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ResumoPolicondrite recidivante é uma condição rara, imunomediada, caracterizada por episódios de inflamação de estruturas cartilaginosas, principalmente orelhas, nariz, articulações e sistema respiratório. Acomete também estruturas ricas em proteoglicanos como olhos, coração, vasos sanguíneos e ouvido interno. Em torno de 1/3 dos casos apresentam associação com outras doenças como vasculites sistêmicas, doenças do tecido conectivo ou síndrome mielodisplásica. Desordens do ouvido interno ocorrem em 40%‐50% dos pacientes. Perda auditiva profunda é rara. O objetivo do estudo foi descrever o caso de uma paciente com diagnóstico de policondrite recidivante associado à perda auditiva severa bilateral e manifestações clínicas de vasculite sistêmica. Este trabalho reforça a importância do diagnóstico precoce e do tratamento imediato em caso de manifestações severas da doença.AbstractRelapsing polychondritis is an uncommon, immune‐mediated condition characterized by episodes of inflammation of cartilaginous structures, especially the ears, nose, joints and respiratory tract. RP also affects proteoglycan‐rich structures such as the eyes, heart, blood vessels and inner ear. Around one third of cases are associated with other diseases such as vasculitides, connective tissue diseases or myelodysplastic syndrome. Disorders of the inner ear occur in 40‐50% of patients. Profound hearing loss is rare. The aim of this study was to describe the case of a patient with relapsing polychondritis associated with severe bilateral hearing loss and clinical manifestations of systemic vasculitis. This study reinforces the importance of an early diagnosis and immediate treatment in case of severe manifestations of the disease.

Policondrite recidivante é uma condição rara, imunomediada, caracterizada por episódios de inflamação de estruturas cartilaginosas, principalmente orelhas, nariz, articulações e sistema respiratório. Acomete também estruturas ricas em proteoglicanos como olhos, coração, vasos sanguíneos e ouvido interno. Em torno de 1/3 dos casos apresentam associação com outras doenças como vasculites sistêmicas, doenças do tecido conectivo ou síndrome mielodisplásica. Desordens do ouvido interno ocorrem em 40%-50% dos pacientes. Perda auditiva profunda é rara. O objetivo do estudo foi descrever o caso de uma paciente com diagnóstico de policondrite recidivante associado à perda auditiva severa bilateral e manifestações clínicas de vasculite sistêmica. Este trabalho reforça a importância do diagnóstico precoce e do tratamento imediato em caso de manifestações severas da doença. Relapsing polychondritis is an uncommon, immune-mediated condition characterized by episodes of inflammation of cartilaginous structures, especially the ears, nose, joints and respiratory tract. RP also affects proteoglycan-rich structures such as the eyes, heart, blood vessels and inner ear. Around one third of cases are associated with other diseases such as vasculitides, connective tissue diseases or myelodysplastic syndrome. Disorders of the inner ear occur in 40–50% of patients. Profound hearing loss is rare. The aim of this study was to describe the case of a patient with relapsing polychondritis associated with severe bilateral hearing loss and clinical manifestations of systemic vasculitis. This study reinforces the importance of an early diagnosis and immediate treatment in case of severe manifestations of the disease.

Relapsing polychondritis is an uncommon, immune-mediated condition characterized by episodes of inflammation of cartilaginous structures, especially the ears, nose, joints and respiratory tract. RP also affects proteoglycan-rich structures such as the eyes, heart, blood vessels and inner ear. Around one third of cases are associated with other diseases such as vasculitides, connective tissue diseases or myelodysplastic syndrome. Disorders of the inner ear occur in 40–50% of patients. Profound hearing loss is rare. The aim of this study was to describe the case of a patient with relapsing polychondritis associated with severe bilateral hearing loss and clinical manifestations of systemic vasculitis. This study reinforces the importance of an early diagnosis and immediate treatment in case of severe manifestations of the disease.

Relapsing polychondritis (RP) is a rare connective tissue disease characterized by inflammation and destruction of cartilaginous tissue1. Although the exact underlying pathologic mechanism is unknown, it is widely accepted that there is a role of an autoimmune response in most cases2. Patients may have lung, renal, ocular, joint, and vascular compromise. Central nervous system (CNS) involvement is rare, and few cases have been described. We describe 3 patients with RP and CNS involvement manifested as hypertrophic pachymeningitis (HP), an inflammatory process that thickens the dura mater.

