Relapsing polychondritis is a rare inflammatory disease affecting cartilage structures and connective tissue of various organs and systems. Presumably has an autoimmune nature. The most common symptom is damage to the auricles, then, in descending order – arthritis, chondritis of the nasal septum, various eye lesions, damage to the respiratory system (trachea, larynx and bronchi), inflammatory processes in the cardiovascular system. At first it flows in waves. Over time, periods of exacerbations and remissions are often replaced by a gradually progressive course. Treatment of relapsing polychondritis is usually conservative, using glucocorticoids. With severe damage to the trachea, larynx and heart valves, surgical intervention is required.
ICD 10
M94.1 Relapsing polychondritis
Meaning
Relapsing polychondritis is an inflammatory disease affecting cartilage and connective tissue. Ears, joints, nasal septum, trachea, larynx, heart valves, eyes, blood vessels and kidneys may suffer. In the initial period, there is usually a series of exacerbations and remissions. Subsequently, the course of rheumatic polychondritis often becomes progressive. Treatment is mostly conservative. In some cases, operations have to be performed to restore the functions of vital organs damaged as a result of inflammation.
The disease can develop at any age, but the peak incidence occurs in the period from 40 to 50 years. Women and men get sick equally often. Recurrent osteochondritis is one of the very rare diseases – to date, only about 800 cases have been registered worldwide. The causes, provoking factors and mechanisms of development are still unknown, however, characteristic changes in the level of antibodies in the blood suggest that the disease has an autoimmune character.
Relapsing polychondritis symptoms
Clinical manifestations of relapsing polychondritis are diverse in the place of lesion, duration and severity. In the first years of the disease, the course is usually recurrent, undulating, in the future it can be replaced by progressive. Relapses can occur both in the same area and in different ones. The onset of the disease may resemble a fever of unknown origin and be accompanied by fever, weakness and muscle pain. Subsequently, local inflammatory processes join the general symptoms.
The first place in the frequency of lesions is occupied by the auricles (from 85 to 95% of cases). The ear becomes painful, swollen, compacted and acquires a purple-purple hue. The lobe remains intact at the same time. The inflammation is usually two–sided: during the first attack, one ear is affected, during the subsequent ones, the other or both. The attack lasts from a few days to several weeks, then the symptoms disappear. As a result of repeated inflammatory processes, the amount of cartilage tissue decreases, and the ear becomes deformed, shapeless, saggy and flabby. If the inflammation spreads to the middle and inner ear, auditory and vestibular disorders are possible.
Arthropathies are in second place by prevalence (from 52 to 85% of cases). They can manifest as arthralgias, mono- or polyarthritis and capture large and small joints, as well as sternocostal joints. In the latter case, pain and restriction of respiratory excursions of the lungs are possible. There is no deformation as a result of inflammation. Symptoms go away on their own or against the background of taking NSAIDs. A little less often, chondritis of the nasal septum is observed (from 48 to 72% of cases), accompanied by rhinorrhea, congestion, an unpleasant feeling of bursting and nosebleeds. With prolonged inflammation or repeated attacks, the cartilage shrinks, as a result of which the back of the nose falls off and saddle-shaped deformity develops.
Eye damage is noted in 50% of cases. Ophthalmoplegia, periorbital edema, proptosis with chemosis (bulging of the eyeball, accompanied by conjunctival edema) is possible, less often – scleritis, episcleritis, conjunctivitis and dry keratoconjunctivitis, uveitis, retinopathy, ischemic neuropathy of the optic nerve, arterial and venous retinal thrombosis. In about a quarter of cases with relapsing polychondritis, respiratory tract damage occurs. In some cases, limited inflammation may be asymptomatic and have no effect on the patient’s condition. However, in general, this is the most severe manifestation of relapsing polychondritis, which occupies the first place among the causes of death of patients.
When the trachea and larynx are affected, shortness of breath, unproductive cough, difficulty breathing, pain, hoarseness of voice and dysphonia appear. With inflammation in the bronchial region, a clinical picture resembling bronchial asthma develops. In the early stages of inflammation, breathing difficulties are caused by swelling of the connective tissue. Subsequently, due to the destruction of cartilage, the affected areas of the respiratory tract may collapse with a deep, sharp inhalation and exhalation.
