Recommendations of diagnosis and treatment of relapsing polychondritis in China / 中华内科杂志

Relapsing polychondritis is an immune mediated systemic inflammatory disease, involving the cartilaginous and proteoglycan rich structures. The characteristic manifestations were inflammation and deformity of ear and nasal cartilage. Here, Chinese Rheumatology Association summarized manifestations, diagnosis and disease activity index evaluation of relapsing polychondritis, standardized treatment regimens, to improve disease prognosis.

Encefalopatía subaguda en una paciente con policondritis recidivante: caso clínico / Subacute encephalopathy associated with relapsing polychondritis: report of one case

Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.

Policondritis recidivante: manifestaciones clínicas y tratamiento / Recurrent polychondritis. Clinical, diagnosis and treatment

La policondritis recidivante es una enfermedad rara caracterizada por la inflamación recurrente del cartílago y otros tejidos en todo el cuerpo. La afectación del oído es la característica más común, pero puede afectar otras áreas del cuerpo, incluidos el cartílago costal, los ojos, la nariz, las vías respiratorias, el corazón, el sistema vascular, la piel, las articulaciones, los riñones y el sistema nervioso. Los signos y síntomas varían de persona a persona según las partes del cuerpo afectadas. La causa subyacente exacta de la policondritis recidivante se desconoce, aunque los científicos sospechan que se trata de una enfermedad autoinmune. Las complicaciones respiratorias (colapso de la tráquea e infecciones) son la causa más común de muerte, seguidas de la afectación cardiovascular. Los objetivos principales del tratamiento son aliviar los síntomas actuales y preservar la estructura del cartílago afectado. Gracias a las mejoras en el tratamiento, en los últimos años las tasas de supervivencia han aumentado, incluso después de 10 años(AU)

Recurrent polychondritis is a rare condition characterized by recurrent inflammation of cartilage and other tissues throughout the body. Ear involvement is the most common feature, but a variety of other areas of the body may be compromised, including costal cartilage, eyes, nose, airways, heart, vascular system, skin, joints, kidneys and nervous system. The signs and symptoms vary from person to person depending on the body parts affected. The exact underlying cause of recurrent polychondritis is unknown; however, scientists suspect it is an autoimmune condition. The main goals of treatment for people with recurrent polychondritis are to alleviate current symptoms and preserve the structure of the affected cartilage(AU)

Leucemia mieloide aguda y policondritis recurrente ¿casualidad o relación causal? / Acute myeloid leukemya and relapsing polychondritis. Chance or causal relationship?

La policondritis recurrente o recidivante es una enfermedad sistémica crónica autoinmune, caracterizada por la inflamación de tejidos cartilaginosos asociada en pocos casos a enfermedades malignas hematológicas. Presentamos el caso de una paciente femenina de 26 años que cursaba concomitantemente con leucemia mieloide aguda (LMA). La manifestación inicial fue una afección cutánea en forma de eritema nodoso, y posteriormente se diagnosticó LMA; durante la fase de aplasia posquimioterapia desarrolló inflamación bilateral del cartílago auricular (condritis auricular) y síndrome vertiginoso con evolución clínica satisfactoria al tratamiento inmunosupresor con glucocorticoides. Conclusiones: Es difícil definir si existe asociación entre la policondritis recidivante y la leucemia mieloide aguda, la quimioterapia o la sumatoria de las dos noxas. Una vez que se establece el diagnóstico se debe iniciar oportunamente la administración de glucocorticoide a altas dosis, ya que pudieran aparecer complicaciones como la necrosis del cartílago y la pérdida de la región afectada. En contraste, el uso de los glucocorticoides tiene una excelente respuesta con modulación completa de la enfermedad, tal como se muestra en el caso presentado(AU)

