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REVIEW
Blanca E R G Bica, Alexandre Wagner S de Souza, Ivânio Alves Pereira
Relapsing polychondritis is a rare multisystem disease involving cartilaginous and proteoglycan-rich structures. The diagnosis of this disease is mainly suggested by the presence of flares of inflammation of the cartilage, particularly in the ears, nose or respiratory tract, and more rarely, in the presence of other manifestations. The spectrum of clinical presentations may vary from intermittent episodes of painful and often disfiguring auricular and nasal chondritis to an occasional organ or even life-threatening manifestations such as lower airway collapse...
April 16, 2024: Advances in Rheumatology
#2
JOURNAL ARTICLE
Antonio Vitale, Valeria Caggiano, Eduardo Martin-Nares, Micol Frassi, Lorenzo Dagna, Pravin Hissaria, Paolo Sfriso, José Hernández-Rodríguez, Guillermo Ruiz-Irastorza, Sara Monti, Abdurrahman Tufan, Matteo Piga, Henrique A Mayrink Giardini, Giuseppe Lopalco, Ombretta Viapiana, Amato De Paulis, Paola Triggianese, Rosetta Vitetta, Alejandra de-la-Torre, Alex Fonollosa, Federico Caroni, Jurgen Sota, Edoardo Conticini, Jessica Sbalchiero, Alessandra Renieri, Giulia Casamassima, Ewa Wiesik-Szewczyk, Derya Yildirim, Andrea Hinojosa-Azaola, Francesca Crisafulli, Franco Franceschini, Corrado Campochiaro, Alessandro Tomelleri, Alicia Callisto, Mark Beecher, Sara Bindoli, Chiara Baggio, Verónica Gómez-Caverzaschi, Laura Pelegrín, Adriana Soto-Peleteiro, Alessandra Milanesi, Ibrahim Vasi, Alberto Cauli, Isabele Parente de Brito Antonelli, Florenzo Iannone, Riccardo Bixio, Francesca Della Casa, Ilaria Mormile, Carmelo Gurnari, Alessia Fiorenza, Germán Mejia-Salgado, Perla Ayumi Kawakami-Campos, Gaafar Ragab, Francesco Ciccia, Piero Ruscitti, Monica Bocchia, Alberto Balistreri, Gian Marco Tosi, Bruno Frediani, Luca Cantarini, Claudia Fabiani
VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history...
March 18, 2024: Seminars in Arthritis and Rheumatism
#3
Abhijeet Mishra, Preetam Chappity, Sanjay K Behera, Mohnish Grover, Gaurav Gupta
Relapsing polychondritis is an autoimmune disorder causing inflammation of cartilaginous structures, sensory epithelium, and cardiovascular system. Hearing loss is a rare and dreadful complication of this pathology. We report a case of relapsing polychondritis in a 38-year-old female who developed gradually progressive bilateral profound hearing loss. She did not have any improvement with medical management. Cochlear implantation was performed to rehabilitate her hearing. As the scala tympani was obliterated, a scala vestibuli insertion was performed...
February 2024: Curēus
#4
JOURNAL ARTICLE
Muhammad Abumanhal, Igal Leibovitch, Michael Zisapel, Tali Eviatar, Yonatan Edel, Ran Ben Cnaan
BACKGROUND: VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a hematoinflammatory disease that typically affects adults. It results from a somatic mutation of the E1 ubiquitin conjugating enzyme encoded by the UBA1 gene. VEXAS is frequently accompanied by myelodysplastic syndrome (MDS). The purpose of this study is to describe the ocular and orbital manifestations of VEXAS patients in a case series in our medical centre. METHODS: A retrospective chart review was performed for all patients who were diagnosed with VEXAS syndrome in a tertiary medical centre over two years...
March 28, 2024: Eye
#5
Sejoon Jun, Selina Chang, Amritpal Kooner, Bliss Colao, Evelyn Greco
A 27-year-old male with no significant past medical history presented with recurrent swelling and pain on the right superior crus of the antihelix initially misdiagnosed as a skin infection. Despite adherence to antibiotic treatment, his condition showed no improvement, leading to further investigation. The patient's detailed clinical examination, family history devoid of autoimmune disorders, and persistent auricular inflammation prompted a reconsideration of the diagnosis. A subsequent biopsy that captured cartilage revealed auricular chondritis, perichondrial inflammation, degeneration of cartilage, and infiltration by inflammatory cells, all of which have been clinically associated with relapsing polychondritis (RP)...
