French practical guidelines for the diagnosis and management of relapsingpolychondritisRelapsingpolychondritis is a rare systemic disease. It usually begins in middle-aged individuals. This diagnosis is mainly suggested in the presence of chondritis, i.e. inflammatory flares on the cartilage, in particular of the ears, nose or respiratory tract, and more rarely in the presence of other manifestations. The formal diagnosis of relapsingpolychondritis cannot be established with certainty before the onset of chondritis, which can sometimes occur several years after the first signs. No laboratory test is specific of relapsingpolychondritis, the diagnosis is usually based on clinical evidence and the elimination of differential diagnoses. Relapsingpolychondritis is a long-lasting and often
Evaluation of Semicircular Canal Function in RelapsingPolychondritis Patients with Dizziness and Sensorineural Hearing Loss Using Video Head Impulse Test. To evaluate semicircular canal function using video head impulse test (vHIT) in relapsingpolychondritis (RP) patients presenting with dizziness and sensorineural hearing loss. Retrospective case review. Tertiary referral center. Three
Relapsingpolychondritis: clinical updates and new differential diagnoses. Relapsingpolychondritis is a rare inflammatory disease characterized by recurrent inflammation of cartilaginous structures, mainly of the ears, nose and respiratory tract, with a broad spectrum of accompanying systemic features. Despite its rarity, prompt recognition and accurate diagnosis of relapsingpolychondritis is crucial for appropriate management and optimal outcomes. Our understanding of relapsingpolychondritis has changed markedly in the past couple of years with the identification of three distinct patient clusters that have different clinical manifestations and prognostic outcomes. With the progress of pangenomic sequencing and the discovery of new somatic and monogenic autoinflammatory diseases, new
Mucosa-Associated Lymphoid Tissue Lymphoma as a Rare Mimicker of RelapsingPolychondritis. Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade B cell extranodal lymphoma characterized by clonal marginal zone lymphocyte proliferation. We report a case of MALT lymphoma with a relapsingpolychondritis (RP)-like phenotype, manifesting as rapidly progressive tracheal stenosis requiring
Clinical analysis of relapsingpolychondritis with airway involvement. To identify the clinical characteristics, treatment, and prognosis of relapsingpolychondritis patients with airway involvement. Twenty-eight patients with relapsingpolychondritis, hospitalised in the First Hospital of Shanxi Medical University between April 2011 and April 2021, were retrospectively analysed. Fifty per cent of relapsingpolychondritis patients with airway involvement had a lower risk of ear and ocular involvement. Relapsingpolychondritis patients with airway involvement had a longer time-to-diagnosis ( < 0.001), a poorer outcome following glucocorticoid combined with immunosuppressant treatment ( = 0.004), and a higher recurrence rate than those without airway involvement ( = 0.004). The rates of positive
RelapsingPolychondritis With Palmoplantar Pustulosis. Palmoplantar pustulosis (PPP) is a very rare cutaneous manifestation observed during relapsingpolychondritis (RP), not found in other conditions associated with saddle nose (eg, granulomatosis with polyangiitis, sarcoidosis, VEXAS [vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic] syndrome, congenital syphilis, leprosy, septal
Ultra-rare genetic variation in relapsingpolychondritis: a whole-exome sequencing study. Relapsingpolychondritis (RP) is a systemic inflammatory disease of unknown aetiology. The objective of this study was to examine the contribution of rare genetic variations to RP. We performed a case-control exome-wide rare variant association analysis that included 66 unrelated European American cases
Comparison of relapsingpolychondritis patients with and without respiratory involvement based on chest computed tomography: a retrospective cohort study. Relapsingpolychondritis (RP) patients with tracheal cartilage involvement are different from other patients. The objective of this study was to compare the clinical features and disease patterns between a respiratory involvement subgroup
Auricular Reconstruction for Bilateral Auricular Deformity Caused by RelapsingPolychondritis. This case report describes woman in her early 20s presented to the hospital with an acquired auricular deformity after an ear piercing and was diagnosed with recurrent polychondritis that required total auricular reconstruction.