Relapsing polychondritis is a systemic, chronic and autoimmune disease characterized by the inflammation of cartilaginous tissues. This disease is associated in a few cases with malignant hematological diseases. We present a case of a patient with relapsing polychondritis and concomitantly with acute myeloid leukemia. A 26-year-old female patient, with cutaneous affection as initial manifestation categorized as erythema nodosum. Then she was diagnosed with acute myeloid leukemia. In the aplasia post-chemotherapy phase, the patient developed bilateral inflammation of the ear cartilage (auricular chondritis) and a vertiginous syndrome with satisfactory clinical evolution to immunosuppressive treatment with glucocorticoids. Conclusion: Relapsing polychondritis usually presents with cartilaginous involvement, such as bilateral atrial chondritis, as shown in the case. Early diagnosis and timely treatment are necessary to achieve a good clinical response. Subsequent studies are necessary to evaluate the association between relapsing polychondritis and hematological alterations such as acute myeloid leukemia and the use of chemotherapy(AU)

Policondritis recidivante como fenómeno paraneoplásico en paciente con síndrome mielodisplásico / Relapsing Polychondritis as paraneoplastic phenomenon in patient with Myelodysplastic Syndrome

Los síndromes mielodisplásicos son un grupo heterogéneo de enfermedades hematológicas, caracterizadas por hematopoyesis ineficaz con riesgo de progresión a leucemia mieloide aguda. Pueden asociarse a manifestaciones autoinmunes en un 10-30% de los pacientes, apareciendo antes, durante o luego del diagnóstico del trastorno hematológico. La prevalencia de policondritis recidivante como fenómeno paraneoplásico es de 0,7-5,4%, presentándose de forma simultánea en la mayoría de los casos. Otros procesos autoinmunes asociados incluyen: vasculitis sistémica, poliartritis seronegativa, dermatosis neutrofílica, citopenias inmunomediadas, presencia de autoanticuerpos y crioglobulinemia. Reportamos el caso de una mujer de 60 años, sin antecedentes patológicos previos, que presentó un cuadro de policondritis recidivante y vasculitis sistémica asociadas a síndrome mielodisplásico.

Myelodysplastic syndromes are a heterogeneous group of hematological diseases, characterized by ineffective hematopoiesis with risk of progression to acute myeloid leukemia. They can be associated to autoimmune manifestations in 10-30% of patients, appearing before, during or after the diagnosis of the hematological disorder. The prevalence of relapsing polychondritis as a paraneoplastic phenomenon is 0.7-5.4%, occurring simultaneously in the majority of cases. Other associated autoimmune processes include: systemic vasculitis, seronegative polyarthritis, neutrophilic dermatosis, immunomediated cytopenias, presence of autoantibodies and cryoglobulinemia. We report the case of a 60-year-old woman, with no previous medical history, who presented with recurrent polychondritis and systemic vasculitis associated with myelodysplasia.

Relapsing polychondritis, a rare cause of valvulopathy: A review of the medical literature / Policondritis recurrente, una rara causa de valvulopatía: revisión de las publicaciones médicas

Abstract Relapsing polychondritis (RP) is an inflammatory disease that involves cartilaginous structures predominantly in the nose, ears, and respiratory tract. Cardiovascular involvement is not common. Despite this, they are the second cause of death in patients with RP. The structures usually affected by this disease are the heart valves, with regurgitation being the most common valvulopathy. We present the case of a patient without the previous diagnosis of RP who was referred to our institute with heart failure secondary to aortic regurgitation, initially attributed to endocarditis.

Resumen La policondritis recurrente (PR) es una enfermedad inflamatoria que afecta a estructuras cartilaginosas, predominantemente las que se encuentran en nariz, pabellones auriculares y vías respiratorias. Las manifestaciones cardiovasculares son poco comunes; sin embargo, son la segunda causa de mortalidad en pacientes con PR. Unas de las estructuras afectadas casi siempre en la PR son las estructuras valvulares y la valvulopatía más común es la insuficiencia aórtica (IA). A continuación se presenta el caso de una paciente sin diagnóstico previo de PR a quien se refirió a este instituto por insuficiencia cardíaca secundaria a IA, atribuida en un principio a endocarditis.

