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Relapsing Polychondritis

Relapsing polychondritis is a rare inflammatory disease characterized by recurrent inflammation of cartilage and other structures rich in proteoglycans, and involves damage to eye, ear, nose and cardiovascular system.


Presentation

Typically, relapsing polychondritis presents with sudden pain and common signs of inflammation at the affected site of inflammation at the onset of the disease [11]. Common symptoms include pain, swelling, erythema, and tenderness of the pinna of one or both ears, nose, throat, joints, and eyes; the ear lobe is typically spared. Fever and weight loss may also occur.

Recurrent inflammation of the cartilages in the ears and nose results in damage to the cartilage of the pinna and the nasal septum causing the deformities called saddle nose and floppy ears [11]. Inner ear involvement may cause vertigo, hearing loss, and nausea. Tracheal involvement may cause throat pain, hoarseness, and difficulty in breathing, which may become life threatening.

Inflammation of the joints present with joint pain, swelling, stiffness, and erythema [12]. Commonly affected joints include knee, ankle, wrist, elbow joints, and the small joints of the feet.

Ocular involvement may be mild or severe enough to cause vision loss. Cataracts are a frequent complication of this disease and may result from the steroid therapy or the inflammatory processes.

An urticarial annular cutaneous eruption is a characteristic early skin manifestation of relapsing polychondritis. Renal involvement is very rare in this disease. MAGIC syndrome is a syndrome in which relapsing polychondritis occurs with Behçet disease.

Other tissues which may be involved in relapsing polychondritis include the aorta, aortic valves, myocardium, pericardium, skin, and neurons. Therefore, possible complications of relapsing polychondritis include aortic aneurysm, aortic valve incompetence, pericarditis, myocarditis, vasculitis and cranial nerve palsies [13].