Auditory and vestibular disorders are observed in every fourth patient with relapsing polychondritis. Possible tinnitus, hearing loss, a feeling of stuffy ear and dizziness. Another group of symptoms that is detected in every fourth case of relapsing polychondritis and, like the defeat of the respiratory tract, often becomes the cause of death of patients, is the defeat of the cardiovascular system. Most often, with relapsing polychondritis, aortic insufficiency is observed, arrhythmias, pericarditis and conduction disorders develop less often.
Diagnostics
The rarity of the disease and the variety of clinical symptoms create considerable difficulties in diagnosis. Symptoms of recurrent osteochondritis may resemble manifestations of bronchopulmonary infections, traumatic injuries, allergies, tumors and various rheumatic diseases. On average, the diagnosis of “relapsing polychondritis” is made a year or more after the appearance of the first symptoms. Most patients are examined by several narrow specialists. The situation is complicated by the absence of pathognomonic instrumental and laboratory tests used to detect relapsing polychondritis.
Currently, when making a diagnosis of relapsing polychondritis, McAdam criteria based on the clinical symptoms of the disease are most often used. The diagnosis is considered reliable when three of the six signs are detected:
- Bilateral inflammatory process in the auricle area.
- The presence of non-erosive seronegative arthritis.
- Inflammation in the nasal septum.
- Eye damage.
- Inflammation of the cartilaginous structures of the trachea, larynx and bronchi.
- Vestibular disorders.
In blood tests, changes characteristic of the inflammatory process are determined: leukocytosis, increased ESR, anemia, an increase in the amount of alpha and gamma globulins. When the respiratory system is affected, tracheal stenosis is detected on radiographs. To clarify the degree of stenosis, to assess changes in the sublingual space and surrounding tissues, computed tomography and magnetic-nuclear tomography are performed. When the joints are affected, the X-ray images show a pattern characteristic of rheumatoid arthritis: erosion, narrowing of the articular gap, periarticular osteoporosis.
Differential diagnosis should be carried out with rheumatoid arthritis, Wegener’s granulomatosis, systemic lupus erythematosus and Takayasu arteritis. It should be borne in mind that in 25-35% of cases, relapsing polychondritis is observed in combination with other autoimmune diseases.
Relapsing polychondritis treatment
For mild lesions and inflammatory processes of moderate severity, low doses of glucocorticoids (15-20 mg) are used. In severe cases (damage to the aorta, heart, blood vessels, respiratory tract, kidneys, eyes and inner ear), the dose of glucocorticoids is increased to 40-60 mg. Pulse therapy is possible, as well as a combination with cytostatic immunosuppressants. With severe bronchial lesions, tracheomalacia, laryngeal and tracheal stenoses, surgical interventions are necessary: tracheoboronchial stenting, segmental resection of the bronchi or tracheostomy. The development of aortic insufficiency is an indication for prosthetics of a valve or a portion of the aorta.
The prognosis for relapsing polychondritis depends on the localization, severity of the lesion and frequency of relapses. The life expectancy of patients after the appearance of the first symptoms of relapsing polychondritis ranges from 10 months to 20 years.
Literature
- Relapsing Polychondritis: An Updated Review. Borgia F, Giuffrida R, Guarneri F, Cannavò SP. Biomedicines. 2018 Aug 2;6(3):84. link
- Red-eared zebra diagnosis: Case of relapsing polychondritis. Leung KK, Edani S. Can Fam Physician. 2018 May;64(5):363-367. link
- Three Cases of Relapsing Polycondritis with Isolated Laryngotracheal Stenosis. Taşlı H, Birkent H, Gerek M. Turk Arch Otorhinolaryngol. 2017 Jun;55(2):77-82. link
- Autoimmune-related nasal septum perforation: A case report and systematic review. Guntupalli L, Patel K, Faraji F, Brunworth JD. Allergy Rhinol (Providence). 2017 Mar 1;8(1):40-44 link
