1,011 results on '"Polychondritis, Relapsing"'
Search Results
2. Rare within rare. Necrotising scleritis and peripheral ulcerative keratitis: eye-threatening complications of relapsing polychondritis
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Damian, Laura, Pamfil, Cristina, Bucșa, Camelia, Nicula, Cristina, Mouthon, Luc, Amoura, Zahir, Cutolo, Maurizio, Burmester, Gerd R., Fonseca, João Eurico, Grapini, Lucica, Arnaud, Laurent, Rednic, Simona, and Repositório da Universidade de Lisboa
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Adult ,Male ,Relapsing polychondritis ,Immunology ,Peripheral ulcerative keratitis ,Middle Aged ,Infliximab ,Rheumatology ,Humans ,Immunology and Allergy ,Female ,Polychondritis, Relapsing ,Necrotising scleritis ,Child ,Corneal Ulcer ,Cyclophosphamide ,Rare connective tissue diseases ,Scleritis - Abstract
© Copyright Clinical and Experimental Rheumatology 2022, Objectives: Relapsing polychondritis (RP) evolves with variable and intermittent involvement of cartilage and proteoglycan-rich structures. Ocular manifestations are present in up to two-thirds of RP patients. Necrotising scleritis (NS) and peripheral ulcerative keratitis (PUK) may be inaugural and may lead to eye perforation and vision loss. We aimed to review NS and PUK in RP, in order to characterise them, to identify successful treatment options and unmet needs. Methods: A systematic review of the currently available evidence in PubMed, EMBASE and Scopus was performed according to PRISMA, including observational studies, single case reports and case series of NS/PUK in RP. Study design, number of patients, age, gender, treatment and outcome, were extracted. Two RP patients also provided their opinion. Results: Five case reports and two case series were eligible for inclusion. We identified 10 RP patients with eye-threatening complications (NS and/or PUK), 9 adults (2 males, 7 females, aged 35-72, median age 57.6 years) and one paediatric patient (F, 11 years). Apart from glucocorticoids, cyclophosphamide was effective in 4 patients; infliximab, high-dose immunoglobulins, dapsone, or cyclosporine were also successfully employed in a case each. Surgical repair was reported in 2 cases. Conclusions: Ocular inflammation is often bilateral and recurring in RP; NS/PUK are rare complications. All patients who develop NS/PUK should be specifically questioned for RP signs and symptoms. Early institution of immunosuppressive therapies is mandatory. Increasing awareness, physicians' and patients' education and a multidisciplinary approach may help improve the prognosis of these serious complications of RP.
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- 2022
3. A 60-year-old Man Presented with Relapsing Polychondritis with Haemophagocytic Lymphohistiocytosis
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A K, Ahmedullah, M M, Hasan, M Z, Amin, M N, Hasan, M A, Rahman, and M N, Islam
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Male ,Bangladesh ,Prednisolone ,Arthritis ,Humans ,Polychondritis, Relapsing ,Middle Aged ,Lymphohistiocytosis, Hemophagocytic - Abstract
Relapsing polychondritis is a rare autoimmune disorder of unknown etiology, which can affect multiple organs. It usually presents with involvement of elastic cartilage of ear and nose and involvement of other organs like kidney and central nervous system. Here, we report a case of 60-year-old man, who initially presented with fever, polyarthritis, erythema nodosum and painful swelling and redness of both external ears admitted in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh on 13th December 2019. Two days after hospitalization, he developed sudden onset of proptosis with ophthalmoplegia. After evaluation, he was diagnosed as relapsing polychondritis with haemophagocytic lymphohistiocytosis and was treated with high dose prednisolone. His clinical condition and laboratory parameters significantly improved after treating with prednisolone during follow up.
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- 2023
4. Successful treatment of relapsing polychondritis with circumferential bronchial wall thickening including the tracheomembranous area with tumor necrosis factor-α inhibitor
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Makiko Maekawa, Motoki Yoshimura, Masako Kadowaki, Miki Nakano, Atsushi Moriwaki, Hitoshi Ueda, and Shigeru Yoshizawa
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Male ,Methotrexate ,Rheumatology ,Tumor Necrosis Factor-alpha ,Prednisolone ,Adalimumab ,Humans ,Immunologic Factors ,Polychondritis, Relapsing ,Middle Aged - Abstract
This is a case of a 55-year-old man who presented with cough and anterior chest pain. Tracheal biopsy confirmed the diagnosis of relapsing polychondritis (RP). Although the patient had circumferential bronchial wall thickening extending to the tracheomembranous area and was positive foRPR3-ANCA, he did not meet the diagnostic criteria for granulomatosis with polyangiitis. The patient was refractory to prednisolone + methotrexate + azathioprine and responded to adalimumab, a biologic tumor necrosis factor-α inhibitor effective in RP refractory cases. Herein, we report a rare case of RP with circumferential bronchial wall thickening extending to the tracheomembranous area.