Saddle Nose
  • Since a saddle nose deformity, malacia of the auricles and sensorineural deafness were also observed, relapsing polychondritis was diagnosed. Measuring the peak expiratory flow rate was useful in the early airway assessment.[ncbi.nlm.nih.gov]
  • Months later she developed saddle nose deformity and hoarseness of voice. CT revealed tracheal/bronchial wall thickening and luminal narrowing. Based on these findings, RP was diagnosed.[ncbi.nlm.nih.gov]
  • Left ear swelling with erythematous change and saddle nose developed during the course of hospitalization, and an ear biopsy demonstrated severe cartilage necrosis.[ncbi.nlm.nih.gov]
  • She gradually developed neurosensory hearing loss, vertigo, and saddle nose while glucocorticosteroid therapy and combined cyclophosphamide could not control her headache. Ultimately, cyclosporin A was tried showing a good response.[ncbi.nlm.nih.gov]
  • Saddle nose deformity and a progressive disease course were observed frequently in the R subgroup.[ncbi.nlm.nih.gov]
Hoarseness
  • A 51-year-old woman presented with one-month history of fever, productive cough, dyspnea, hoarseness and polyarthritis. Computed tomography (CT) depicted diffuse bronchotracheal stenosis, which deteriorated in exhalation.[ncbi.nlm.nih.gov]
  • A 35-year-old male presented with complaints of hoarseness, tinnitus and dyspnea for 19 years, with a history of several diagnostic and therapeutic interventions for laryngeal and respiratory tract.[ncbi.nlm.nih.gov]
  • Months later she developed saddle nose deformity and hoarseness of voice. CT revealed tracheal/bronchial wall thickening and luminal narrowing. Based on these findings, RP was diagnosed.[ncbi.nlm.nih.gov]
  • She is in good health, but saddle-nose deformity, atrophy of external ears and hoarsness is remained.[jstage.jst.go.jp]
  • They include: pain inflammation or swelling in the cartilage or joints eye inflammation hoarseness shortness of breath or wheezing difficulty with hearing or balance Related Departments & Divisions Division of Rheumatology[pennmedicine.org]
Stridor
  • Imaging data was subsequently correlated with corresponding clinical symptoms like fever, dyspnea, stridor, uveitis, pain, hearing impairment as well as with acute-phase-inflammatory parameters like C-reactive protein (CRP) and erythrocyte sedimentation[ncbi.nlm.nih.gov]
  • Respiratory symptoms are seen in 20% of patients at presentation and eventually 60% will develop respiratory tract involvement which is manifested by a combination of symptoms including laryngeal tenderness, hoarseness, dyspnoea, and stridor/wheeze.[radiopaedia.org]
  • Laryngotracheal involvement, which may present as hoarseness, stridor, or local tenderness, may be life threatening if the airway is involved.[rheumaknowledgy.com]
Chest Wall Pain
  • wall pain or, less often, swelling of the involved cartilage. [3] The involvement of the ribs is seen in 35% of persons with RP but is rarely the first symptom. [3] Other manifestations [ edit ] Relapsing polychondritis may affect many different organ[en.wikipedia.org]
  • wall pain or, less often, swelling of the involved cartilage. [3] The involvement of the ribs is seen in 35% of persons with RP but is rarely the first symptom. [3] Other manifestations Relapsing polychondritis may affect many different organ systems[infogalactic.com]
Nasal Congestion
  • Common symptoms include: Fatigue or malaise Fever Red, swollen, painful (inflamed) ears, hearing loss, dizziness Ears that are "floppy," that is, they are softer than normal, limp or droopy Inflammation over the bridge of the nose, nasal congestion Arthritis[drugs.com]
Relapsing Polychondritis
  • Our aim was to estimate the incidence, prevalence and mortality of relapsing polychondritis and describe the clinical features of relapsing polychondritis in a large population.[ncbi.nlm.nih.gov]
  • Laryngotracheal reconstruction for relapsing polychondritis was performed using hyoid bone pedicled on sternohyoid muscle. Airway management in relapsing polychondritis can improve quality of life and palliate patients effectively.[ncbi.nlm.nih.gov]
  • We describe the case of a patient with relapsing polychondritis associated to Budd-Chiari syndrome due to antiphospholipid syndrome.[ncbi.nlm.nih.gov]
  • Relapsing polychondritis is a rare childhood disorder of unknown etiology, characterized by inflammatory, recurrent and destructive cartilage lesions.[ncbi.nlm.nih.gov]
  • Brain infarction associated with internal carotid artery thrombus, in a setting of relapsing polychondritis, has never been reported.[ncbi.nlm.nih.gov]
Fever
  • Fever of unknown origin (FUO) is a common initial presentation leading to a diagnostic challenge. A 3-month history of moderate-to-high fever was reported in an otherwise healthy 54-year-old man.[ncbi.nlm.nih.gov]
  • Beginning manifestations, such as fever, are often unspecific, leading to RP misdiagnosis.[ncbi.nlm.nih.gov]
  • Two years later, he developed abdominal pain and a fever. A contrast-enhanced computed tomography scan showed enhancement of the mesentery and massive ascites.[ncbi.nlm.nih.gov]
  • We report on a case of fever of unknown origin in which 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) was performed to make a diagnosis of RP.[ncbi.nlm.nih.gov]
  • A 51-year-old woman presented with one-month history of fever, productive cough, dyspnea, hoarseness and polyarthritis. Computed tomography (CT) depicted diffuse bronchotracheal stenosis, which deteriorated in exhalation.[ncbi.nlm.nih.gov]
Anemia
  • Reports of RP patients with autoimmune hemolytic anemia (AIHA) are extremely rare and cases with the mixed-type AIHA has not been reported.[ncbi.nlm.nih.gov]
  • Laboratory evaluation revealed microhematuria, mild leukocytosis, anemia, and an elevated serum total protein level and Westergren sedimentation rate.[nejm.org]
  • […] of upper respiratory tract including larynx or tracheal cartilage; (f) cochlear or vestibular damage with sensorineural hearing loss, tinnitus or vertigo Laboratory Nonspecific elevated sedimentation rate, mild leukocytosis, normochromic normocytic anemia[pathologyoutlines.com]
  • Supportive, nonspecific findings include increased ESR, anemia of chronic disease, and hypergammaglobulinemia. Antibodies to type II collagen are found in 50% of patients.[rheumaknowledgy.com]
Malaise
