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

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

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.
Central Nervous System* ; Diagnosis* ; Polychondritis, Relapsing*

Here we describe a case of rapidly expanding ascending aortic aneurysm in a patient with relapsing polychondritis. To prevent aneurysm rupture, the patient underwent emergent surgical repair. Silent inflammation can progress in the aorta wall, even in asymptomatic patients with mild disease activity under immunosuppressive treatment, leading to the rapid growth of aortic aneurysms. Close monitoring with routine imaging is needed once a patient with relapsing polychondritis is diagnosed with an aortic aneurysm.
Aneurysm ; Aorta ; Aortic Aneurysm* ; Aortitis ; Humans ; Inflammation ; Polychondritis, Relapsing* ; Rupture

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

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