Policondritis recidivante asociada a enfermedad por IgG4 / Relapsing polychondritis associated with IgG4-related disease

La policondritis recidivante (RP) es un trastorno autoinmune sistémico poco frecuente que se caracteriza por episodio y deterioro progresivo de la inflamación del cartílago. Aproximadamente el 30% de los pacientes con RP tienen enfermedad concurrente. Sin embargo, hay tres casos previos reportados de RP relacionado con enfermedad relacionada con la inmunoglobulina G4 (IgG4-RD). Nosotros presentamos otro caso de una mujer de 37 años que desarrolló RP aproximadamente 1 año antes del diagnóstico de IgG4-RD. La asociación entre ER-IgG4 y RP sigue sin estar clara.

Relapsing polychondritis (RP) is a rare systemic autoimmune disorder characterized by the episodic and progressive deterioration of cartilage inflammation. Approximately 30% patients with RP have concurrent disease. However, there are three cases reports of RP complicated by immunoglobulin G4-related disease (IgG4-RD). Here we report another case of a 37-year-old female who developed RP approximately 1 years before IgG4-RD diagnosis. The association between IgG4-RD and RP remains unclear.

Policondritis recidivante asociada a enfermedad por IgG4 / Recurrent Polychondritis Associated with IgG4 Disease

La policondritis recidivante (RP) es un trastorno autoinmune sistémico poco frecuente que se caracteriza por episodio y deterioro progresivo de la inflamación del cartílago. Aproximadamente el 30% de los pacientes con RP tienen enfermedad concurrente. Sin embargo, hay tres casos previos reportados de RP relacionado con enfermedad relacionada con la inmunoglobulina G4 (IgG4-RD). Nosotros presentamos otro caso de una mujer de 37 años que desarrolló RP aproximadamente 1 año antes del diagnóstico de IgG4-RD. La asociación entre ER- IgG4 y RP sigue sin estar clara.

Relapsing polychondritis (RP) is a rare systemic autoimmune disorder characterized by the episodic and progressive deterioration of cartilage inflammation. Approximately 30% patients with RP have concurrent disease. However, there are three cases reports of RP complicated by immunoglobulin G4-related disease (IgG4-RD). Here we report another case of a 37-year-old female who developed RP approximately 1 years before IgG4-RD diagnosis. The association between IgG4-RD and RP remains unclear

Clinical Features of Relapsing Polychondritis Patients Presented with Arthropathy / 中国医学科学院学报

Objective To explore the clinical characteristics of relapsing polychondritis(RP)patients presented with arthropathy. Methods We retrospectively analyzed the clinical data of 201 RP patients who were hospitalized in our center between December 2005 and February 2019.After 16 patients with co-existing other autoimmune diseases and malignancies were ruled out,185 RP patients entered the final analysis,among whom 16 RP patients were presented with arthropathy and 169 without arthropathy.The demographic data,clinical manifestations,laboratory findings,and prognosis were compared between these two groups. Results Five of the 16 RP patients with arthropathy at presentation were misdiagnosed as rheumatoid arthritis.Compared with RP patients without arthropathy at presentation,RP patients with arthropathy at presentation had a longer disease course[(37.50±66.50)months

18F-FDG PET/CT in diagnosis of relapsing polychondritis / 中国医学影像技术

Objective To explore the value of 18F-FDG PET/CT in diagnosis of relapsing polychondritis (RP). Methods 18F-FDG PET/CT imaging features of 28 patients of RP (RP group) and 28 healthy volunteers (control group) were retrospectively analyzed. The lesions' location, shape, margins, density and the maximum standardized uptake (SUVmax) were analyzed. ROC curve was used to evaluate the efficacy of SUVmax of lesions in diagnosis of RP. Results In all 28 RP patients, increased density, blurred boundary and thickened wall of trachea and bronchus were found in 24 cases. Four cases showed increased density and fuzzy boundary, as well as edema and thickening in larynx cartilages. Totally 91 lesions were found with PET/CT, located in the nasal cartilages, bilateral auricular cartilages, costal cartilages, laryngeal cartilages, trachea and bronchial cartilages, respectively. SUVmax of lesions on each site in RP group was higher than those in control group (all P0.90), while SUVmax of nasal cartilages had a moderate diagnostic value (AUC=0.71). Conclusion 18F-FDG PET/CT manifestations of RP have some characteristics, and can display multiple lesions simultaneously, therefore being helpful to diagnosis of RP.