  • The literature on relapsing polychondritis has been reviewed, and an additional case with swollen tender, painful ears, and polyarthritis associated with fever and malaise is presented.[archderm.jamanetwork.com]
  • Although chondritis, perichondritis, and iritis have been reported as frequent components of this syndrome, fever, malaise, and an elevated sedimentation rate[jamanetwork.com]
  • It often starts with nonspecific symptoms including fever and malaise, similar to the flu. On the average, it takes about three years after symptoms begin to get the diagnosis. Most patients are in the age range 40 to 60 years.[empowher.com]
  • DESIGN/METHODS: A former healthy 65 year old man developed transient scleritis of both eyes, followed by chondritis of both ears and 3 months later presented with acute memory loss, malaise and fatigue.[n.neurology.org]
Constitutional Symptom
  • Constitutional symptoms are often present (fever, fatigue, weight loss). Because of the variability in clinical presentation, diagnosis is often delayed up to 2 years.[clinicaladvisor.com]
  • Immunofluorescence studies most often reveal faint deposists of C3, IgG or IgM in the primarily mesangium. [4] Constitutional symptoms [ edit ] These symptoms could consist of asthenia , fever , anorexia , and weight loss .[en.wikipedia.org]
  • Immunofluorescence studies most often reveal faint deposists of C3, IgG or IgM in the primarily mesangium. [4] Constitutional symptoms These symptoms could consist of asthenia , fever , anorexia , and weight loss .[infogalactic.com]
Chest Pain
  • The patient came to the hospital with a history of chest pain for one month.[scielo.br]
  • However, during much of 2006, I did experience several episodes of chest pain, lasting from a few seconds to 50 minutes at the longest. I was told that it might be angina.[tedmontgomery.com]
  • She was re-admitted 2 months later with left-sided chest pain, cough and fever. There was tenderness over the costochondral joints anteriorly and over the manubrium and xiphoid regions. She was treated with antibiotics and discharged.[ispub.com]
  • pain (at least 70%) Acropapular dermatitis SENLAT Syndrome (aka TIBOLA/DEBONEL) Necrotic eschar of scalp Painful lymphandenopathy Joint involvement- knee and ankle Swelling of ear Blue-coloured earlobe Painless ear swelling with redness Pinna- deformed[sites.google.com]
Mitral Valve Prolapse
  • Echocardiography showed a mild mitral valve prolapse and regurgitation. Our patient had the history of auricular polychondritis but she had not been diagnosed.[ncbi.nlm.nih.gov]
  • Aortic regurgitation is seen in 4 to 6 % and mitral regurgitation or mitral valve prolapse in 2 to 4 % [ 5 , 6 ].[link.springer.com]
Cauliflower Ear
  • Chronic disease may result in a flabby, droopy ear, cauliflower ear, or saddle nose deformity. Acute involvement of the tracheal cartilage may cause collapse of the airway with obstruction and pulmonary infections.[histopathology-india.net]
  • Chronic disease may result in a flabby, droopy ear or in a cauliflower ear. Involvement of the nose may result in saddle nose deformity.[dermnetnz.org]
  • ear and saddle node deformities Clinical diagnosis requires 3 of the following - (a) recurrent chondritis of both auricles; (b) nonerosive inflammatory arthritis; (c) chondritis of nasal cartilage; (d) ocular inflammation including conjunctivitis, keratitis[pathologyoutlines.com]
  • ear deformity may result. [3] The outer part of the ear may be either floppy or hardened by calcifications of the scar tissue that replaces the cartilage. [3] These cauliflower ear deformities occur in about 10% of persons with RP. [3] Nose [ edit ][en.wikipedia.org]
Arthritis
  • However, Chinese juvenile RP had more severe ocular inflammation (57 %: 40-47 %), arthritis (100 %: 71-90 %), cardiovascular (14 %: 3-10 %) and skin involvement (20 %: 10-11 %) than Caucasian juvenile RP.[ncbi.nlm.nih.gov]
  • Seminars in arthritis and rheumatism . 42 (1): 70–83. doi : 10.1016/j.semarthrit.2011.12.007 . PMID 22417894 . a b McAdam, LP; O'Hanlan, MA; Bluestone, R; Pearson, CM (May 1976).[en.wikipedia.org]
  • She also had ear condritis and arthritis, being treated with prednisolone and methotrexate.[ncbi.nlm.nih.gov]
  • The clinical presentation is heterogeneous and an association with other autoimmune disorders such as rheumatoid arthritis or different forms of vasculitis has been described.[ncbi.nlm.nih.gov]
  • Rheumatoid arthritis (RA) is a systemic, chronic inflammation of the joints resulting in synovitis.[ncbi.nlm.nih.gov]
Arthralgia
  • However, arthralgia recurred as the dose of methylprednisolone was tapered to 30 mg daily. Concomitant treatment with sulphasalazine 1000 mg b.i.d. was initiated but failed to improve the arthralgia consistently within 6 months.[rheumatology.oxfordjournals.org]
  • The five most common symptoms of patients with RP diagnosed with (18)F-FDG PET-CT were cough, fever, chest tightness, sore throat and arthralgia.[ncbi.nlm.nih.gov]
  • These include: Weight loss External ear pain Dizziness , vertigo , tinnitus (ringing in the ears) Ataxia of vestibular origin (loss of balance) Hearing impairment—46 percent of patients in later stages suffer from hearing problems Arthralgia (joint pain[belmarrahealth.com]
  • Nasal cartilage inflammation is the next most common manifestation, followed by arthritis that varies from arthralgias to symmetric or asymmetric nondeforming arthritis involving large and small joints, with a predilection for the costochondral joints[merckmanuals.com]
  • Most commonly the ears and noses of middle-aged people are affected with episodes of tender swelling, often accompanied by fever, arthralgias, and episcleritis.[medical-dictionary.thefreedictionary.com]
Joint Effusion
  • ADHD) Cognitive Impairments Insomnia Hypersomnia Poor c oncentration Malaise Lethargy Acute hip pain Marrow edema Joint swelling Arthritis Ankle arthritis Oligoarthritis Monoarthritis Monoarticular arthritis Pauciarticular arthritis Temporomandibular joint[sites.google.com]
Psychiatric Manifestation
  • If the CNS is involved, it can result in psychiatric manifestations. Patients with RP always respond well to glucocorticoids and immunosuppressants.[ncbi.nlm.nih.gov]
Impulsivity
  • We report a patient with RP who showed progressive severe bronchial stenosis on three-dimensional computed tomography (3D-CT) and impulse oscillation (IOS) with 3D color imaging using a Mostgraph .[ncbi.nlm.nih.gov]

