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 (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.
Congenital Abnormalities ; Costal Cartilage ; Diagnosis ; Ear ; Follow-Up Studies ; Humans ; Inflammation ; Nasal Cartilages ; Nose ; Polychondritis, Relapsing ; Recurrence ; Respiratory System ; Rhinoplasty ; Transplants

Identification of certain abnormalities of the chest wall can be extremely helpful in correctly diagnosing a number of syndromic conditions and systemic diseases. Additionally, chest wall abnormalities may sometimes constitute diagnoses by themselves. In the present pictorial essay, we review a number of such conditions and provide illustrative cases that were retrospectively identified from our clinical imaging database. These include pentalogy of Cantrell, Klippel-Feil syndrome, cleidocranial dysplasia, Poland syndrome, osteopetrosis, neurofibromatosis type 1, Marfan syndrome, Gardner syndrome, systemic sclerosis, relapsing polychondritis, polymyositis/dermatomyositis, ankylosing spondylitis, hyperparathyroidism, rickets, sickle cell anemia, thalassemia, tuberculosis, septic arthritis of the sternoclavicular joint, elastofibroma dorsi, and sternal dehiscence.
Anemia, Sickle Cell ; Arthritis, Infectious ; Cleidocranial Dysplasia ; Diagnosis ; Gardner Syndrome ; Hyperparathyroidism ; Klippel-Feil Syndrome ; Marfan Syndrome ; Neurofibromatosis 1 ; Osteopetrosis ; Pentalogy of Cantrell ; Poland Syndrome ; Polychondritis, Relapsing ; Retrospective Studies ; Rickets ; Scleroderma, Systemic ; Spondylitis, Ankylosing ; Sternoclavicular Joint ; Thalassemia ; Thoracic Wall ; Tuberculosis

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

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

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

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 (RP) is an uncommon disease that is characterized by inflammation and destruction of cartilaginous structures. When tracheobronchial tree is involved, respiratory obstructive symptoms can occur. A 35-year-old man, with a previous diagnosis of RP, was scheduled for rigid bronchoscopy to relieve dyspnea, caused by subglottic stenosis. After laser splitting of the subglottic web, the spontaneous respiration of the patient was insufficient, and hypercarbia developed progressively even with assisted ventilation. After 20 minutes of aggressive hyperventilation to reduce end-tidal CO2 level, sudden extreme tachycardia and hypotension developed. Ventilation rate was reduced and prolonged expiration time was allowed to alleviate a near-tampon status from dynamic hyperinflation. After the hemodynamic status was stabilized, the patient was transferred to the ICU for mechanical ventilation. He received ICU care for 30 days, and now, he was on supportive care on a ward, considering Y stent insertion to prevent luminal collapse from tracheobronchomalacia.
Adult ; Bronchoscopy* ; Constriction, Pathologic ; Diagnosis ; Dyspnea ; Hemodynamics ; Humans ; Hyperventilation ; Hypotension ; Inflammation ; Laryngostenosis ; Phenobarbital ; Polychondritis, Relapsing* ; Respiration ; Respiration, Artificial ; Respiratory Insufficiency* ; Stents ; Tachycardia ; Tracheobronchomalacia ; Ventilation

Aged ; Biopsy, Fine-Needle ; methods ; Cross-Sectional Studies ; Female ; Humans ; Male ; Middle Aged ; Polychondritis, Relapsing ; diagnosis ; diagnostic imaging ; Positron-Emission Tomography ; methods ; Tomography, X-Ray Computed ; methods

PURPOSE: To report a rare case of a 62-year-old man who presented with bilateral eyelid swelling, chemosis, and hyperemia of the conjunctiva, which wax and waned, being the initial manifestation of relapsing polychondritis. CASE SUMMARY: A 62-year-old man presented with pain and erythematous swelling of the right eyelid for 2 days. There were no other symptoms except chemosis and hyperemia of the conjunctiva. After 1 week, the same symptoms occurred in the left eye, while the lesion of the right eye underwent improvement and aggravation repeatedly. Blood culture tests, Orbital CT, and MRI were performed, but could not confirm a diagnosis. During the follow-up period, erythematous swelling of the left auricle and laryngopharyngitis appeared and erythematous plaques were scattered on the extremities. Punch biopsies were performed; histopathologic examinations concluded to the diagnosis of chondritis. The diagnosis of relapsing polychondritis was confirmed through histologically diagnosed chondritis and repeated ocular symptoms with systemic features. CONCLUSIONS: When patients repeatedly present bilateral eyelid swelling with hyperemia and chemosis of the conjunctiva, relapsing polychondritis should be considered in the differential diagnosis.
Biopsy ; Conjunctiva ; Diagnosis, Differential ; Extremities ; Eye ; Eyelids ; Follow-Up Studies ; Humans ; Hyperemia ; Inflammation ; Middle Aged ; Orbit ; Polychondritis, Relapsing