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1.
Medicine (Baltimore) ; 98(44): e17768, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31689839

RESUMO

Relapsing polychondritis (RP) is a rare immune-mediated disease affecting cartilaginous structures. Respiratory tract manifestations are frequent and constitute a major cause of morbidity and mortality. The present review of the literature was designed to assess the efficacy of tumor necrosis factor alpha (TNF-α) inhibitors in respiratory tract involvement of RP.A MEDLINE literature search was performed from January 2000 to December 2016 to identify all studies and case reports of anti-TNF-α therapy in RP. Articles published in English or French concerning patients with respiratory tract involvement were eligible. Two authors (JB, FL) independently reviewed and extracted data concerning each patient and 2 personal cases were added. Treatment efficacy was assessed according to systemic and/or respiratory criteria.A total of 28 patients (mean age: 41.6 years; 16 females/12 males) were included in the final analysis. Anti-TNF-α therapy was associated with improved health status and respiratory symptoms in 67.8% and 60.1% of cases, respectively.These results suggest that TNF-α inhibitors could be considered for the treatment of respiratory tract involvement of RP.


Assuntos
Antirreumáticos/uso terapêutico , Policondrite Recidivante/tratamento farmacológico , Doenças Respiratórias/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Policondrite Recidivante/complicações , Sistema Respiratório/efeitos dos fármacos , Doenças Respiratórias/etiologia , Resultado do Tratamento
2.
Cutis ; 103(4): 237-240, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31116809

RESUMO

Relapsing polychondritis (RP) is an inflammatory condition that has been widely accepted as autoimmune in nature and can occur in patients with immune system dysregulation. Although RP has been well documented in patients with other autoimmune conditions, such as vasculitis, its presence in patients with human immunodeficiency virus (HIV) infection has been infrequently described. We describe a case of RP in an HIV-positive patient without other identifiable autoimmune disease.


Assuntos
Infecções por HIV , Policondrite Recidivante/diagnóstico , Adulto , Dapsona/administração & dosagem , Dapsona/uso terapêutico , Diagnóstico Diferencial , Orelha Externa/patologia , Humanos , Masculino , Policondrite Recidivante/tratamento farmacológico
3.
BMJ Case Rep ; 12(5)2019 May 23.
Artigo em Inglês | MEDLINE | ID: mdl-31126928

RESUMO

Relapsing polychondritis (RP) is a rare progressive and destructive multisystem disorder characterised by recurrent inflammation of cartilaginous structures. It is a rare disease in paediatrics compared with adults. In children, the diagnosis is either delayed or overlooked due to low incidence. Auricular chondritis has been described in more than half of paediatric cases with RP. However, isolated auricular chondritis has not been reported as the only presentation of pediatric-onset RP. We described a lad who presented with isolated auricular chondritis, which is refractory to conventional treatment, including glucocorticoids and methotrexate as steroid-sparing agent. Remission of his disease's relapses was sustained with infliximab. Limited auricular involvement as a presenting feature of RP in the absence of systemic association is very rare in children. We describe a case of successful use of infliximab on limited auricular chondritis disease.


Assuntos
Policondrite Recidivante/diagnóstico , Adolescente , Antirreumáticos/administração & dosagem , Antirreumáticos/uso terapêutico , Diagnóstico Diferencial , Pavilhão Auricular , Humanos , Infliximab/administração & dosagem , Infliximab/uso terapêutico , Masculino , Policondrite Recidivante/tratamento farmacológico , Policondrite Recidivante/patologia
5.
Rheumatol Int ; 38(11): 2095-2101, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30084004

RESUMO

Observational study of patients with relapsing polychondritis (RPC) and brief evaluation of widely used diagnostic criteria. A retrospective analysis of 18 patients with RPC treated in the past 15 years at the Charté-Universitätsmedizin Berlin was performed. Three different diagnostic criteria were applied to our cohort. Sensitivities of diagnostic criteria of McAdam et al., Damiani and Levine and Michet et al. were calculated as well as the 5- and 10-year survival. Analysis of diagnostic criteria revealed a sensitivity of 88.9% using Damiani and Levine criteria, 66.7% for Michet et al. and 50% for McAdam et al., respectively. Modifying the criteria of Michet et al. increases the sensitivity to 88.9%. The 5- and 10-year survival were 100 and 90.9%, respectively. Current diagnostic criteria in RPC should be reappraised covering the diversity of clinical findings with the aim to improve clinical care and research in RPC.


