No abstract available.
Central Nervous System* ; Diagnosis* ; Polychondritis, Relapsing*

OBJECTIVE: To enhance the understanding of the diagnosis and treatments of relapsing polychondritis (RP). METHOD: We present a 37-years-old woman with RP, and review the literatures. RESULT: Initial presenting symptoms of the patient was auricle perichondritis. The patient was diagnosed as sudden deafness firstly. After 2 months, she consulted otolaryngologists at second time with a variety of involvement of the ear, nose, larynx or heart. She was diagnosed as relapsing polychondritis finally. CONCLUSION RP was rare autoimmune system disease, early clinical manifestation were atypical, misdiagnosis and diagnostic errors usually occurred. Corticosteroids, immunosuppressive agents, dapsone and surgical operation could be used in these patients to control symptoms.
Adult ; Female ; Humans ; Polychondritis, Relapsing ; diagnosis ; therapy

Relapsing polychondritis is a rare autoimmune disease of unknown etiology with episodic but potentially progressive inflammatory manifestations. Auricular, articular and nasal manifestations are the most frequent disturbances. Fever is one of the manifestations of this disease but it rarely appears as an initial presentation. In this situation, the diagnosis is delayed until other manifestations are obvious. We report a case of relapsing polychondritis, which was presented as fever of unknown origin. Ten months after the onset of fever, auricular chondritis appeared and gave us the key to diagnosis.
Autoimmune Diseases ; Diagnosis ; Fever of Unknown Origin* ; Fever* ; Polychondritis, Relapsing*

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
Arthritis, Rheumatoid ; Delayed Diagnosis ; Diagnostic Errors ; Humans ; Joint Diseases/diagnosis* ; Polychondritis, Relapsing/diagnosis* ; Prognosis ; Retrospective Studies

Relapsing polychondritis is a rare inflammatory disorder affecting the cartilaginous structures throughout the body. Although there is no pathognomonic laboratory tests, clinical features, when coupled with histopathologic findings, enable one to make a diagnosis of relapsing polychondritis. A 26-year-old man presented with purplish, swollen, and tender auricles and injected eyes. His symptoms had developed eight months ago and fluctuated irrespective of various medications such as corticosteroids and antibiotics. Histopathologic examination revealed loss of basophilic staining of the cartilage with some dissolution of its structures. Treatment was initiated with dapsone, However, the remission was too immediate to be considered as a effect of dapsone.
Adrenal Cortex Hormones ; Adult ; Anti-Bacterial Agents ; Basophils ; Cartilage ; Dapsone ; Diagnosis ; Humans ; Polychondritis, Relapsing*

OBJECTIVETo analyze the clinical features, diagnosis, treatment and prognosis of relapsing polychondritis (RP) in childhood.

METHODSA retrospective analysis of three cases of childhood RP from our hospital on clinical features, diagnosis and treatment was performed, data of the other sixteen cases from MEDLINE were also reviewed. Clinical features of all nineteen children with RP were compared with adults with RP.

RESULTSThe age of the three cases at the time of diagnosis ranged from 10 years to 15 years (the minimum age was 2 years in the literature). The course from onset of clinical symptom to making definite diagnosis varied from 3 months to 9 months (from 3 months to 2 years in the literature). In the three cases, childhood RP affected multiple system/organs, and produced diverse clinical manifestations such as arthritis, auricular chondritis, nasal chondritis (leading to a saddle nose deformity), conjunctivitis and so on. Also, tracheostomy was performed because of severe difficulty in breathing for all the 3 cases. Compared with adult RP, involvement of respiratory system existed in 78.9% of childhood patients, which was more frequent and serious than that of adult cases with RP (35.2%); arthritis was the most common clinical manifestation and first symptoms; Fewer children with RP were associated with other autoimmune diseases than adults. Although both erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) blood levels were high in the three cases, anti-nuclear antibody (ANA), double stranded DNA (dsDNA) and extractable nuclear antigen (ENA) were all negative, it was difficult to define the diagnosis of RP because there were no specific laboratory indicators for diagnosis. The confirmation of diagnosis of two cases was relied on clinical criteria, while another case got pathological material for diagnostic support. CT images with three-dimensional reconstruction of the respiratory tract were helpful for early diagnosis of childhood RP. All the three patients were responsive to glucocorticoid, especially to methylprednisolone, however, recurrence of RP was frequent. Treatment with etanercept was successful in one case with refractory RP.

