2,043 results match your criteria: "Polychondritis"

Anakinra in relapsing polychondritis: a case report and review of the literature.

Immunotherapy

December 2024

Department of Clinical Immunology and Allergy, Flinders Medical Centre, Bedford Park, South Australia.

Relapsing polychondritis is rare and affects non-synovial fibrocartilage. Currently, there is a paucity of treatment algorithms, especially for those with refractory disease. A middle-aged man presented with polychondritis affecting the nose, ears, joints, and larynx.

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Relapsing polychondritis (RP) is a rare immune-mediated systemic inflammatory disease with diverse clinical manifestations. Independent involvement of the respiratory system in RP is uncommon. In the event of respiratory involvement as the initial airway-only manifestation, the diagnosis of RP is challenging and might be delayed, and patients with respiratory involvement exhibit a poor prognosis.

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[Clinical characteristics of 6 cases of relapsing polychondritis in children with airway involvement].

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi

November 2024

Department of Otorhinolaryngology Head and Neck Surgery, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery, National Center for Children's Health(NCCH), Beijing100045, China.

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[Living with… chronic atrophic polychondritis].

Rev Prat

November 2024

Service de médecine interne 2, centre de référence des maladies auto-immunes et autoinflammatoires systémiques rares de l'adulte d'Île-de-France, Centre et Martinique, hôpital de La Pitié-Salpêtrière, AP-HP, Paris, France. Association francophone contre la polychondrite chronique atrophiante (AFPCA).

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Mucosa-Associated Lymphoid Tissue Lymphoma as a Rare Mimicker of Relapsing Polychondritis.

J Rheumatol

November 2024

Nobuyuki Yajima, MD, MPH, PhD, Division of Rheumatology, Department of Medicine, Showa University School of Medicine, Tokyo, and Department of Healthcare Epidemiology, School of Public Health, Kyoto University Graduate School of Medicine, Kyoto, and Center for Innovative Research for Communities and Clinical Excellence, Fukushima, Japan.

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Relapsing polychondritis in childhood: A case report.

Medicine (Baltimore)

October 2024

Department of Otorhinolaryngology, Linyi People's Hospital, Linyi, China.

Article Synopsis
  • - Relapsing polychondritis (RP) is a rare autoimmune disease that harms cartilage, particularly in the ears, nose, and airways, making early diagnosis vital to avoid severe airway damage and complications, as highlighted by a case study of a 12-year-old girl.
  • - The patient faced severe respiratory distress and underwent tracheal intubation and later a tracheostomy, with symptoms evolving to joint pain and a saddle nose deformity, leading to a clinical diagnosis of RP.
  • - Treatment included antibiotics, corticosteroids, and the biologic drugs tocilizumab and methotrexate, and three years after diagnosis, the girl is stable, underscoring the importance of prompt recognition of
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  • VEXAS syndrome is a newly identified disorder caused by mutations in the UBA1 gene, leading to serious symptoms like recurrent fevers, inflammation, and organ issues, including potential end-organ damage and death.
  • Treatment options are limited, but using the drug tocilizumab, which targets interleukin-6, has shown promise in managing the condition.
  • A case study of a 71-year-old male patient highlights the effectiveness of tocilizumab followed by siltuximab, with positive outcomes and no relapses reported for over a year.
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  • A case study of a female patient in her 50s with primary amyloidosis affecting multiple organs, alongside a history of relapsing polychondritis and chronic kidney disease.
  • Initial tests were negative for amyloid but a second biopsy confirmed amyloid protein presence as AL-lambda amyloidosis.
  • Despite medical interventions, the patient experienced worsening symptoms, eventually leading to ICU admission and death due to pulseless ventricular tachycardia, emphasizing the importance of accurate diagnosis for treatment decisions.
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  • The study investigates the challenges in diagnosing and managing relapsing polychondritis (RP) with airway involvement, emphasizing the importance of accurate diagnosis to avoid serious complications.
  • Among 34 patients diagnosed with RP, 4 showed severe airway issues after being misdiagnosed with asthma, highlighting the need for better diagnostic practices.
  • Key interventions included steroid treatment, rituximab, and various surgical procedures; early identification of RP and a multidisciplinary approach are crucial for improving patient outcomes and reducing the risk of complications.
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  • A 65-year-old woman with recurrent polychondritis was hospitalized for airway narrowing caused by a fractured metallic stent.
  • She underwent a bronchoscopic procedure where the stent was cauterized using Argon Plasma Coagulation (APC) to improve the airway.
  • The procedure was successful, with the airway ballooning allowing clear passage through the trachea and main bronchi, and no complications were reported.
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Article Synopsis
  • Relapsing Polychondritis (RP) is a rare and serious inflammatory disease that can lead to life-threatening airway collapse.
  • A patient who was misdiagnosed with refractory asthma experienced severe airway collapse, requiring advanced treatments like bronchoscopic laser tracheobronchoplasty and stent placement.
  • After successful stabilization and discharge, the patient managed their symptoms with a tapering glucocorticoid regimen and did not require rehospitalization.
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Red stripe on left forearm after stung by fish gill: A case report.