Reconstructive rhinoplasty with costal cartilage grafting: A case report of relapsing polychondritis

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology characterized by recurrent episodes of inflammation and the destruction of cartilaginous tissues, primarily involving the ear, nose, and the respiratory tract. Nasal chondritis is present in 24% of patients at the time of diagnosis and develops subsequently in 53% throughout the diseases progress. Progressive destruction of nasal cartilage leads to the characteristic flattening of the nasal bridge, resulting in the saddle nose deformity. In patients with RP, surgical management for saddle nose is carefully decided due to the disease relapsing characteristics. We present a RP patient with a saddle nose deformity who underwent reconstruction rhinoplasty with autologous costal cartilage grafting. At 6-month follow-up, the patient retained good esthetic results and showed neither complication nor relapse of RP.

Policondritis recidivante: presentación de un caso / Recurrent polychondritis: A case report

Paciente del sexo femenino de 31 años de edad, antecedentes de buena salud y madre diabética. Acudió a consulta por presentar condritis biauricular y nasal, artralgias y disnea progresiva de unas 3 semanas de evolución. Por su cuadro clínico bastante típico, así como por los resultados de los estudios complementarios, se diagnostica policondritis recidivante (PR), se prescriben esteroides y tratamiento convencional para la insuficiencia respiratoria crónica (IRC)con evolución tórpida a pesar del tratamiento(AU)

A 31-year-old female patient, with healthy medical history and a diabetic mother, attended the clinic for biatrial and nasal chondritis, arthralgia and progressive dyspnea of about three weeks' evolution. Due to its typically clinical picture, as well as to the results of complementary studies, recurrent polychondritis (PR) is diagnosed, steroids and conventional treatment are prescribed for chronic respiratory failure (CRF). Despite treatment, the patient had torpid evolution(AU)

Insidious airway involvement in patients with relapsing polychondritis / 中华风湿病学杂志

Objective To investigate clinical and imaging characteristics of patients with relapsing polychondritis (RP) with insidious airway involvement.Methods Data collected prospectively and consecutively for patients with the diagnosis of RP and their disease activity evaluation was analyzed.The t-test and Mann-Whitney U test were used for statistical analysis between the two groups forquantitative datain normal distri-bution and non-normal distribution respectively,while Chi-square test was use for qualitative data analysis.Results Two hundred and sixteen patients with complete data from Dec 1,2007 to Jul 31,2016 were enrolled with a M:F ratio of 1:1.Mean age of disease onset was (44±16)(8～86) years.The median disease duration was 12 (0.3～480) month.The median relapsing polychondritis disease activity index (RPDAI) was 35 (8～67),the median RPODI was 2.4 (0.1～84).The top three initial presentation were auricular chondritis (28.7％),airway chondritis (24.1％) and ocular involvement (22.2％) respectively.RPODI was significantly higher in auricular chondritis (4.4) (Z=-2.084,P＜0.05) and lower in nose chondritis (0.6) (Z=-2.425,P＜0.05).Up to 81.5％ of the patients were found with airway damage and 52.3％ of them were asymptomatic.Airway damage was mostly located in trachea (79.0％ 139/176) and common features on CT scan werecharacterized by airway wail thickening (72.7％,128/176).Airway narrowing was mostly seen in symptomatic patients while calcification was seen more in asymptomatic patients.Insidious hearing-loss and nose chondritis were found not related to airway damage while obvious auricular chondritis (x2=15.580,P＜0.01),ocular involvement (x2=8.105,P＜0.01) were found to be more in patients with asymptomatic airway damage.All diagnosis before RP was organ-driven.Conclusion Airway involvement in RP is one of the three most common disease initial presentation-sand half of them are asymptomatic.RPODI is a reasonable marker for disease evaluation.Routine follow-up of airway damage (wall thickness,calcification and lumen narrowing) is essential for early RP recognition.