CONCLUSIONSIt is very important to obtain detailed medical history, complete physical examination and do necessary laboratory and imaging tests (e.g. CT images with three-dimensional reconstruction of the airways, pulmonary function tests and so on) for reducing misdiagnosis or missed diagnosis. Glucocorticoid and immunosuppressants are usually effective for childhood RP, but the effects may not be lasting. Etanercept may be a new option to treat refractory RP in childhood.

Adolescent ; Child ; Female ; Humans ; Male ; Polychondritis, Relapsing ; diagnosis ; drug therapy ; physiopathology ; Research Report ; Retrospective Studies

Relapsing polychondritis is a rare multisystem disorder of unknown etiology characterized by episodic recurrent inflammation and degeneration of cartilage and connective tissue. Autoantibodies against cartilaginous collagen may play a crucial role in the pathogenesis of the disease. Laryngotracheobronchial involvements are rare but the most severe manifestations of the diseases. Although uncommon, this disease should be noted and included in a differential diagnosis in patients who complain of difficulty breathing. Along with a review of the related literatures, we report here on an unusual case of bilateral vocal cord immobility as an initial manifestation of relapsing polychondritis.
Autoantibodies ; Cartilage ; Collagen ; Connective Tissue ; Diagnosis, Differential ; Humans ; Inflammation ; Polychondritis, Relapsing ; Respiration ; Vocal Cords

OBJECTIVE: To discuss the clinical characteristics and early diagnosis and treatment of relapsing polychondritis(RP). METHOD: Twenty-six RP patients received the therapies of antibiotics, glucocorticosteroid, immunosuppressive agent,tracheotomy, tracheal sent implantation or non-invasive ventilation. RESULT: Twenty-six cases (88.5%) had auricle involvement, 20 cases (76.9%) had respiratory tract involvement, 18 cases (69.2%) had joints involvement, 10 cases (38.5%) had nose involvement, 6 cases (23.1%) had eyes involvement, 4 cases (15.4%) had cochlea and (or) vestibular involvement. Some patients with cardiac, vascular, kidney damage as well. One case died of severe pulmonary infection. One case died of respiratory failure. The symptoms of other 24 cases were stable or improved in different degrees. CONCLUSION The clinical manifestations are diverse in RP patients, mainly in department of Otolaryngology, the prognoses of patients with airway involvement are worse, and it may improve the curative effect by an early diagnosis and a timely treatment.
Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Polychondritis, Relapsing ; diagnosis ; therapy ; Prognosis ; Stents ; Tracheotomy ; Young Adult

Relapsing polychondritis is a rare autoimmune disease involving the cartilaginous structures of the whole body. Its diagnosis can be difficult when the typical clinical features such as auricular chondritis are absent. Here, we report on a case of a 51-year-old female who presented with cough, dyspnea, and polyarthritis. Chest computed tomography showed the diffuse involvement of tracheobronchial cartilage. According to Damiani's criteria, she was diagnosed as relapsing polychondritis even though there was no unique involvement of auricular cartilage, and high dose steroid and immunosuppressive therapy were then started. This case indicated that patients who have tracheobronchial cartilage involvement without definite auricular chondritis should be considered for relapsing polychondritis as a differential diagnosis.
Arthritis ; Autoimmune Diseases ; Cartilage ; Cough ; Diagnosis ; Diagnosis, Differential ; Dyspnea ; Ear Cartilage ; Female ; Humans ; Middle Aged ; Polychondritis, Relapsing* ; Thorax

Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS complications of RP.
Brain Edema ; Central Nervous System* ; Dementia ; Diagnosis ; Ear ; Humans ; Inflammation ; Joints ; Limbic Encephalitis ; Meningitis, Aseptic ; Meningoencephalitis ; Nose ; Polychondritis, Relapsing* ; Trees