Objective:Explore the clinical diagnosis and treatment strategies of recurrent multiple chondritis in children with airway involvement as the main cause. Methods:From June 2021 to May 2023, five children with relapsing polychondritis were admitted to our department, all of whom met the Damiani criteria. Tracheotomy was performed in all five patients. Endoscopy indicated subglottic stenosis, with one case classified as grade Ⅲ and four cases as grade Ⅳ according to the Myer-Cotton classification. Enhanced CT scans of the head and neck showed fixed lumen narrowing and stenosis, including thickening of the soft tissue in the laryngeal cavity and partial absorption of the laryngotracheal cartilage anterior wall. In one patient, tracheal wall thickening and deformation with stenosis and calcification involved the posterior tracheal wall. One patient underwent "laryngeal tracheal reconstruction with hyoid graft, T-tube implantation, and bioabsorbable corticosteroid-eluting stent implantation", while three patients underwent "balloon dilatation, T-tube implantation, and bioabsorbable corticosteroid-eluting stent implantation" . Follow-up was performed every two months after the initial surgery, and three bioabsorbable corticosteroid-eluting stents were placed on the upper left, upper right, and lower T-tube, respectively, during this time. The entire follow-up period was six months. Results:All the 5 cases were in the outpatient department, of which 1 case had been extubation, 3 cases were still in the outpatient follow-up, and the remaining 1 case was still being treated in the rheumatology and immunology department due to poor control of the primary disease. Conclusion:In relapsing polychondritis children with airway involvement, tracheotomy could be used to quickly improve the symptoms of dyspnea in the disease progression. In the stable stage of the disease, the surgical method of 'Balloon dilatation + T-tube implantation + Bioabsorbable corticosteroid-eluting stents implantation' was adopted to reduce the secondary injury caused by surgical trauma as much as possible and improve the survival and quality of life of the children.
Humans ; Polychondritis, Relapsing/surgery* ; Child ; Male ; Female ; Tracheotomy ; Laryngostenosis ; Child, Preschool ; Trachea/surgery* ; Adolescent ; Stents

Relapsing polychondritis (RP) is a disease that confounds clinicians worldwide due to the difficulty in confirming its diagnosis and determining the best treatment plan. We report a case of a 34-year-old lady who presented with bilateral inflamed auricular swelling and red eyes, which turned out to be relapsing polychondritis. The patient was successfully treated with long-term oral steroids and is on regular follow-up. This report highlights how the subtle signs of her condition were picked up, leading to her diagnosis of RP and the treatment regime that successfully reversed her symptoms. The challenge of picking up subtle signs of the disease is best met by working as a team in a multidisciplinary setting. A literature study shows that RP is a rare disease that can present with many symptoms.
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

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

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

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

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.
Atrophy* ; Autoimmune Diseases ; Brain* ; Bronchi ; Cartilage ; Ear ; Encephalitis* ; Heart ; Inflammation ; Joints ; Larynx ; Multiple Sclerosis ; Neurologic Manifestations ; Neuromyelitis Optica ; Nose ; Polychondritis, Relapsing* ; Skin ; Therapeutic Uses ; Trachea

No abstract available.
Cranial Nerve Diseases ; Granuloma, Plasma Cell* ; Polychondritis, Relapsing*

Relapsing polychondritis is a rare, multisystem autoimmune disease. It is characterized by recurrent inflammation of the cartilage and connective tissues in the body. In this paper, we described a case of relapsing polychondritis initially presented symptoms of cognitive dysfunction and personality changes. A 63-year-old male reprented fever, cognitive impairment and personality changes. Brain magnetic resonance imaging revealed leptomeningeal and periauricular hyperintensities. A cerebrospinal fluid examination showed aseptic meningitis. As he reprented hearing difficulties, audiometry showed the sensory neural hearing loss. On physical examination, erythematous swollen auricles were noted. Auricle biopsy consisted with inflammation with perichondritis. He was diagnosed with relapsing polychondritis accompanied by leptomeningeal meningitis, and treated with methylprednisolone (62.5 mg/day for 3 days) followed by prednisolone 60 mg/day and methotrexate 7.5 mg/week. Fever and painful swellings on the both ears subsided. He showed improvement in cognitive function and personality. Although relapsing polychondritis is rare, it should be considered to be a possible cause of leptomeningeal meningitis.
Audiometry ; Autoimmune Diseases ; Biopsy ; Brain ; Cartilage ; Cerebrospinal Fluid ; Cognition ; Cognition Disorders* ; Connective Tissue ; Ear ; Fever ; Hearing ; Hearing Loss ; Humans ; Inflammation ; Magnetic Resonance Imaging ; Male ; Meningitis ; Meningitis, Aseptic ; Methotrexate ; Methylprednisolone ; Middle Aged ; Physical Examination ; Polychondritis, Relapsing* ; Prednisolone

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