Whole ear involvement in non-elder patients with relapsing polychondritis / 中华风湿病学杂志

Objective To investigate the clinical and audiological characteristics of non-elder patients with relapsing polychondritis (RP).Methods Clinical and audiological data of patients with RP under 60 years old were collected consecutively and analyzed.The t-test and Mann-Whitney U test were used for statistical analysis between the two groups in quantitative data in normal distribution and non-normal distribution respectively,while Chi-square test was use for qualitative data analysis.Results One hundred and seventy four patients with complete data who fulfilled the Michet criteria were enrolled with a M∶F=1∶1.1.The mean age of disease onset was (39±13) (8-60) years;the median time of disease duration was 12 (1-480) months;the median relapsing polychondritis disease activity index (RPDAI) was 38(10-77) and the median RPODI was 2.4(0.1-56).Auricular chondritis (32.8％,57/174),ocular involvement (24.7％,43/174) and airway chonchritis (21.3％,37/174) were the top three onset-pattern.All parts of external,middle and inner ear were involved in RP.Inner-ear damage was the most common (95.4％,166/174) with insidious cochlea and vestibule equally distributed.Auricular chondritis was predominant in external ear involvement (55.2％,96/174);ET dysfunction was included in eardrum abnormalities of neglected middle-ear involvement (29.9％,52/174).Positive HL by active detection was 71.8％(125/174) with 14.3(25/174) HL fulfilled world health organization (WHO)-2006 criteria,including 52.0％(13/25) disabling HL.Sensorineural hearing loss (SNHL) was predominant in RP after 21 years old while 60.0％(6/10) was seen in childhood RP with non-SNHL.At least two parts of ear involvement were seen in almost all patients with heavy overlap.Conclusion All parts of ear are involved in non-elder RP with age related clinical characteristics.Active detection is a key to find insidious middle-and inner-ear involvement for early RP recognition.RPODI is a potential marker for RP evaluation.

Relapsing polychondritis: prevalence of cardiovascular diseases and its risk factors, and general disease features according to gender / Policondrite recidivante: prevalência de doenças cardiovasculares e seus fatores de risco e características gerais da doença de acordo com o gênero

ABSTRACT The comorbidities in relapsing polychondritis have been scarcely described in the literature. Moreover, apart from a few relapsing polychondritis epidemiological studies, no studies specifically addressing relapsing polychondritis distribution according to gender are available. Therefore, the objectives of the present study were: (a) to analyze the prevalence of cardiovascular diseases and its risk factors in a series of patients with relapsing polychondritis; (b) to determine the influence of gender on relapsing polychondritis. A cross-sectional tertiary single center study evaluating 30 relapsing polychondritis cases from 1990 to 2016 was carried out. To compare comorbidities, 60 healthy individuals matched for age-, gender-, ethnicity- and body mass index were recruited. The mean age of relapsing polychondritis patients was 49.0 ± 12.4 years, the median disease duration 6.0 years, and 70% were women. A higher frequency of arterial hypertension (53.3% vs. 23.3%; p = 0.008) and diabetes mellitus (16.7% vs. 3.3%; p = 0.039) was found in the relapsing polychondritis group, compared to the control group. As an additional analysis, patients were compared according to gender distribution (9 men vs. 21 women). The clinical disease onset features were comparable in both genders. However, over the follow-up period, male patients had a greater prevalence of hearing loss, vestibular disorder and uveitis events, and also received more cyclophosphamide therapy (p < 0.05). There was a high prevalence of arterial hypertension and diabetes mellitus, and the male patients seemed to have worse prognosis than the female patients in the follow up.