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- 2022
5. Presentation, Diagnosis, and Management of Subglottic and Tracheal Stenosis During Systemic Inflammatory Diseases
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François Gonin, Alexis Régent, Luc Mouthon, Véronique Le Guern, Candice Lacroix, Emmanuel Martinod, André Coste, Paul Legendre, Benjamin Terrier, Xavier Puéchal, Pascal Cohen, J. Catano, Jérémie Dion, Yurdagul Uzunhan, Romain Paule, Christine Lorut, and Sophie Périé
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Adult ,Male ,Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Sarcoidosis ,Subglottic stenosis ,Critical Care and Intensive Care Medicine ,Crohn Disease ,stomatognathic system ,Interquartile range ,medicine ,Humans ,Polychondritis, Relapsing ,Glucocorticoids ,Relapsing polychondritis ,Retrospective Studies ,Anti-neutrophil cytoplasmic antibody ,Laryngoscopy ,Skin Diseases, Vesiculobullous ,business.industry ,Granulomatosis with Polyangiitis ,Calcinosis ,Laryngostenosis ,Amyloidosis ,Middle Aged ,medicine.disease ,Tracheal Stenosis ,Stenosis ,Methotrexate ,Female ,Radiology ,Tomography, X-Ray Computed ,Cardiology and Cardiovascular Medicine ,Granulomatosis with polyangiitis ,business ,Immunosuppressive Agents - Abstract
Background Subglottic stenosis (SGS) and tracheal stenosis (TS) are characterized by a narrowing of the airways. The goal of this study was to describe the characteristics and prognosis of nontraumatic and nontumoral SGS or TS. Research Question What are the inflammatory etiologies of SGS and TS, and what are their characteristics and prognosis? Study Design and Methods This multicenter, observational retrospective study was performed in patients with SGS or TS that was neither traumatic nor tumoral. Results Eighty-one patients were included, 33 (41%) with granulomatosis with polyangiitis (GPA) and 21 (26%) with relapsing polychondritis (RP). GPA-related stenoses exhibited circumferential subglottic narrowing in 85% of cases, without calcifications. In contrast, RP-related stenoses displayed anterior involvement in 76%, in a longer distance from vocal cords (4 cm), with calcifications in 62%, and extension to bronchi in 86%. Other diagnoses included bullous dermatoses (n = 3), amyloidosis (n = 3), sarcoidosis (n = 2), and Crohn’s disease (n = 2); the remaining stenoses (n = 15) were idiopathic. SGS/TS was the initial manifestation of the disease in 66% of cases, with a median interval from stenosis to disease diagnosis of 12 months (interquartile range, 0-48 months). Despite the use of glucocorticoids in 80%, combined with methotrexate in 49%, endoscopic procedures were required in 68% of patients. Relapses of stenoses occurred in 76% without any difference between causes (82% in GPA, 67% in RP, and 75% in idiopathic SGS/TS). Three patients died due to the stenosis, two of RP and one of GPA. Interpretation These data show that GPA and RP are the two main inflammatory diseases presenting with SGS/TS. GPA-related stenoses are mostly subglottic and circumferential, whereas RP-related stenoses are mostly tracheal, anterior, and calcified with a frequent extension to bronchi. Relapses of stenoses are common, and relapse rates do not differ between causes. Diagnosis and management of SGS/TS require a multidisciplinary approach.