Workup

No laboratory finding is necessary to confirm a diagnosis of relapsing polychondritis, but it may assist in identifying associated complications. Common baseline blood investigations which might be helpful include complete blood count, blood urea nitrogen, erythrocyte sedimentation rate (ESR), acute phase protein levels. If there is anemia, peripheral blood film typically reveals a normochromic normocytic finding. ESR and C-reactive proteins are usually elevated. Serology is also indicated and includes antinuclear antibody (ANA) test and antineutrophil cytoplasmic antibody (ANCA) titers.

Urinalysis and liver function tests may also be necessary, especially as a workup for vasculitis. Purified protein derivative test is necessary to exclude tuberculosis as an infective cause of polychondritis. Screening tests for syphilis are also indicated.

Cultures are necessary and should be determined by the presentation of the disease. Sputum cultures and acid fast bacilli should be considered in patients with respiratory symptoms and disease signs. Cartilage biopsies from the airway should also be sent for bacterial, fungal, and acid-fast bacilli cultures. If fever is present, blood cultures may be necessary; cultures of the cerebrospinal fluid is necessary to exclude meningitis or CNS vasculitis.

Imaging studies are necessary for detailed evaluation of the pathology and exclusion of systemic complications. Chest X-rays, spiral CT scan (without contrast) PET scans, and MRI are also indicated [14] [15].

Treatment

Mild cases of relapsing polychondritis may respond well to nonsteroidal anti-inflammatory drugs (NSAIDs). However, oral corticosteroids are the mainstay of treatment and are reserved for moderate to severe cases of the disease. Initial treatment is administered at 0.5 to 1.0 mg/kg. Immunosuppressants including azathioprine, dapsone and cyclosporine are reserved for recalcitrant cases which are unresponsive to corticosteroids [16].

Surgery may be indicated for gross complications such as aortic aneurysm, aortic valve incompetence, and airway obstruction.

Prognosis

The disease relapses and remits in an episodic pattern with each episode being more severe than the previous. Each episode usually lasts several weeks, with periods of remission lasting several years.

The prognosis of the disease is determined by the degree of systemic involvement and treatment responsiveness of the patient. Involvement of the larynx, trachea, and bronchi may cause a life-threatening acute respiratory obstruction.

Airway involvement occurs in about 50% of all cases, while cardiovascular involvement occurs in 24-52% of cases. Cardiovascular complications are the second most common cause of mortality in patients with relapsing polychondritis [10]. The commonest cause of death in relapsing polychondritis is infection, which is  secondary to steroid therapy or airway compromise, systemic vasculitis, and unrelated coexisting malignancy.

