symmetrical, and rarely unilateral. Acute exacerbations are characterized by redness, swelling, heat, pain, and erythematous nodules in the external auditory meatus. The lesions may be limited or diffuse.
2. Auditory and/or vestibular involvement
The lesion invades the external auditory canal or the eustachian tube, resulting in stenosis or occlusion, which may cause hearing impairment; the lesion involves the middle ear and the inner ear, which may be manifested as auditory and/or vestibular impairment; when the combined vasculitis involves the branches of the internal auditory artery, auditory abnormality and vestibular impairment may also occur. It is often accompanied by rotational dizziness, ataxia, nausea and vomiting.
3. Nasal chondritis
Sudden onset, manifested by pain and redness and swelling, relieved after a few days. If repeated episodes can cause limited collapse of nasal cartilage, forming saddle nose deformity. Some patients even have a sudden subsidence of the bridge of the nose within 1 to 2 days of the onset of the disease. The patient often accompanied by nasal congestion, nasal discharge and nasal nodules.
4. Ocular inflammatory lesions
The main manifestation is inflammation of the attachment of the eye, which can be unilateral or symmetrical. The most common are conjunctivitis, keratitis, iridocyclitis, scleritis and uveitis. Retinopathy is also common, such as retinal microaneurysms, hemorrhage and exudation, retinal vein occlusion, arterial embolism, retinal detachment, optic neuritis and ischemic optic neuritis.
5. Joint lesions
Polyarthritis is the 2nd common initial symptom of the disease, typically presenting as a wandering, asymmetric, non-deforming arthritis, which may involve the peripheral or mid-axial joints of all sizes. Respiratory chondritis may lead to atrophy and collapse of the nasal cartilage, manifesting as a saddle nose deformity. Involvement of the larynx, trachea, and bronchi may result in hoarseness, air obstruction, suprachondral tenderness of the thyroid, cough, wheezing, or stridor. The most commonly involved joints are the metacarpophalangeal joints, proximal interphalangeal joints, and knee joints, followed by the ankle, wrist, and elbow. Joints adjacent to the sternum, such as the costal cartilages, sternal styloid and sternoclavicular joints, may also be involved. Sacroiliac joints and pubic symphysis can also be involved in RP. Localized pain and tenderness are present, which may be accompanied by swelling. Patients with RP may also be accompanied by destructive arthropathic diseases, such as adult psoriatic arthritis, juvenile rheumatoid arthritis, Wright’s syndrome, desiccation syndrome, and ankylosing spondylitis.
6. Larynx, trachea and bronchial tree cartilage lesions
Most patients complain of chronic cough, sputum, followed by shortness of breath, often diagnosed as chronic bronchitis, lasting 6 months to decades, and ultimately dyspnea, recurrent respiratory infections and wheezing, and sometimes pre-tracheal and thyroid cartilage tenderness, hoarseness or loss of voice.
7. Cardiovascular lesions
Recurrent polychondritis can also involve the cardiovascular system, with an incidence of 30%, including aortic aneurysm, aortic valve embolism of large vessels, inflammation of small or large vessels and heart valve damage, pericarditis and myocardial ischemia. And can cause death. Cardiovascular complications: cardiovascular collapse due to complete conduction block and acute aortic atresia; aortic valve rupture. Macrovascular involvement may lead to vascular aneurysms (aorta, subclavian artery) or thrombosis due to vasculitis or coagulopathy. In some cases, aneurysms of the ascending aorta and aneurysms of the thoracic, abdominal, aorta and subclavian arteries may occur.
8. Skin
10% are the first symptoms. Recurrent polychondritis can have a variety of skin and mucosal lesions, skin lesions are non-specific, such as erythema nodosum, seborrheic membrane inflammation, reticulocyanosis, urticaria, cutaneous polyarteritis nodosa and aphthous ulcers and so on.
9. Nervous system
Acute or subacute lesions of pairs II, III, IV, VI, VII and VIII of the cerebral nerves may result in ophthalmoplegia, optic neuritis, facial paralysis, hearing loss and vertigo. Other neurologic complications include hemiplegia, chronic headache, ataxia, seizures, confusion, dementia, and meningoencephalitis.
10. Kidney
The most common pathologic tissue types are mild thylakoid hyperplasia and focal segmental crescentic glomerulonephritis. Others include glomerulosclerosis, IgA nephropathy, and interstitial tubulointerstitial nephritis.