February 2024: Curēus
#6
Salman Husein, Yuna Murayama, Andrew Koo, Michael Wakefield, Rosaria Buccoliero
The presented case highlights a rare instance of relapsing polychondritis (RP) manifesting as seronegative limbic encephalitis, an uncommon neurological complication. A 70-year-old female patient with a history of RP-related inflammation, along with neuropsychiatric symptoms, was diagnosed through multidisciplinary collaboration. Swift administration of steroid therapy, followed by azathioprine, led to remarkable physical and cognitive recovery. This case emphasises the importance of a multidisciplinary approach in diagnosing and treating complex autoimmune disorders with neurological manifestations...
November 2023: Clinical Medicine: Journal of the Royal College of Physicians of London
#7
Tejas Sinha, Jian Fu, Ashish Bains, Andrew Gangemi
The patient is a 49-year-old woman who had never used tobacco with a history of relapsing polychondritis and episcleritis. She sought treatment at our clinic for evaluation of multiple lung masses. She originally received a diagnosis by chest radiography performed to rule out sarcoidosis as the cause of episcleritis showing an abnormal findings. She had no contributory surgical, family, or social history. The autoimmune markers were notable for positive rheumatoid factor (153 IU/mL) and elevated erythrocyte sedimentation rate (97 mm/h) and C-reactive protein (65...
March 2024: Chest
#8
JOURNAL ARTICLE
Taisuke Nakagawa, Yasuaki Kotetsu, Katsumi Takizawa, Michihiro Yoshimi, Isamu Okamoto, Shohei Takata
We present the case of a 61-year-old man who developed Coronavirus disease 2019 (COVID-19) and died during treatment for relapsing polychondritis. The patient was intubated and treated with steroid pulse therapy, remdecivir, antibacterial agents, baricitinib, and tocilizumab. However, his respiratory condition worsened, and he died 108 days after disease onset. An autopsy revealed diffuse alveolar damage in the fibrotic phase in all lung lobes, diffuse pulmonary ossification, and cytomegalovirus-infected cells in the middle lobe of the right lung...
March 4, 2024: Internal Medicine
#9
REVIEW
Anca Cardoneanu, Ioana Irina Rezus, Alexandra Maria Burlui, Patricia Richter, Ioana Bratoiu, Ioana Ruxandra Mihai, Luana Andreea Macovei, Elena Rezus
Relapsing polychondritis is a chronic autoimmune inflammatory condition characterized by recurrent episodes of inflammation at the level of cartilaginous structures and tissues rich in proteoglycans. The pathogenesis of the disease is complex and still incompletely elucidated. The data support the important role of a particular genetic predisposition, with HLA-DR4 being considered an allele that confers a major risk of disease occurrence. Environmental factors, mechanical, chemical or infectious, act as triggers in the development of clinical manifestations, causing the degradation of proteins and the release of cryptic cartilage antigens...
February 13, 2024: International Journal of Molecular Sciences
#10
JOURNAL ARTICLE
Risa Hirata, Masahiko Nakamura, Shuto Yamada, Masaki Tago
No abstract text is available yet for this article.
February 20, 2024: BMJ Case Reports
#11
JOURNAL ARTICLE
T Didier, B Laure
No abstract text is available yet for this article.
February 15, 2024: Journal Français D'ophtalmologie
#12
JOURNAL ARTICLE
Shao-Ting Wang, Jinglan Wang, Xiaoxing Gao, Keqi Chen, Kai-Feng Xu, Xinlun Tian
BACKGROUND: Patients with relapsing polychondritis (RP) sometimes experience upper airway collapse or lower airway stenosis, and bronchoscopy may provide a valuable typical image to confirm the diagnosis. This study aimed to identify potential risk factors associated with severe adverse effects during bronchoscopy. METHODS: We performed a retrospective cohort study of 82 consecutive patients with RP hospitalized at Peking Union Medical College Hospital between January 1, 2012 and December 31, 2022...
February 9, 2024: Orphanet Journal of Rare Diseases
#13
JOURNAL ARTICLE
Vishnusai Karri, Abdullah Abou-Samra, Brian King, Adam Carlson, Arthi Venkat
PURPOSE: Ophthalmic manifestations of varying severity are often associated with systemic autoimmune conditions. Superior orbital fissure syndrome (SOFS) is a rare cranial neuropathy affecting nerves passing through the superior orbital fissure that causes a distinctive pattern of extraocular and pupillary findings. We report the coexistence of SOFS, relapsing polychondritis (RP) and Sjögren's syndrome (SS) in a 52-year-old female who presented with a past medical history of hypothyroidism, Raynaud's syndrome, and intermittent dry mouth and a 1-week history of worsening chemosis, proptosis, diplopia, and painful ophthalmoplegia...