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- 2022
6. Ultrasonography of auricular cartilage is a potential tool for diagnosing relapsing polychondritis and monitoring disease activity
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Mitsuharu Yoshida, Yoshinori Taniguchi, Takeshi Yoshida, Hirofumi Nishikawa, and Yoshio Terada
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Male ,Rheumatology ,Disease Progression ,Humans ,Female ,Polychondritis, Relapsing ,Ear Cartilage ,Middle Aged ,Retrospective Studies ,Ultrasonography - Abstract
To assess the clinical utility of ultrasonography in the diagnosis and monitoring of disease activity in relapsing polychondritis (RP).Auricular and nasal chondritis of 6 patients with RP were assessed by ultrasonography before treatment initiation. Changes in the ultrasonographic and clinical findings and serum inflammatory markers were longitudinally assessed. Ultrasonography was also performed in 6 patients with repeat ear trauma, 6 patients with auricular cellulitis and 6 healthy controls for comparison among groups.In all cases of RP, ultrasonographic findings before treatment revealed low-echoic swollen auricular and nasal cartilage and perichondral soft-tissue with increased power Doppler signals (PDS) corresponding to biopsy findings. After 2-month treatment with prednisolone (PSL) combined with methotrexate, clinical and serum inflammatory markers were completely resolved. Although swollen perichondral soft-tissue, cartilage and PDS on auricular ultrasonography were also significantly improved, PDS remained in 2 of 6 cases, which showed flare early after tapering PSL. Finally, ultrasonographic findings of RP were substantially differentiated between patients with repeat trauma and cellulitis and healthy controls based on the thickness of soft tissue around the cartilage, PDS and subperichondral serous effusion.Assessment of RP lesions by ultrasonography is useful for the evaluation of cartilaginous lesions and monitoring of disease activity, especially when considering the treatment response and the timing of drug tapering.
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- 2021
7. Relapsing polychondritis or relapsing polychondritis-like symptoms associated with IgG4-RD. Case reports and review of the literature
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Sergio Paira, María Marcela Schmid, Josefina Gallino Yanzi, and Jesica Gallo
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Autoimmune disease ,Pathology ,medicine.medical_specialty ,Coronavirus disease 2019 (COVID-19) ,business.industry ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,fungi ,Disease ,medicine.disease ,Autoimmune Diseases ,Immunoglobulin G ,parasitic diseases ,medicine ,Humans ,Chondritis ,Immunoglobulin G4-Related Disease ,Polychondritis, Relapsing ,business ,Tracheal cartilages ,Infiltration (medical) ,Relapsing polychondritis - Abstract
Relapsing polychondritis (RP) is a rare systemic disorder characterised by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterised by the infiltration of IgG4-bearing plasma cells into systemic organs. However, 25–35% of patients with RP have a concurrent autoimmune disease. The coexistence of RP and IgG4 is rare considering that, to the best of our knowledge, there are only four previous reports of RP or RP-like symptoms associated with IgG4-RD. We herein report two cases which could be RP or RP-like symptoms associated with IgG4-RD.
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- 2021
8. Encefalopatía subaguda en una paciente con policondritis recidivante. Caso clínico
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Walter Feuerhake, Constanza Vera, J.L. Castillo, José Manuel Matamala, Aaron Vidal, and Oscar Neira
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Brain Diseases ,Pathology ,medicine.medical_specialty ,Cyclophosphamide ,Leukocytosis ,business.industry ,Encephalopathy ,Central nervous system ,Inflammation ,General Medicine ,medicine.disease ,Cerebrospinal fluid ,medicine.anatomical_structure ,medicine ,Chondritis ,Polychondritis, Relapsing ,medicine.symptom ,Pleocytosis ,business ,Relapsing polychondritis ,medicine.drug - Abstract
Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.
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- 2021
9. Small cell lung cancer with relapsing polychondritis: A report of one case and the review of literature
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Yuan Liu, Ning Wang, Jian Xu, Ying Bi, Xue Han, Meng Dai, and Chunfang Liu
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Pharmacology ,Lung Neoplasms ,Immunology ,Immunology and Allergy ,Humans ,Bronchi ,Female ,Polychondritis, Relapsing ,Middle Aged ,Lung ,Small Cell Lung Carcinoma - Abstract
The present study reports the clinical data of a patient with small cell lung cancer who developed relapsing polychondritis. We report a case of a 57-year-old female presented with cough, expectoration, and fever. A Computed Tomography (CT) scan performed at the hospital revealed diffuse thickening of bronchial walls in both lungs. Bronchoscopy revealed that the tracheal mucosa was thickened, narrowed, and collapsed, and the bronchoscope could pass through. The bronchial mucosa on both sides was thickened and edematous, the surface was rough, each bronchus was narrow, and the intervertebral ridges were widened. Needle biopsy: considering small cell carcinoma in combination with immunohistochemical results. Her symptom was not improved after anti-infective therapy. The left auricle was red and swollen, the auricle collapsed, and the left eye had subconjunctival hemorrhage during her hospitalization without obvious cause. After multidisciplinary consultation, pulmonary small cell lung cancer cT0N2Mx rumen lymph node metastasis and RP were considered. Treatment: Prednisone, orally for RP. Chemotherapy combined with radiotherapy was given for small cell lung cancer. The chemotherapy regimen was carboplatin combined with etoposide. The patient has already been followed for 1 year after receiving chemoradiotherapy; the condition of the patient is stable at present. Based on the case of our patient, for cases of RP with symptoms such as auricle chondritis, ocular inflammatory disease, and nasal chondritis, we should pay great attention to whether the case is caused by lung cancer with relapsing polychondritis. Because of the rarity of the disease, the clinician should improve the recognition of the disease in order to strive for early diagnosis and therapy.