Assuntos
Policondrite Recidivante/diagnóstico , Adulto , Idoso , Produtos Biológicos/uso terapêutico , Feminino , Alemanha , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Policondrite Recidivante/tratamento farmacológico , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
6.
Drug Metab Pers Ther ; 33(2): 105-108, 2018 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-29715182

RESUMO

BACKGROUND: Relapsing polychondritis (RP) is a rare autoimmune disorder, and myelodysplastic syndrome (MDS) is accompanied by RP at variable rates. Herein, we report a case with RP and MDS who responded dramatically to 5-azacitidine for MDS. CASE PRESENTATION: With conventional immunosuppressive treatment, our patient had several episodes of different side effects, including infections. With the diagnosis of MDS and initiation of azacitidine treatment, all the manifestations of RP disappeared, and remission was achieved for MDS. Although he had relapses of either RP or MDS after several years of azacitidine treatment, all relapses were controlled well with the initiation of azacitidine treatment every time. CONCLUSIONS: Azacitidine should be kept in mind as a treatment option for RP patients with MDS.


Assuntos
Azacitidina/uso terapêutico , Imunossupressores/uso terapêutico , Síndromes Mielodisplásicas/tratamento farmacológico , Policondrite Recidivante/tratamento farmacológico , Adulto , Humanos , Masculino , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/imunologia , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/imunologia , Indução de Remissão , Fatores de Tempo , Resultado do Tratamento
10.
Ann Rheum Dis ; 77(8): 1172-1178, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29535124

RESUMO

OBJECTIVES: To assess the efficacy and the safety of biologics in a cohort of patients with relapsing polychondritis (RP). METHODS: We conducted a French multicentre retrospective cohort study including patients treated with biologics for RP. Efficacy outcomes were clinical response (partial or complete) and complete response during the first 6 months of exposure, plus daily corticosteroid dose at 6 months. Other outcomes were adverse drug reactions (ADRs), persistence of biologics and factors associated with a response. RESULTS: This study included 41 patients exposed to 105 biologics (tumour-necrosis factor (TNF) inhibitors, n=60; tocilizumab, n=17; anakinra, n=15; rituximab, n=7; abatacept, n=6). Overall response rate during the first 6 months of exposure was 62.9%. Complete response rate was 19.0%. Reduced corticosteroid doses were highly variable among patients. ADRs were mostly infections (n=42). Reasons for biologic withdrawal (73.3%) were insufficient efficacy (34.3%; ranging from 23.5% for tocilizumab to 72.7% for etanercept), loss of efficacy (18.1%) and ADRs (20.9%; mostly for anakinra: 46.7%). Persistence was comparable among biologic classes. Among TNF inhibitors, the highest persistence was observed with adalimumab. Differences in clinical response rates were observed depending on biologics and organ involvement. There were trends towards a lower response rate in cases with associated myelodysplastic syndrome and for a higher response rate for nasal/auricular chondritis, sternal chondritis and concomitant exposure to non-biologic disease-modifying antirheumatic drugs. CONCLUSIONS: This study describes the efficacy of biologics for refractory RP. However, the number of complete responses was low and there were concerns about the risk of ADRs, particularly infections.