11. Others
Anemia and weight loss are the most common systemic symptoms, often accompanied by fever during acute attacks. Muscle pain and liver function impairment may also occur. Generally speaking, laboratory abnormalities are nonspecific, such as anemia of chronic disease in most patients.
Examination
1. Laboratory examination of RP
The main manifestations are normocytic normochromic anemia, markedly increased white blood cell count, elevated platelets, increased eosinophil count, increased sedimentation rate, hypoalbuminemia, hypogammaglobulinemia and hypocomplementemia.
2. Serologic examination
Rheumatoid factor and antinuclear antibody are positive. False-positive serologic reaction for syphilis. Circulating immune complexes are often positive. Indirect fluorescence immunoassay shows that anti-cartilage antibody and anti-natural collagen type II antibody are generally positive during the active period, and can turn negative after hormone treatment. Therefore, positive anti-natural collagen type II antibodies may be helpful in the diagnosis of RP. Uric acid mucopolysaccharide is positive, which can be 4.21 times greater than the normal value during the exacerbation of the disease, and it can indicate the degree of cartilage destruction.
3. Auxiliary examination
(1) X-ray examination Chest radiograph shows pulmonary atelectasis and pneumonia. Tracheobronchial tomography can show generalized stenosis of trachea and bronchus, especially the lateral view with both arms stretched back and chest lifted can show limited collapse of trachea. It also shows progressive enlargement of the aortic arch and calcification of the ascending and descending aorta, auricles, nose, trachea, and larynx. X-rays of the peripheral joints show decreased bone density in the periarticular area and occasional progressive narrowing of the joint cavity without invasive destruction. The spine is generally normal, with a few reports of severe kyphosis, joint space narrowing, and invasive and fused changes in the lumbar spine and intervertebral discs. There is partial occlusion and irregular invagination of the pubic and sacroiliac joints.
(2) CT examination The degree and extent of stenosis of the trachea and bronchial tree can be detected, and thickening and calcification of the trachea and bronchial wall, narrowing and deformation of the lumen, and enlarged mediastinal lymph nodes can be detected. End-expiratory CT scan can observe the degree of airway collapse. High-resolution CT can show inflammation of subsegmental bronchial tubes and lung lobules.
(3) Cardiac ultrasound may reveal ascending or descending aortic aneurysm, pericarditis, impaired myocardial contraction, mitral or tricuspid regurgitation, and atrial thrombus. Electrocardiogram may show first degree or complete atrioventricular block.
(4) Fiberoptic bronchoscopy Direct observation of the involved airway can show inflammation, deformation, collapse of the tracheobronchial tree, etc., to further clarify the diagnosis and observe the course of the disease.
(5) Lung function test: Measurement of inspiratory and expiratory flow curves shows that there is obstruction in both expiratory and inspiratory airways. By analyzing the flow-volume curves, the maximum expiratory flow rate and the maximum inspiratory flow rate at 50% of the lung volume can be obtained, so that the proportion of fixed stenosis and variable stenosis in the dyspnea can be differentiated and the location of the stenosis can be determined.
(6) Biopsy may provide further diagnostic evidence, but is not necessary if clinical symptoms are typical. Biopsies can be taken from nasal cartilage, airway cartilage, or cartilage of the auricular cartilage, etc. However, biopsies may stimulate recurrent episodes of polychondritis, resulting in new deformities. Therefore, special attention should be paid to the fact that the cartilage of the auricle should be removed from behind the ear.
Diagnosis
The diagnosis of recurrent polychondritis is usually based on clinical features, not necessarily biopsy, and the following diagnostic criteria have been proposed by McAdam et al: symmetric otitis media, non-destructive, seronegative polyarthritis, rhinocartilaginous chondritis, ophthalmoplegia, respiratory chondritis, and cochlear or vestibular dysfunction. The diagnosis can be established by meeting at least 3 of these. If the clinical picture is uncertain, other causes of chondritis must be excluded, especially infectious diseases. Biopsies and cultures or other tests necessary to exclude syphilis, leprosy, fungal or other bacterial infections should be performed.
Stenosis of the trachea and bronchi can be determined by radiologic imaging and CT scanning, which is the preferred test for safety, speed, and accuracy. Typically, a narrowing of the airway lumen due to thickening and collapse of the supporting cartilaginous structures is seen.