Generally, early diagnosis and prompt treatment are associated with a good outcome in patients.

Etiology

The cause of relapsing polychondritis is largely unknown. A possible role of genetic factors has been suggested. This is based on the findings of familial clusters with the disease and its increased incidence in individuals with HLA-DR4 haplotype.

Some studies have also indicated hormonal factors as etiologic agents in relapsing polychondritis. In one particular study, two men were found to develop relapsing polychondritis after being administered with luteinizing hormone-releasing hormone LHRH). In another report, a woman with a known diagnosis of arthritis mutilans developed an acute exacerbation of the disease with a new onset of auricular damage, hearing loss, weight loss, and nasal septal damage after being injected with human chorionic gonadotropin [3].

Epidemiology

Relapsing polychondritis has an incidence rate of approximately 0.71 per 1 million population annually [4]. Peak onset of the disease occurs at the age of 40-50; however, it affects persons of any age group [5].

Relapsing polychondritis shows no sexual predilection and affect persons of all races, but has been found to be more common among Caucasians.

Sex distribution
Age distribution

Pathophysiology

Researchers have studied the possibility of autoimmunity as the main pathophysiologic mechanism of the disease. Some studies have identified the presence of circulating autoantibodies against cartilage-specific collagens of types II, IX, and XI in 30%-70% of cases. Studies also revealed that autoantibodies to collagen type II are present in the acute phases of the disease and that the levels of the antibodies are directly proportional to the severity of the episode [6]. These anticollagen types I, II, and III antibodies are thought to appear as a result of carriage destruction. Furthermore, antibodies to collagen type II are not specific to relapsing polychondritis, they also occur in rheumatoid arthritis.

Anticollagen IX and XI antibodies have also been identified and are shown to be associated with anticollagen II antibodies. Other studies have identified an increased titer of antibodies to an extracellular matrix protein called matrillin 1 in patients with relapsing polychondritis. This protein is predominantly found in the trachea. This study showed that this antibody titer was higher than in patients with  systemic lupus erythematosus, Wegener's granulomatosis, rheumatoid arthritis, and healthy individuals [7].

Furthermore, researchers have identified five proteins which may be autoantigens linked to the pathophysiology of relapsing polychondritis. These include alpha enolase, vimentin, colligin-1/2, calreticulin, and tubulin-alpha ubiquitous-6. The tubulin-alpha ubiquitous-6 is a family of proteins which are the main constituents of microtubules. Calreticulin is a calcium binding chaperon which is involved in cardiac function.

All these autoantigens, except tubulin alpha, have been associated with common autoimmune diseases such as Behçet disease, rheumatoid arthritis, and mixed connective tissue disease.

The predominant histological feature of relapsing polychondritis is tissue lymphocytic and neutrophilic infiltration. However, there have been few studies done to determine the association of cellular immunity with the pathogenesis of relapsing polychondritis. HLA-DR4 has been linked to an increased risk of relapsing polychondritis. This was also proven by a double-transgenic mouse model which demonstrated that several molecules of HLA class II are required to express predisposition to the disease.

Natural killer T (NKT) cells occur in two forms: CD4+ and CD4-/CD48-. The antigen presenting cells present antigens to these natural killer T cells via CD1d, the major histocompatibility complex (MHC)-like molecule. The number and function of these NKT cells are significantly reduced in autoimmune processes including those involved in type 1 diabetes mellitus, systemic lupus erythematosus, multiple sclerosis, and rheumatoid arthritis open link.

Studies have also observed reduced levels of CD4-/CD-8, CD4+, V- alpha+, and V-beta11 cells in patients with active relapsing polychondritis as compared to their levels in health individuals. It has also been shown that CD4+ NKT cells play significant role in the responsiveness of T1-helper in patients with relapsing polychondritis [8].

In individuals with acute exacerbation of relapsing polychondritis, interleukin 8, monocyte chemoattractant protein-1, and macrophage inflammatory protein 1-alpha were observed to be significantly increased. These chemokines are all pro-inflammatory agents and cause activation of neutrophils, eosinophils and monocytes.

Furthermore, a study confirmed the role of T-cell response to collagen type II in the pathogenesis of relapsing polychondritis. The study identified T cells directed against and specific for a certain region of collagen type II [9].

Prevention

Preventive measures against relapsing polychondritis are not well established as the cause of the disease is unknown. However, prevention of the complications of the disease is necessary as a tertiary method of prevention. A tracheal stent may be inserted in cases with tracheal involvement to prevent airway collapse or obstruction.