Assuntos
Produtos Biológicos/uso terapêutico , Policondrite Recidivante/tratamento farmacológico , Adulto , Idoso , Produtos Biológicos/efeitos adversos , Esquema de Medicação , Quimioterapia Combinada , Feminino , Glucocorticoides/administração & dosagem , Humanos , Masculino , Pessoa de Meia-Idade , Indução de Remissão/métodos , Estudos Retrospectivos , Resultado do Tratamento , Fator de Necrose Tumoral alfa/antagonistas & inibidores
13.
Clin Rheumatol ; 37(1): 251-255, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28361234

RESUMO

Aseptic meningitis is an extremely rare neurologic complication of relapsing polychondritis (RP). 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. She was finally diagnosed of aseptic meningitis due to RP after full exclusion of infectious causes. 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. Only 18 previous cases were found in the literature and the clinical manifestation, cerebrospinal fluid (CSF) characteristics, imaging features, and therapy considerations of RP-related aseptic meningitis were summarized by reviewing the literature. Aseptic meningitis due to RP is a rare condition of undetermined pathoetiology. Its diagnosis is primarily based on clinical manifestations combined with CSF and MRI examinations plus adequate exclusion of possible infections. Corticosteroid is the basic therapy but choice of protocol should be individualized.


Assuntos
Encéfalo/diagnóstico por imagem , Meningite Asséptica/etiologia , Policondrite Recidivante/complicações , Ciclofosfamida/uso terapêutico , Ciclosporina/uso terapêutico , Dexametasona/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Imagem por Ressonância Magnética , Meningite Asséptica/diagnóstico por imagem , Meningite Asséptica/tratamento farmacológico , Pessoa de Meia-Idade , Policondrite Recidivante/diagnóstico por imagem , Policondrite Recidivante/tratamento farmacológico , Resultado do Tratamento
14.
Arthritis Care Res (Hoboken) ; 70(8): 1124-1131, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29245173

RESUMO

OBJECTIVE: To assess patient-reported symptoms and burden of disease in relapsing polychondritis (RP). METHODS: Patients with RP completed a disease-specific online survey to identify symptoms attributed to illness. Patients were divided into subgroups based upon presence or absence of ear/nose, airway, or joint involvement. Pathway to diagnosis, treatment, and disease-related complications were assessed within each subgroup. RESULTS: Data from 304 respondents were included in this analysis. Prior to diagnosis, most patients with RP went to the emergency room (54%), saw > 3 physicians (54%), and had symptoms for >5 years (64%). A concomitant diagnosis of fibromyalgia and absence of ear/nose or joint involvement was associated with diagnostic delay >1 year. Common diagnoses prior to RP diagnosis included asthma in patients with airway involvement (35% versus 22%; P = 0.03) and ear infection in patients with ear/nose involvement (51% versus 6%; P < 0.01). Patients with joint involvement were more likely to receive a glucocorticoid-sparing agent (85% versus 13%; P < 0.01). Most patients reported a major complication, including disability (25%), tracheomalacia (16%), or hearing loss (34%). Patients with airway involvement reported more tracheomalacia (20% versus 4%; P < 0.01). Disability (24% versus 7%; P < 0.01) and hearing loss (39% versus 11%; P < 0.01) were prevalent in the joint involvement subgroup. CONCLUSION: Patient-reported data in RP highlight a significant burden of disease. Patterns of organ involvement may lead to diagnostic delay and influence treatment decisions, ultimately impacting the development of disease-related complications. Timely diagnosis, standardization of treatment approaches, and prevention of disease-related complications are major unmet needs in RP.