February 7, 2024: Ocular Immunology and Inflammation
#14
Takanori Ohno, Ayako Shimada, Yuuko Terada, Toshitaka Ito, Kazuyuki Miyamoto
Relapsing polychondritis is a rare disease that causes progressive and recurrent destruction of cartilage in the auricles, eyes, nose, and airways. A 90-year-old man was brought to the emergency department with fever, low SpO2 , and effortful breathing. Arterial blood gas analysis showed that PaCO2 levels had accumulated to 120 mmHg. Although CT showed marked thickening of the bronchial wall from the central to the peripheral region, the cause was unknown. At the family's request, the patient was not placed on a ventilator, and treatment was started with steroids alone...
December 2023: Curēus
#15
JOURNAL ARTICLE
Kenji Funakoshi, Yasuhiro Kato, Masashi Narazaki, Atsushi Kumanogoh
No abstract text is available yet for this article.
January 24, 2024: BMJ Case Reports
#16
Shu Kojima, Satohiro Matsumoto, Yudai Koito, Takaya Miura, Masanari Sekine, Takeshi Uehara, Takeharu Asano, Yasuhiro Yamaguchi, Hirosato Mashima
A 51-year-old Japanese man was diagnosed with left-sided ulcerative colitis (UC) at age 41. He was treated with mesalazine and azathioprine and maintained remission. At age 51, the patient developed bloody stools, abdominal pain, scleritis, arthritis, cough, bloody sputum, and pericardial effusion. Considering that pericardial effusion is an atypical extraintestinal complication of UC, and the patient met the diagnostic criteria for relapsing polychondritis (RP), a diagnosis of RP complicating a relapse of UC was made...
2024: Biologics: Targets & Therapy
#17
JOURNAL ARTICLE
Greta Winter, Tara Löffelmann, Shaakira Chaya, Hannah Kaiser, Nils Kristian Prenzler, Athanasia Warnecke, Martin Wetzke, Thorsten Derlin, Diane Renz, Thomas Stueber, Florian Länger, Katharina Schütz, Nicolaus Schwerk
Relapsing polychondritis (RP) is a rare immune-mediated disease that primarily affects the cartilaginous structures of the ears, nose and airways. The clinical spectrum ranges from mild to severe disease characterized by progressive destruction of cartilage in the tracheobronchial tree leading to airway obstruction and acute respiratory failure. Early diagnosis is crucial to prevent irreversible airway damage and life-threatening complications. Due to its rarity and variability of symptoms, the diagnosis of RP is often delayed particularly in childhood...
January 15, 2024: Klinische Pädiatrie
#18
C Couselo-Rodríguez, M Á Viejo-Rodríguez, L Carballeira-Seoane, C Álvarez-Álvarez, E Cervates-Pérez, M Á García-González, Á Flórez
No abstract text is available yet for this article.
January 12, 2024: Actas Dermo-sifiliográficas
#19
JOURNAL ARTICLE
Pengcheng Cui, Daqing Zhao, Leping Liang, Pengfei Liu, Shuqin Li
OBJECTIVE: Tracheal transplantation could be a better option for patients with long segmental laryngotracheal stenosis or defects, but the need for immunosuppressants limits its widespread use due to the antigenicity of the tracheal epithelium. Chemically treated or cryopreserved nonviable tracheal allografts have no immunogenicity but lead to necrosis and stenosis in long-term outcomes. The present report describes the 5-year outcomes of de-epithelialized viable tracheal allotransplantation without immunosuppressants in a patient with severe laryngotracheal stenosis...
January 10, 2024: Annals of Otology, Rhinology, and Laryngology
#20
JOURNAL ARTICLE
Suying Duan, Haiyang Luo, Yunchao Wang, Dongbin Jiang, Jiajia Liu, Jiaqi Li, Honglin Zheng, Taiqi Zhao, Chenyang Liu, Hang Zhang, Chengyuan Mao, Lei Zhang, Yuming Xu
BACKGROUND: Commonly clinically diagnosed with relapsing polychondritis (RP), vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS) is a recently identified autoinflammatory disease caused by UBA1 somatic mutations. The low frequency and dynamic changes challenge the accurate detection of somatic mutations. The present study monitored these mutations in Chinese patients with RP. We included 44 patients with RP. Sanger sequencing of UBA1 was performed using genomic DNA from peripheral blood...
January 2, 2024: Orphanet Journal of Rare Diseases
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