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].

• Relapsing Polychondritis

  Relapsing, Polychondritis, Relapsing [Disease/Finding], polychondritis relapsing, chronic polychondritis, Systemic chondromalacia (disorder), Relapsing polychondritis, Chronic polychondritis, Systemic chondromalacia, Meyenburg's disease, Relapsing polychondritis [fpnotebook.com]

  Relapsing polychondritis is more common in whites and the middle-aged. [visualdx.com]

  Relapsing polychondritis. Radiology 1973 ; 106 : 313 –315 ↵ Gungor F, Ozdemir T, Tucdemir F, Paksoy N, Karayalcin B, Erkilic M. Tc-99m MDP bone scintigraphy in relapsing polychondritis. [ajnr.org]

• Pain

  Nasal pain, hoarseness, throat pain, and difficulty talking are common presenting symptoms. Several clinical criteria have been developed for establishing the diagnosis of relapsing polychondritis (see Diagnostic Pearls). [visualdx.com]

  Other cartilage in the body can be damaged, leading to various symptoms, such as red or painful eyes, hoarseness, cough, difficulty breathing, rashes, and pain around the breastbone. [merckmanuals.com]

  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. [symptoma.com]

  The patient came to the hospital with a history of chest pain for one month. [scielo.br]

• Swelling

  Negative-pressure pulmonary edema (NPPE) due to laryngeal swelling from relapsing polychondritis is rare and has not been reported. [ncbi.nlm.nih.gov]

  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. [symptoma.com]

  There was no history of joint pain or swelling of joints or any other skin lesions in the past. [ijdvl.com]

• Fever

  INTRODUCTION: Fever of unknown origin (FUO) is a common initial presentation leading to a diagnostic challenge. PATIENT CONCERNS: A 3-month history of moderate-to-high fever was reported in an otherwise healthy 54-year-old man. [ncbi.nlm.nih.gov]

  weight loss Laryngitis No symptoms Not conducted n.d. 18.0 118 Steroid 10 Sato et al. [ 18 ] 59/F Initial Fever Hoarseness, cough, sore throat Hearing loss, polyarthritis Not conducted n.d. 5.16 77 Steroid 11 De Geeter et al. [ 19 ] 67/M Initial Fever [doi.org]

  Flares are accompanied by fever, asthenia, weight loss, and sometimes liver or lymph node enlargement. Relapsing polychondritis is frequently associated with other autoimmune diseases, Behçet disease, and myelodysplastic syndromes. [visualdx.com]

• Weight Loss

  Flares are accompanied by fever, asthenia, weight loss, and sometimes liver or lymph node enlargement. Relapsing polychondritis is frequently associated with other autoimmune diseases, Behçet disease, and myelodysplastic syndromes. [visualdx.com]

  [ 19 ] 67/M Initial Fever, weight loss, malaise Sore throat, cough, dyspnoea No symptoms Rib cartilage n.d. 20.0 130 Steroid 12 Yokoyama [ 20 ] et al. 60/M Initial Fever, weight loss, malaise Cough, wheezing No symptoms Not conducted >113 11.59 n.d. [doi.org]

  A 39-year-old man presented to the hospital in April 2011 with a 2-month history of tonsillitis, night sweats, fatigue, weight loss, shortness of breath on exertion and a dry cough. [ncbi.nlm.nih.gov]

  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 [symptoma.com]

• Saddle Nose

  We describe here a case of sarcoidosis that presented with saddle nose resembling relapsing polychondritis. [ncbi.nlm.nih.gov]

  Complications include saddle-nose deformity/joint deformity, vertigo, tinnitus, pulmonary infection, laryngotracheobronchial stricture, scleritis or iritis, epiglottitis, blindness, valve regurgitation, aortic dissection, renal failure, and death. [visualdx.com]

  It is characterized by inflammation and degeneration of cartilage and can result in deformities such as floppy ear and saddle nose. Loss of cartilage in the respiratory tract can lead to respiratory obstruction. [fpnotebook.com]