RESUMO Há escassez de estudos na literatura sobre as comorbidades na policondrite recidivante (PR). Além disso, exceto por alguns estudos epidemiológicos sobre a PR, não existem trabalhos que analisem especificamente a distribuição da PR de acordo com o gênero. Portanto, os objetivos do presente estudo foram: (a) analisar a prevalência de doenças cardiovasculares e seus fatores de risco em uma série de pacientes com PR; (B) determinar a influência do gênero na PR. Fez-se um estudo transversal unicêntrico que avaliou 30 casos de PR entre 1990 e 2016. Para comparar as comorbidades, foram recrutados 60 indivíduos saudáveis pareados por idade, gênero, etnia e índice de massa corporal. A idade média dos pacientes com PR foi de 49,0 ± 12,4 anos. A duração média da doença foi de 6,0 anos e 70% eram mulheres. Foi observada uma maior frequência de hipertensão arterial (53,3% vs. 23,3%, p = 0,008) e diabetes mellitus (16,7% vs. 3,3%; p = 0,039) no grupo PR em comparação com o grupo controle. Em uma análise adicional, os pacientes foram comparados de acordo com a distribuição de gênero (nove homens versus 21 mulheres). As características clínicas iniciais da doença foram comparáveis em ambos os sexos. No entanto, durante o período de seguimento, os pacientes do sexo masculino tiveram maior prevalência de perda auditiva, envolvimento vestibular e eventos de uveíte e também receberam mais tratamento com ciclofosfamida (p < 0,05). Houve uma alta prevalência de hipertensão arterial e diabetes mellitus e os pacientes do sexo masculino apresentaram pior prognóstico do que as pacientes do sexo feminino no seguimento.

The value of 18F-fluorodeoxyglucose posotron emission tomography/computed tomography for detecting the relapse of polychondritis in patients with fever of unknown origin / 中华风湿病学杂志

Objective To analyze the value of 18F-fluorodeoxyglucose (FDG) posotron emission tomography/computed tomography (PET/CT) in the detection of relapsing polychondritis (RP) in fever of unknown origin (FUO) patients.Methods Retrospective analysis of the clinical data of 3 patients with FUO,who were final clinical diagnosed as RP,were conducted.Clinical symptoms,laboratory tests and 18F-FDG PET/CT image data were analyzed,and literature were reviewed.Results Three patients with FUO,2 had increased FDG uptake in PET/CT in multiple cartilages,including ear,nose,trachea/bronchial and rib cartilage (SUVmax 2.5-7.5),which were consistent with RP.One case with a history of RP,PET/CT detected pulmonary infla rrmatory lesions which might be the cause of fever.At the same time,PET/CT showed RP presentation in CT with nasal and airway structure changes,but there was no abnormal FDG uptake.Conclusion 18F-FDG PET/CT has an important clinical value in diagnosis and differential diagnosis of RP,and also in follow-up and clinical response observation.

Relapsing polychondritis in Singapore: a case series and review of literature

<p><b>INTRODUCTION</b>This study aimed to describe the clinical presentation, treatment and outcome of patients with relapsing polychondritis (RP) who were seen at a large tertiary-care academic medical institution in Singapore.</p><p><b>METHODS</b>The medical records of all patients diagnosed with RP at the Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, between 2005 and 2013 were reviewed. The diagnosis of RP was made using the modified McAdam criteria.</p><p><b>RESULTS</b>Ten patients were diagnosed with RP during the study period. Among these patients, five fulfilled the modified McAdam criteria and five were probable cases of RP. The most common clinical presentations were auricular chondritis (n = 9), episcleritis or scleritis (n = 5), and large airway involvement (n = 3). All of the patients received prednisolone. Five patients developed haematological disorders. Patients with both RP and haematological disorders had a longer duration of RP symptoms prior to diagnosis, compared to patients with RP who did not develop haematological disorders (average duration of symptoms 14.7 months vs. 4.2 months).</p><p><b>CONCLUSION</b>The high frequency of patients with haematological malignancies in this series was unexpected. Myelodysplastic syndrome has been reported, but other haematological malignancies are rarely associated with RP. As the association between haematological malignancies and RP is currently unclear, the threshold for haematological/lymphoproliferative screening should be lowered in patients with RP.</p>