Assuntos
Medidas de Resultados Relatados pelo Paciente , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/tratamento farmacológico , Perfil de Impacto da Doença , Inquéritos e Questionários , Análise de Variância , Estudos Transversais , Diagnóstico Tardio , Progressão da Doença , Feminino , Humanos , Modelos Logísticos , Masculino , Análise Multivariada , Percepção , Policondrite Recidivante/psicologia , Prognóstico , Qualidade de Vida , Índice de Gravidade de Doença , Estados Unidos
15.
Tuberk Toraks ; 65(3): 245-248, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29135403

RESUMO

Relapsing polychondritis (RP) is a rare autoimmune disease, characterized by episodic inflammatory attacks on cartilaginous tissue. Elastic cartilage of the ears and nose, hyaline cartilage of the peripheral joints, vertebral fibrocartilage, tracheobronchial cartilage, and tissues rich in proteoglycan such as those in the eyes, heart, blood vessels, and inner ear are more likely to be affected. 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. He was diagnosed to have inflammation of the tracheobronchial cartilage, cardiac valvulopathy and conductive hearing loss after intensive diagnostic studies. He responded well to low-dose steroids in combination with methotrexate. RP is a diagnostically challenging condition and may cause significant morbidities during diagnosis. RP should be considered in differential diagnosis of airway obstruction as a rare condition.


Assuntos
Policondrite Recidivante/diagnóstico , Policondrite Recidivante/tratamento farmacológico , Adulto , Diagnóstico Diferencial , Perda Auditiva/etiologia , Humanos , Neoplasias Pulmonares/diagnóstico , Masculino , Policondrite Recidivante/complicações , Esteroides/uso terapêutico
17.
Medicine (Baltimore) ; 96(41): e7951, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29019876

RESUMO

RATIONALE: Relapsing polychondritis (RP) is a rare collagen disease characterized by inflammation and destruction of cartilage throughout the body. The paper details the clinical course of a case of RP with unique circumferential peripheral keratitis. PATIENT CONCERNS: A 54-year-old Japanese woman was referred to the hospital presenting with auricular and ocular pain. DIAGNOSES: Based on the auricle biopsy results and the three presenting symptoms (bilateral auricular chondritis, inflammatory arthritis and ocular inflammation), her condition was diagnosed as RP. INTERVENTIONS: The three presenting symptoms gradually improved with prednisolone (PSL), methylprednisolone and cyclophosphamide combination therapy, followed by PSL, methotrexate and infliximab combination therapy. However, one month after the initial visit, despite ongoing treatment, a unique circumferential peripheral keratitis suddenly occurred, in which the corneal infiltration gradually clumped together and shrank at the peripheral area. The eye and ear pain showed exacerbations and remissions on reducing the dosage of steroid drugs. The general condition was improved on altering systemic therapy to PSL, methotrexate and tocilizumab. OUTCOMES: Keratitis gradually disappeared within 10 months of the initial visit. LESSONS: This is the first report of a case of RP causing unique circumferential peripheral keratitis. This keratitis occurred despite use of focal and systemic steroids and showed improvement with general recovery. This may indicate that stabilization of general condition is important for recovery from keratitis in RP.


Assuntos
Anticorpos Monoclonais Humanizados/administração & dosagem , Artrite , Ciclofosfamida/administração & dosagem , Infliximab/administração & dosagem , Ceratite , Policondrite Recidivante , Prednisolona/administração & dosagem , Antirreumáticos/administração & dosagem , Artrite/etiologia , Artrite/patologia , Técnicas de Diagnóstico Oftalmológico , Monitoramento de Medicamentos/métodos , Quimioterapia Combinada/métodos , Cartilagem da Orelha/patologia , Feminino , Humanos , Ceratite/tratamento farmacológico , Ceratite/etiologia , Ceratite/patologia , Ceratite/fisiopatologia , Metotrexato/administração & dosagem , Pessoa de Meia-Idade , Policondrite Recidivante/complicações , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/tratamento farmacológico , Policondrite Recidivante/fisiopatologia , Resultado do Tratamento
18.
Int J Dermatol ; 56(12): 1379-1386, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28994110