Paciente con poliartritis crónica de manos y pies, quien además relataba enrojecimiento ocular e inflamación dolorosa de sus orejas en forma recurrente, junto con adenopatías, esplenomegalia y diaforesis nocturna. Se consideró el diagnóstico de policondritis recurrente asociado con neoplasia hematológica probablemente linfoproliferativa. Al final enumeramos algunas manifestaciones reumáticas de trastornos neoplásicos, haciendo especial énfasis en policondritis recurrente como posible manifestación paraneoplásica, resaltando que existen muy pocas asociaciones reportadas en la literatura local e internacional entre policondritis recurrente y linfoma maligno. This a clinical case of a patient with chronic polyarthritis of the hands and feet, who also reported red eyes and recurrent painful inflamation of the ears, as well as a cervical mass, splenomegaly and diaphoresis during the night. The diagnosis of polycondritis was made associated to hematological neoplasia of the leukemia/lymphoma type, recurrent associated to chronic lymphoids. At the end, there is a list of some rheumatic manifestations of neoplastic disorders and special emphasis is placed on relapsing polychondritis as a possible para-neoplastic manifestation, highlighting that there are very few associations reported in the national and international literature between relapsing polychondritis and malignant lymphoma.

La policondritis recidivante es una enfermedad sistémica, de etiología desconocida, que se caracteriza por una inflamación recurrente, que afecta a estructuras cartilaginosas, aparato cardiovascular, ojos y oidos. La afectación respiratoria ocurre en el 56% de los pacientes durante el curso de la enfermedad, pero como forma inicial de presentación sólo ocurre en el 14% de los casos. Los pacientes desarrollan síntomas severos devidos a la afectación de la glotis y del árbol traqueobronquial, siendo la causa de muerte en el 50% de los casos. La inespecificidad de los sintomas respiratorios da lugar a que, si el cuadro no se acompaña inicialmente de signos característicos, pueda confundirse con otro proceso, retrasándose el diagnóstico. Las pruebas de función pulmonar son de enorme importancia, apareciendo un patrón obstructivo no reversible tras broncodilatador, y un plateaud en la curva flujo-volumen que va a ayudar a localizar y evaluar la severidad de la obstrucción. La TAC de alta resolución es una exploración no invasiva que identifica las anormalidades de la tráquea y de los bronquios con mas precisión que la broncofibroscopia, por lo que debe realizarse al inicio de los síntomas respiratorios junto con las pruebas de función pulmonar. Presentamos el caso de una paciente cuyo proceso debutó con sintomatología respiratoria sugestiva de asma bronquial, que precedió en seis meses a la aparición del signo guía la condritis nasal.

La policondritis recidivante es una enfermedad sistémica, de etiología desconocida, que se caracteriza por una inflamación recurrente, que afecta a estructuras cartilaginosas, aparato cardiovascular, ojos y oidos. La afectación respiratoria ocurre en el 56% de los pacientes durante el curso de la enfermedad, pero como forma inicial de presentación sólo ocurre en el 14% de los casos. Los pacientes desarrollan síntomas severos devidos a la afectación de la glotis y del árbol traqueobronquial, siendo la causa de muerte en el 50% de los casos. La inespecificidad de los sintomas respiratorios da lugar a que, si el cuadro no se acompaña inicialmente de signos característicos, pueda confundirse con otro proceso, retrasándose el diagnóstico. Las pruebas de función pulmonar son de enorme importancia, apareciendo un patrón obstructivo no reversible tras broncodilatador, y un plateaud en la curva flujo-volumen que va a ayudar a localizar y evaluar la severidad de la obstrucción. La TAC de alta resolución es una exploración no invasiva que identifica las anormalidades de la tráquea y de los bronquios con mas precisión que la broncofibroscopia, por lo que debe realizarse al inicio de los síntomas respiratorios junto con las pruebas de función pulmonar. Presentamos el caso de una paciente cuyo proceso debutó con sintomatología respiratoria sugestiva de asma bronquial, que precedió en seis meses a la aparición del signo guía la condritis nasal. Relapsing polychondritis is a systemic disease of unknown etiology characterised by relapsing inflammation affecting cartilaginous structures, cardiovascular system, eyes and ears. Respiratory involvement occurs in 56% of patients during the disease progression, but only in 14% of cases as an initial presentation. Patients develop severe symptoms due to the disease affecting the glottis and the tracheobronquial tree, which represents the cause of death in 50% of cases. The unspecificity of respiratory symptoms makes that the disease may be confounded with some other if it is not accompanied with a typical presentation, which may cause a delay in the diagnosis. Pulmonary function tests are of great importance, since an obstructive pattern not reversible after bronchodilator administration and a plateau in flow-volume curves are of great help when assessing the severity of the obstruction. High resolution computed tomography is a non-invasive test more precise than bronchoscopy in identifying tracheal and bronchi abnormalities, so it should be performed at the onset of the respiratory symptoms together with the pulmonary function tests. We present the case of a patient, whose disease started with respiratory semiology suggesting bronchial asthma, which preceded in six months the main sign nasal chondritis.