Relapsing Polychondritis Presented with Encephalitis Followed by Brain Atrophy

Relapsing polychondritis (RP) is a rare autoimmune disease that is characterized by inflammatory reaction of unknown etiology and destruction of cartilaginous structures. Characteristic symptoms of this disease include cartilage inflammation of the ear, nose, larynx, trachea, bronchi, joints, eyes, heart and skin. Concomitance with neurologic symptom is very rare in RP, and the detailed underlying mechanism of neurological involvement associated with RP is not fully understood. We herein described an unusual recurrent case of inflammatory brain lesions associated with RP, with attention to clinical manifestations, autoimmune disease involvement, and therapeutic effects.

Policondritis recidivante / Relapsing polychondritis

La policondritis recidivante, es una infrecuente enfermedad inflamatoria del tejido conjuntivo, de etiología desconocida. Se caracteriza por inflamación episódica y progresiva de tejido cartilaginoso, principalmente del pabellón auricular, nariz y árbol traqueo-bronquial. Se presenta el caso de una paciente femenina de 58 años, que consulta por enrojecimiento y dolor en pabellón auricular derecho, con episodios previos similares en ambos oídos y nariz, además de estudio por poliartritis simétrica. Al examen físico destaca: eritema, edema y dolor local en pabellón auricular, respetando lóbulo. Con exámenes complementarios normales y presencia de tres criterios característicos de McAdam, se diagnostica policondritis recividante, iniciando prednisona oral, con respuesta favorable. Pese a ser una enfermedad poco frecuente, es importante considerarla en casos recurrentes de eritema y dolor local en regiones cartilaginosas, principalmente en pabellón auricular y nariz, de tal forma de lograr un diagnóstico precoz, para suprimir las crisis y mejorar el pronóstico de estos pacientes.

Relapsing Polychondritis (RP) is a rare inflammatory connective tissue disease of unknown etiology. It is characterized by episodic and progressive inflammation of cartilage tissue, especially ear, nose and tracheobronchial tree. We report the case of 58 year old Chilean female patient, who consulted for redness and pain in the right ear, with similar previous episodes in both ears and nose; along with symmetric polyarthritis study it is presented. Physical examination highlighted erythema, edema and local pain in ear, respecting lobe. With normal complementary examinations and presence of three diagnostic criteria of McAdam y col. relapsing polychondritis diagnosed, initiating oral prednisone, with favorable response. Despite being a rare disease, it is important to consider in recurrent cases of erythema and local pain in cartilaginous regions, mainly in ear and nose, so to achieve early diagnosis, to suppress the crisis and improve the prognosis of these patients.

Relapsing polychondritis initially presenting with hoarseness and difficulty breathing in a 21-year-old male

@#<p style="text-align: justify;"><strong>OBJECTIVE:</strong> To present a case of relapsing polychondritis initially presenting with hoarseness and difficulty breathing and to discuss the diagnostic criteria and typical CT scan findings of relapsing polychondritis. <br /><strong>METHODS:</strong> <br /><strong>Design:</strong> Case Report<br /><strong>Setting:</strong> Tertiary Private Hospital in Metro Manila<br /><strong>Patient:</strong> One<br /><strong>RESULT:</strong> A 21-year-old man who was initially managed as a case of bronchial asthma for persistent hoarseness and recurrent difficulty breathing was found to have severe laryngeal edema on endoscopy, and soft tissue expansion of the cricoid cartilage with calcifications and irregular first tracheal ring on CT scan. He also had recurrent eye redness and developed bilateral aural inflammation, and was subsequently diagnosed to have relapsing polychondritis.<br /><strong>CONCLUSION:</strong> Relapsing polychondritis is a rare autoimmune disease characterized by recurrent inflammation and eventual destruction of cartilage throughout the body. Typical manifestations may not always be present, causing a delay in diagnosis. It should be considered in patients with intractable respiratory symptoms not responsive to treatment for upper respiratory tract infections or asthma. A CT scan may reveal signs of cartilage destruction and help in diagnosis.                  </p>