Summary

Relapsing polychondritis is a rare inflammatory disease characterized by recurrent episodes of inflammation of the cartilage and cartilaginous tissues. The disease usually involves the ear, nose, cardiovascular system, and the eye.

The etiology of relapsing polychondritis is largely unknown, however autoimmunity, genetic factors, and hormonal involvement have all been shown to contribute to its etiogenesis.

Relapsing polychondritis can occur at any age; however, it has its peak onset in the 5th decade of life. It occurs without sexual or racial preferences.

Presentation of the disease varies from mild inflammatory features to severe life-threatening sequalae [1]. Common features of the disease include unilateral or bilateral ear inflammation presenting as pain, erythema, swelling, and warmth of the pinna, with no involvement of the ear lobule. Ear findings are seen in 90-95% of cases. Involvement of the inner ear and vestibular apparatus may lead to vertigo, tinnitus, nausea, and ataxia. Conductive or sensorineural hearing loss can also occur as a complication of relapsing polychondritis.

Nasal chondritis occurs in 50% of cases and manifests as pain and tenderness of the nasal septum and over the nasal skin. Recurrent damage of the nasal cartilage results in the saddle nose deformity. Airway involvement may cause hoarseness, aphonia, wheezing and stridor, dyspnea, and in severe cases, severe airway obstruction [2].

In close to 50% of patients, cardiovascular involvement is present and may manifest as great vessel vasculitis, aortic aneurysm, and valvular defects

Ophthalmic features of relapsing polychondritis include proptosis, conjunctivitis, periorbital edema, peripheral uveitis, and episcleritis. Skin manifestation include presence of purpura, papules, and aphthosis.

Diagnosis of relapsing polychondritis is mainly clinical, however, laboratory investigations and imaging studies may help to corroborate the diagnosis and exclude possible complications.

Mainstay of treatment of relapsing polychondritis is corticosteroid, however mild cases may resolve with Nonsteroidal anti-inflammatory drugs (NSAIDs).

Patient Information

Relapsing polychondritis is a rare disease characterized by episodes of painful inflammation of the cartilage in the joints and other structures in which cartilages are present such as the nose and the ear. It is characterized by severe pain, redness and swelling of the tissues involved.

The cause of this disease is largely unknown, however, researchers have suggested autoimmunity as the basis for this disease. Autoimmunity is a phenomenon whereby the antibodies, which help fight off harmful substances in the body, confuse normal tissues for foreign bodies. This confusion leads to destruction of the normal tissue by the antibodies.

Because autoimmunity is largely genetic, relapsing polychondritis is therefore said to be possibly inherited. Some other scientific reports have observed that hormones may be involved in the development of this disease. This disease can occur to persons of any age and race, but it occurs most commonly in individuals between 40 to 50 years of age.

Relapsing polychondritis presents in episodic manner with asymptomatic intervals. It usually presents with swelling, redness, and severe pain in one or both ears. Severe cases may present with deafness and loss of balance. Basically, the disease affects any part of the body with cartilage. The nose is also commonly affected and it becomes swollen, red, and weak.

The disease may affect the eye causing blindness, the airway involvement may manifest as cough, shortness of breath, and hoarseness of voice. In severe cases, it may cause obstruction of the airway, such that it becomes difficult taking air into the lungs, this may cause death of the patient. Occasionally, it may affect the heart, causing abnormal heart sounds called murmurs and heart failure.

With recurrent inflammation, cartilage damage may occur causing weakness of the tissues involved. This results in floppy ears and saddle nose. Sometimes, the damage done to the cartilages that connect the ribs to the breastbone makes the chest appear like a hollow space. This condition is called pectus excavatum.

Early diagnosis and treatment of this disease promotes survival of the patient. However, some complications may lead to death of the patient, commonest of these is infection. Other common causes of death in these patients include heart damage and airway compromise.

This condition can be diagnosed without any laboratory investigations. If there are at least three of the following symptoms, a diagnosis of relapsing polychondritis is made:

  • Inflammation (pain, swelling, and redness) of one or both ears.
  • Painful swelling of several joints.
  • Inflammation of the nasal cartilage.
  • Cartilage damage in the airway.
  • Problems with hearing or balance.
  • Inflammation of the eye.