Thoracic Manifestations of Ankylosing Spondylitis, Inflammatory Bowel Disease, and RelapsingPolychondritis. Ankylosing spondylitis, inflammatory bowel disease (IBD), and relapsingpolychondritis are immune-mediated inflammatory diseases with variable involvement of lungs, heart and the chest wall. Ankylosing spondylitis is associated with anterior chest wall pain, restrictive lung disease , obstructive sleep apnea, apical fibrosis, spontaneous pneumothorax, abnormalities of cardiac valves and conduction system, and aortitis. Patients with IBD can develop necrobiotic lung nodules that can be misdiagnosed as malignancy or infection. Relapsingpolychondritis involves large airways in at least half of the patients. Relapsingpolychondritis can mimic asthma in some patients. Medications used
Long-term Outcome of Metallic Stenting For Central Airway Involvement In RelapsingPolychondritis. Placement of uncovered self-expandable metallic stents was found to successfully alleviate critical airflow limitation in patients with relapsingpolychondritis (RP) with central airway involvement by several reports. However, the long-term outcome of airway metallic stenting in patients with RP
Patient Perceived Burden of Disease in Pediatric RelapsingPolychondritis. To assess patient-reported burden of disease in pediatric patients with relapsingpolychondritis (RP) and to compare those findings to adult patients. A survey based on known clinical symptoms of RP was developed and administered to patients with a pediatric diagnosis of RP. Adult patients completed a similar survey
Tracheobronchial involvement of relapsingpolychondritis. Recent studies show that relapsingpolychondritis patients with tracheobronchial involvement are distinct from others in terms of clinical characteristics, therapeutic management, and disease evolution. Tracheobronchial involvement affects 20 to 50% of patients and may reveal the disease. It should be sought at the time of diagnosis endoscopic intervention should be considered with caution and performed only by expert endoscopists after careful evaluation of the risks and benefits of such procedures, which can lead to damage or perforation of the airways and bronchospasm. Early detection and management of tracheobronchial involvement in relapsingpolychondritis has significantly improved the prognosis of patients, especially
Case report of mixed-type autoimmune hemolytic anemia in a patient with relapsingpolychondritis. Relapsingpolychondritis (RP) is a rare autoimmune-related disease and may be associated with other autoimmune diseases. Here we reported a case of RP patients with mixed-type AIHA. The patient was diagnosed with RP in March 2008 which was treated and the patient was in stable condition. Laboratory
Pleocytosis in a patient with relapsingpolychondritis accompanied by meningoencephalitis: a case report. Relapsingpolychondritis (RP) is an uncommon immune-related disease with unknown causes. It is characterized by inflammation of cartilaginous or non-cartilaginous structures, such as the ears, nose, respiratory tract, eyes, and joints. Neurological involvement is rare in RP. We report a case
Clinical features of Chinese patients with relapsingpolychondritis. To characterise the ocular and extraocular findings in patients with relapsingpolychondritis (RP) patients. Retrospective, longitudinal study. A total number of 16 patients with ocular RP, seen in our tertiary uveitis referral centre over a time period of 10 years were included in this study. Routine ocular examinations
Relapsingpolychondritis patients were divided into three subgroups: patients with respiratory involvement (R subgroup), patients with auricular involvement (A subgroup), and overlapping patients with both involvements (O subgroup), and each group had dis Relapsingpolychondritis (RP) is a multisystem disorder of cartilaginous tissues. Previously, we found that patients with respiratory
Efficacy and safety of biologics in relapsingpolychondritis: a French national multicentre study. To assess the efficacy and the safety of biologics in a cohort of patients with relapsingpolychondritis (RP). 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
Refractory relapsingpolychondritis: challenges and solutions Relapsingpolychondritis is a severe systemic immune-mediated disease characterized by an episodic and progressive inflammatory condition with progressive destruction of cartilaginous structures. This disease has for nearly a century kept secrets not yet explained. The real incidence and prevalence of this rare disease are unknown . The multiple clinical presentations and episodic nature of relapsingpolychondritis cause a significant diagnosis delay. No guidelines for the management of patients with relapsingpolychondritis have been validated to date. The challenges remain, both in the understanding of its pathophysiology and diagnosis, evaluation of its activity and prognosis, and its treatment. Possible solutions involve the sharing
RelapsingPolychondritis following Treatment with Secukinumab for Ankylosing Spondylitis: Case Report and Review of the Literature Relapsingpolychondritis (RP) is an autoimmune disorder that often occurs concomitantly with other autoimmune diseases, though RP has been infrequently associated with ankylosing spondylitis (AS). There is a small, but growing, body of the literature demonstrating