• Hoarseness

  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]

  Nasal pain, hoarseness, throat pain, and difficulty talking are common presenting symptoms. Several clinical criteria have been developed for establishing the diagnosis of relapsing polychondritis (see Diagnostic Pearls). [visualdx.com]

  Other cartilage in the body can be damaged, leading to various symptoms, such as red or painful eyes, hoarseness, cough, difficulty breathing, rashes, and pain around the breastbone. [merckmanuals.com]

• 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]

  Clinical symptoms include dyspnea, cough, wheezing, stridor, and dysphonia. [ajnr.org]

  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, dyspnea, and stridor/wheeze. [radiopaedia.org]

• Chest Wall Pain

  Symptoms include chest wall pain or, less often, swelling of the involved cartilage. The involvement of the ribs is seen in 35% of persons with RP but is rarely the first symptom. [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]

• Aphthous Stomatitis

  stomatitis Recurrent canker sores 0011107 Renal insufficiency Renal failure Renal failure in adulthood [ more ] 0000083 Scleritis 0100532 Sensorineural hearing impairment 0000407 Thrombophlebitis 0004418 Uveitis 0000554 Showing of 39 | Last updated: [rarediseases.info.nih.gov]

• Heart Block

  Circumscript areas of malperfusion Thromboembolic events Increased CSF cell count CNS vasculitis Stenosis in the left posterior cerebral artery consistent with vasculitic inflammation MCA lesions Advanced heart block Advanced heart block Neurodevelopment [sites.google.com]

  Other manifestations include thoracic and abdominal aortic aneurysms, myocarditis, pericarditis, silent myocardial infarction, abnormal electrocardiograms, paroxysmal atrial tachycardias, and first-degree or even complete heart block (2, 6, 25). [web.archive.org]

  Rhythm disturbances include incidentally discovered electrocardiographic abnormality, paroxysmal atrial tachycardia, first-degree heart block and complete heart block [ 6, 27, 37 ]. [link.springer.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]

• Chest Pain

  The patient came to the hospital with a history of chest pain for one month. [scielo.br]

  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]

  Nausea Abdominal upset and/or pain Loss of appetite Anorexia Backache Hypotonia (floppy baby syndrome) Leg pain Nocturnal pain in back and legs Swollen and/or painful knee Swollen knee Chest pain (at least 70%) Acropapular dermatitis SENLAT Syndrome [sites.google.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. [ncbi.nlm.nih.gov]

  […] gender preference, usually symptomatic in 40s to 60s although affects all ages 90% have involvement of auricular cartilage, usually bilateral, with swelling, erythema and tenderness Earlobes are typically spared Variable relapsing of disease May cause cauliflower [pathologyoutlines.com]

  The ear lobes, containing no cartilage, are classically spared by the inflammation. 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]

• Erythema

  An 87-year-old man with a history of relapsing polychondritis presented to the emergency department after 4 days of worsening left periorbital swelling and erythema. [ncbi.nlm.nih.gov]

  […] process (antibodies to type II collagen) associated with other autoimmune disorders Whites, no gender preference, usually symptomatic in 40s to 60s although affects all ages 90% have involvement of auricular cartilage, usually bilateral, with swelling, erythema [pathologyoutlines.com]

  In 1997 the patient was admitted to our hospital because of swelling and erythema of both ears (Fig. 1 ). Clinical examination revealed inflammation of the cartilage‐containing portion of the ears with sparing of the earlobe. [rheumatology.oxfordjournals.org]

• Arthritis

  Relapsing polychondritis can also develop in patients with preexisting forms of inflammatory arthritis, such as rheumatoid arthritis and systemic lupus erythematosus. [web.archive.org]

  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]

  Immunization of a different strain of rats with native chick type II collagen was associated with auricular chondritis, in addition to the intended collagen-induced arthritis. [emedicine.com]

• 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]