Taking a sample from the affected tissue (commonly the ear) with a needle (biopsy) may reveal characteristic features of the disease when the sample is examined under a microscope. However, blood and urine tests may be necessary to exclude complications of the disease. Computed tomography scans and X-rays of the chest may be recommended if there are signs of airway compromise.

Mild cases of relapsing polychondritis can be treated with nonsteroidal antiinflammatory drugs (NSAIDs) such as ibuprofen. In more severe cases, corticosteroids are prescribed in doses which are gradually reduced as the patient begins to improve. In patients who do not respond to treatment with steroids by resolution of the symptoms, other drugs which suppress the activities of the antibodies are prescribed. These drugs are called immunosuppressants, examples of which include azathioprine, cyclosporine, and cyclophosphamide.

Generally, all these medications do not cure the disease, but alleviate the symptoms. Additionally, surgery may be recommended for some of these complications such as airway obstruction and heart valve damage.

References

Article

  1. Pearson CM, Kline HM, Newcomer VD; Relapsing polychondritis. N Engl J Med. 1960; 263:51-8.
  2. Gorard C, Kadri S; Critical airway involvement in relapsing polychondritis. BMJ Case Rep. Sep 11; 2014. pii: bcr2014205036. doi: 10.1136/bcr-2014-205036. 
  3. Labarthe MP, Bayle-Lebey P, Bazex J. Cutaneous manifestations of relapsing polychondritis in a patient receiving goserelin for carcinoma of the prostate. Dermatology. 1997;195(4):391-4.
  4. Hazra N, Dregan A, Charlton J, et al. Incidence and mortality of relapsing polychondritis in the UK: a population-based cohort study. Rheumatology (Oxford). 2015; 54 (12):2181-7. 
  5. Melikoglu MA, Senel K. Relapsing polychondritis: inflamed joints and ears. Balkan Med J. 2015; 32(1):121-3.
  6. Foidart JM, Abe S, Martin GR, et al. Antibodies to type II collagen in relapsing polychondritis. N Engl J Med. 1978;299(22):1203-7.
  7. Hansson AS, Heinegard D, Piette JC, et al. The occurrence of autoantibodies to matrilin 1 reflects a tissue-specific response to cartilage of the respiratory tract in patients with relapsing polychondritis. Arthritis Rheum. 2001; 44(10):2402-12.
  8. Takagi D, Iwabuchi K, Iwabuchi C, et al. Immunoregulatory defects of V alpha 24V+ beta 11+ NKT cells in development of Wegener's granulomatosis and relapsing polychondritis. Clin Exp Immunol. 2004;136(3):591-600.
  9. Buckner JH, Van Landeghen M, Kwok WW, Tsarknaridis L. Identification of type II collagen peptide 261-273-specific T cell clones in a patient with relapsing polychondritis. Arthritis Rheum. 2002; 46(1):238-44.
  10. Sharma A, Gnanapandithan K, Sharma K, et al. Relapsing polychondritis: a review. Clin Rheumatol. 2013; 32(11):1575-83.
  11. Sharma A, Law AD, Bambery P, et al. Relapsing polychondritis: clinical presentations, disease activity and outcomes. Orphanet J Rare Dis. 2014; 9:198. doi: 10.1186/s13023-014-0198-1.
  12. Michet CJ Jr, McKenna CH, Luthra HS, et al. Relapsing polychondritis: survival and predictive role of early disease manifestations. Ann Intern Med. 1986; 104:74-8.
  13. Plepkorn M, Brown C, Zone J. Auricular chondritis as a rheurnatologic manifestation of Lucio’s phenomenon: clinical improvement after plasmapheresis. Ann Intern Med. 1983; 98:49-51.
  14. Lee KS, Ernst A, Trentham DE, Lunn W, et al. Relapsing polychondritis: prevalence of expiratory CT airway abnormalities. Radiology. 2006; 240(2):565-73.
  15. Yamashita H, Takahashi H, Kubota K, et al. Utility of fluorodeoxyglucose positron emission tomography/computed tomography for early diagnosis and evaluation of disease activity of relapsing polychondritis: a case series and literature review. Rheumatology (Oxford). 2014; 53(8):1482-90.
  16. Bowness P, Hawley IC, Morris T, Dearden A, Walport MJ. Complete heart block and severe aortic incompetence in relapsing polychondritis: clinicopathologic findings. Arthritis Rheum. 1991;34: 97-100.

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Last updated: 2018-06-21 22:33