Medicine (Baltimore)

September 2024

Department of Emergency Medicine, MacKay Memorial Hospital, Taipei, Taiwan.

Rationale: Acute infectious lymphangitis represents a common complication of cellulitis, typically attributed to streptococcal infections after damaged skin integrity.

Patient Concern: This is a 51-year-old woman with a medical history of relapsing polychondritis, managed with steroid and methotrexate therapy in the outpatient department. She presented with a progressive redness and swelling of the left hand, accompanied by purulent discharge, persisting for 5 days.

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Article Synopsis
  • - Relapsing polychondritis (RP) is a rare autoimmune disorder that causes inflammation and damage to cartilage in the body.
  • - The text reports an association between COVID-19 vaccination and the onset of RP, highlighting a possible side effect of the mRNA vaccines.
  • - It emphasizes the need for healthcare providers to be aware that RP could emerge as a complication following vaccination against COVID-19.
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  • The study aimed to assess semicircular canal function through video head impulse tests (vHIT) in patients with relapsing polychondritis (RP) who experienced dizziness and hearing loss.
  • Out of six ears tested, five showed dysfunction in the semicircular canals, with a notable correlation between vestibulo-ocular reflex gain and hearing loss severity.
  • This research highlights the importance of evaluating vestibular function in RP patients and suggests that early diagnosis using vHIT could improve treatment outcomes.
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Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder caused by abnormal histiocytes and T cell activation. In adults, it is predominantly associated with infections, cancers, and autoimmune diseases. Relapsing polychondritis (RP), another rare disease, is diagnosed based on symptoms without specific tests, featuring cartilage inflammation characterized by swelling, redness, and pain, rarely inducing HLH.

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Article Synopsis
  • * A systematic review of studies revealed a median diagnostic delay of 1 year, with most patients presenting common symptoms like ear and nasal chondritis, while treatments primarily included corticosteroids and monoclonal antibodies.
  • * Findings highlight the need for increased awareness and research on pediatric RP, as well as the importance of effective treatments like corticosteroids and therapies such as infliximab.
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Article Synopsis
  • Relapsing Polychondritis (RP) is a rare autoimmune disease, and delaying treatment worsens patient outcomes; current diagnostic criteria lack modern imaging techniques like CT and SPECT/CT that could improve diagnosis.
  • A case study involving five RP patients showed that respiratory symptoms like cough and wheezing were misdiagnosed, but SPECT/CT imaging provided crucial information for earlier diagnosis.
  • Using SPECT/CT for early diagnosis of RP is effective, allowing for prompt treatment and improving the quality of life for affected individuals.
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  • Relapsing polychondritis (RP) is a rare autoimmune disease characterized by inflammation of cartilage, affecting areas like the ear, nose, and airway.
  • Paraneoplastic RP can develop in patients after being diagnosed and treated for specific cancers.
  • In a case study, a lung adenocarcinoma patient presented with paraneoplastic RP symptoms that improved after three cycles of chemotherapy, highlighting the role of FDG PET/CT in early diagnosis and treatment planning.
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Autoimmune inner ear disease associated with antiphospholipid antibodies.

Clin Exp Rheumatol

November 2024

Autoimmune and Systemic Diseases Unit, Department of Internal Medicine, Hospital Universitario Virgen de las Nieve, Granada, and MP03-Lupus, Instituto de Investigación Biosanitaria ibs. GRANADA, Granada, Spain.

Article Synopsis
  • Autoimmune diseases affect about 8% of the population in Western countries, but autoimmune vestibular disorders, like autoimmune inner ear disease (AIED), are often underdiagnosed due to the absence of specific tests for inner ear antigens.
  • A study was conducted on 55 patients with AIED to assess the presence of antiphospholipid antibodies (aPL), finding a 29% positivity rate with lupus anticoagulant being the most common.
  • Results showed that the main symptoms were bilateral hearing loss, with only 25% of patients experiencing audiometric improvement, highlighting the need for better recognition and treatment of aPL in AIED cases.
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[Aortitis].

Rev Med Interne

December 2024

Service de médecine interne, Nantes université, CHU de Nantes, 44000 Nantes, France.

Aortitis is a rare disease entity of unknown prevalence. Primary aortitis mainly affects the thoracic aorta. They are most often diagnosed on imaging by grade III 18-FDG uptake of the aortic wall on PET, or by circumferential thickening>2.

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