RESUMO

BACKGROUND: Relapsing polychondritis is an autoimmune multisystemic disease with primary chondral involvement. Its high mortality and morbidity make it a real clinical challenge. CASE DESCRIPTION: A 32-year-old woman with a history of relapsing polychondritis, refractory to multiple treatments, with multisystem compromise, imminent risk of death due to severe tracheobronchial damage and difficult ventilatory support, and successful treatment with infliximab. DISCUSSION AND EVALUATION: Several treatments have been described in the literature, such as nonsteroidal anti-inflammatory drugs, corticosteroids, dapsone, azathioprine, cyclosporine, cyclophosphamide, and methotrexate. However, the cases refractory to conventional therapy may lead to chronicity, irreversibility, and death. As a result, a third-line therapy could improve the prognosis of these patients. CONCLUSIONS: Biological therapy is a good option for disease control and quality of life improvement. In addition, the physician should consider these treatments to avoid the chronicity and risk of death of these patients.


Assuntos
Fármacos Dermatológicos/uso terapêutico , Infliximab/uso terapêutico , Policondrite Recidivante/tratamento farmacológico , Adulto , Feminino , Humanos , Policondrite Recidivante/complicações , Policondrite Recidivante/diagnóstico , Terapia de Salvação
20.
Autoimmun Rev ; 16(9): 903-910, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28705782

RESUMO

BACKGROUND: Systemic inflammatory and autoimmune diseases (SIADs) associated with myelodysplastic syndromes are often difficult to treat. Corticosteroids are efficient but only usually at high doses. The use of biologics needs to be specified. METHODS: In a French multicenter retrospective study, we analyzed the efficacy and safety of biologics (tumor necrosis factor-α [TNF-α] antagonists, tocilizumab, rituximab and anakinra) for SIADs associated with myelodysplastic syndromes (MDSs). Clinical, biological and overall treatment responses were evaluated. When several lines of treatment were used, data were analyzed before and at the end of each treatment line and were pooled to compare overall response among steroids, disease-modifying anti-rheumatic drugs (DMARDs) and biologics. RESULTS: We included 29 patients (median age 67years [interquartile range 62-76], 83% males) with MDS-related SIADs treated with at least one biologic. The MDSs were predominantly refractory anemia with excess blasts 1 (38%) and refractory cytopenia with multilineage dysplasia (21%). The SIADs were mainly arthritis (n=6; 20%), relapsing polychondritis (n=8; 30%) and vasculitis (n=10; 34%). During a 3-year median follow-up (IQR 1.3-4.5), a total of 114 lines of treatments were used for all patients: steroids alone (22%), DMARDs (23%), TNF-α antagonists (14%), anakinra (10%), rituximab (10%), tocilizumab (7%) and azacytidine (9%). Considering all 114 lines, overall response (complete and partial) was shown in 54% cases. Overall response was more frequent with steroids (78%) and rituximab (66%) than DMARDs (45%) and other biologics (33%) (p<0.05). Rituximab had better response in vasculitis and TNF-α antagonists in arthritis. During follow-up, 20 patients (71%) presented at least one severe infection. CONCLUSION: This nationwide study demonstrates the efficacy of steroids for SIAD-associated MDSs but a high frequency of steroid dependence. The response to biologics seems low, but rituximab and azacytidine seem promising.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Síndromes Mielodisplásicas/tratamento farmacológico , Policondrite Recidivante/tratamento farmacológico , Idoso , Anticorpos Monoclonais Humanizados/farmacologia , Anticorpos Monoclonais Humanizados/uso terapêutico , Antirreumáticos/farmacologia , Artrite Reumatoide/mortalidade , Produtos Biológicos/farmacologia , Produtos Biológicos/uso terapêutico , Intervalo Livre de Doença , Quimioterapia Combinada , Feminino , França , Humanos , Fatores Imunológicos/uso terapêutico , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/mortalidade , Policondrite Recidivante/mortalidade , Estudos Retrospectivos , Rituximab/farmacologia , Rituximab/uso terapêutico , Resultado do Tratamento
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