Treatment
Mild cases of polychondritis, limited to the joints, nose, or ears, can be treated with nonsteroidal anti-inflammatory drugs. In more severe cases of polychondritis, such as scleritis, uveitis, and those presenting with systemic symptoms, glucocorticoid therapy, prednisone (or equivalent amounts of other agents), and immunosuppressive agents such as azathioprine or cyclophosphamide must be initiated. As soon as efficacy occurs, the glucocorticoid dose should be tapered until it is discontinued. Cyclosporine has been used in refractory cases with good results. Intravenous shock therapy with methylprednisolone has been reported to be successful in acute airway obstruction where oral glucocorticoids have failed.
Traditional treatment includes aspirin or other nonsteroidal anti-inflammatory drugs, ampicillin, and hormones.
1. Patients with mild disease
Aspirin or other non-steroidal anti-inflammatory drugs and aminosulfone may be used.
2. Patients with moderate to severe disease
Glucocorticoids and immunosuppressants are to be chosen.
3. Other treatments
(1) Surgery For patients with severe epiglottic or subepiglottic obstruction leading to severe dyspnea, tracheotomy and fistula should be performed immediately, even with appropriate ventilation, in order to obtain the opportunity for further drug treatment. Tracheal intubation is generally not an option, as it can cause sudden death by occlusion of the airway, and if unavoidable, a thinner intubation tube should be chosen. Surgical resection is feasible for limited tracheal stenosis due to chondritis, but it does not significantly improve the prognosis. In cardiac valvular lesions or refractory heart failure due to valvular insufficiency, valve repair or valve replacement is indicated. Aortic aneurysm can also be surgically removed.
(2) Metal stents For multiple or extensive tracheal or bronchial stenosis, metal stents can be placed under fiberoptic or X-ray guidance, which can significantly relieve dyspnea.
Prognosis
Patients with RP who are diagnosed early and treated promptly have the potential to prolong their survival, with 5-year survival rates and 10-year survival rates for recurrent polychondritis. Common causes of death are infections and cardiovascular disease such as systemic vasculitis or ruptured hemangiomas.The prognosis of patients with RP is more difficult to determine. Indicators of a poor prognosis include advanced age of the patient at diagnosis, anemia, laryngotracheal involvement, saddle-nose deformity, respiratory symptoms, and microscopic hematuria, and the prognosis is even worse in patients with concomitant vasculitis and those who do not respond well to oral hormones.
Questions you may be concerned about
What is the mortality rate of recurrent polychondritis?
The mortality rate for recurrent polychondritis is about one-third. Prompt treatment may prolong survival.
Recurrent polychondritis is a relatively rare systemic multisystem autoimmune disease. It is often treated clinically with high-dose prednisone and cyclophosphamide. If not treated properly, rapid progression of the disease can lead to kidney damage and respiratory failure and death. Systemic vasculitis has a higher mortality rate than those with respiratory obstruction.
There is no specific cure for this disease, the key is early diagnosis, timely treatment, it is possible to prolong the survival of patients, patients with recurrent chondritis should actively take treatment measures, pay attention to rest, do not blindly take drugs on their own.
Is recurrent polychondritis limited to both ears affecting life expectancy?
Recurrent polychondritis is limited to both ears and is an ear chondritis, which usually does not affect life expectancy.
Osteochondritis dissecans is a common clinical symptom of recurrent polychondritis dissecans, and the lesions are mostly confined to the cartilaginous parts of the auricle, including the ear wheel and the ear screen, and may occasionally encroach on the external auditory canal.
In the early stage, the main manifestations are redness, swelling, heat and pain of the skin of the auricle, as well as erythema nodules and other uncomfortable symptoms, and in the later stage, it can involve the inner ear, resulting in hearing and vestibular function damage. Symptoms can usually be relieved with regular treatment and will not affect the patient’s life expectancy.
Recurrent polychondritis is an autoimmune disease. Once diagnosed, it can be treated with glucocorticoid drugs such as prednisone and dexamethasone under the guidance of a doctor, which can effectively control inflammation and alleviate the symptoms; for patients who have poor response to glucocorticoid drugs, it can be combined with immunosuppressant drugs such as cyclophosphamide and methotrexate to carry out the treatment.
Once recurrent polychondritis occurs, it is recommended to receive regular treatment as soon as possible, which can effectively relieve symptoms and improve the quality of life.