  Arthralgia (this may affect any synovial joint. However, metacarpophalangeal, proximal interphalangeal and knee joints are the most commonly affected). Nasal pain and chondritis. [patient.info]

  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 [msdmanuals.com]

• Neck Pain

  Rib pain Throat or neck pain Trouble breathing and speaking Trouble swallowing Rashes Depending on where RP affects you, it can cause problems with a heart valve or kidney issues as well. [webmd.com]

  Neck stiffness Neck pain Weakness Inability to think clearly Dementia Attention deficit disorder (ADD) Attention deficit hyperactivity disorder (ADHD) Cognitive Impairments Insomnia Hypersomnia Poor c oncentration Malaise Lethargy Acute hip pain Marrow [sites.google.com]

• Joint Effusion

  […] swelling Arthritis Ankle arthritis Oligoarthritis Monoarthritis Monoarticular arthritis Pauciarticular arthritis Temporomandibular joint effusion- TMJ Bed wetting Bladder pain Urinary urgency Testicular pain UTI's Menstrual irregularities Ocular motor [sites.google.com]

• Leg Pain

  Nausea Abdominal upset and/or pain Loss of appetite Anorexia Backache Hypotonia (floppy baby syndrome) Leg pain Nocturnal pain in back and legs Swollen and/or painful knee Swollen knee Chest pain (at least 70%) Acropapular dermatitis SENLAT Syndrome [sites.google.com]

• Anterior Uveitis

  There were old posterior synechiae between the lens and iris, indicating prior anterior uveitis (Panel B). [nejm.org]

  RESULTS: Scleritis in patients with RP was often bilateral (92.3%), diffuse (76.9%), recurrent (84.6%), sometimes with decreased vision (46.2%), anterior uveitis (38.5%), peripheral keratitis (15.4%) and ocular hypertension (30.8%). [ncbi.nlm.nih.gov]

  She has a history of recurrent episodes of episcleritis, scleritis, and anterior uveitis which was controlled by topical steroids, mydriatics, and oral nonsteroidal anti-inflammatory drugs. [nature.com]

  There are also other ocular manifestations that occur in persons with RP, these include keratoconjunctivitis sicca, peripheral keratitis (rarely with ulcerations), anterior uveitis, retinal vasculitis, proptosis, lid edema, keratoconus, retinopathy, iridocyclitis [en.wikipedia.org]

• Chemosis

  Initial presentation was at 5 years of age with periorbital edema and chemosis of the conjunctiva with a definite diagnosis established at 9 years of age following an acute monoarticular arthropathy of the knee. [ncbi.nlm.nih.gov]

  An 83-year-old man previously diagnosed with RPC presented to our clinic with acute unilateral chemosis, conjunctivitis, lid edema, proptosis, and extraocular muscle restriction. [journals.lww.com]

• Headache

  We reported a case of a 58-year-old Chinese female with intractable headache, puffy ears, pleocytosis, and cranial magnetic resonance imaging (MRI) showing thickened and enhanced meninges. [ncbi.nlm.nih.gov]

  Vision – Vision is blurred, headaches, episcleritis, or scleritis Inflammation of the joints is known as arthritis and causes pain, stiffness of all the joints in the knees, hands, wrist, ankles as well as feet. [byebyedoctor.com]

  Diagnosis of Dementia with Lewy Bodies (DLB) Diagnose patients with dementia with Lewy Bodies based on the 2017 criteria Arteriovenous Malformation Coma/Level of Consciousness Demyelinating Disease Dermatome Map Functional Outcome Head & Neck Trauma Headache [qxmd.com]

  With Lyme Disease Fevers and/or chills Migratory pains and stiffness in joints Persisting joint involvement Muscle aches and pain Refractory arthritis Arthritis of the knee Lyme arthritis appearing as septic arthritis or juvenile rheumatoid arthritis Headaches [sites.google.com]

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].

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.

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.

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].

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.

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].

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.

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).

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.

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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.