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- 2022
10. Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS syndrome) with prominent supraglottic larynx involvement: a case-based review
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Camila Andrea Guerrero-Bermúdez, Andrés Felipe Cardona-Cardona, Edwin Jesús Ariza-Parra, Juan Ignacio Arostegui, Anna Mensa-Vilaro, Jordi Yague, Gloria Vásquez, and Carlos Horacio Muñoz-Vahos
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Male ,Rheumatology ,Myelodysplastic Syndromes ,Giant Cell Arteritis ,Vacuoles ,Humans ,General Medicine ,Polychondritis, Relapsing ,Larynx ,Sweet Syndrome ,Immunosuppressive Agents ,Aged ,Polyarteritis Nodosa - Abstract
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS syndrome) is a recently described genetic disorder that gathers autoinflammatory symptoms and myeloid dysplasia. The first description was reported in 2020, and subsequently, a growing number of cases have been described worldwide. Herein, we describe a case of a 72-year-old male patient with VEXAS syndrome with p.Met41Val mutation of the UBA1 gene, prominent supraglottic larynx involvement, and costochondritis. To our knowledge, this is the first report of VEXAS syndrome in Colombia and South America. This disease could present features of relapsing polychondritis, polyarteritis nodosa, giant cell arteritis, and Sweet syndrome, associated with hematologic involvement, including cytopenias, myelodysplastic syndrome, or thromboembolic disease. Supraglottic larynx chondritis and costochondritis are atypical manifestations. These features were proposed previously to differentiate relapsing polychondritis from VEXAS syndrome but are not entirely reliable like in the case described. A diagnosis of VEXAS should be considered in male patients with incomplete or complete features of the previously described conditions, refractory to treatment, requiring high-dose glucocorticoids, and associated progressive hematologic abnormalities. Key Points • VEXAS syndrome is a recently described genetic (somatic mutations in UBA1 gene) disorder that gathers autoinflammatory and hematologic manifestations. • VEXAS syndrome should be considered in male patients with incomplete or complete features of relapsing polychondritis, polyarteritis nodosa, giant cell arteritis, and Sweet syndrome, refractory to treatment, associated with hematologic involvement, including cytopenias, myelodysplastic syndrome, or thromboembolic disease. • Glucocorticoids ameliorate symptoms effectively. However, other treatment options are limited due to a lack of evidence. Traditional immunosuppressants and biological therapy have been used empirically with limited efficacy and a transient effect. Bone marrow transplant offers a curative approach, but it has high morbidity and mortality.
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- 2022
11. A 'Leopard Man' Aspect on 18F-FDG PET/CT Revealing a VEXAS Syndrome
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Alexandre, Fagart, Thomas, Quemeneur, Guillaume, Collet, Franck, Demailly, and Clementine, Rousselin
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Male ,Adult ,Fluorodeoxyglucose F18 ,Positron Emission Tomography Computed Tomography ,Positron-Emission Tomography ,Giant Cell Arteritis ,Mutation ,Humans ,Radiology, Nuclear Medicine and imaging ,Polychondritis, Relapsing ,General Medicine ,Aged - Abstract
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset autoinflammatory disease caused by somatic UBA1 mutations first described in 2020. Most of these patients met clinical criteria for an inflammatory syndrome (relapsing polychondritis, Sweet syndrome, polyarteritis nodosa, or giant-cell arteritis) or a hematologic condition (myelodysplastic syndrome or multiple myeloma) or both. We described here an FDG PET/CT "leopard man" appearance, with abnormal marrow recruitment the findings, in a 70-year-old man diagnosed with a VEXAS syndrome.
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- 2022
12. Relapsing Polychondritis as a Cause of Sudden and Unexpected Death With Central Nervous System Involvement
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Alexander Lokken and Amber Wang
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Adult ,Central Nervous System ,Male ,Death, Sudden ,Meningoencephalitis ,Humans ,Polychondritis, Relapsing ,Pathology and Forensic Medicine - Abstract
Relapsing polychondritis (RP) is a rare inflammatory disease process that affects cartilaginous tissues throughout the body. Although the pathogenesis remains unknown, RP is thought to be an autoimmune disorder in which host immune cells are conditioned to attack the body's cartilage, such as the ears, nose, eyes, joints, and airways, resulting in inflammation and destruction of otherwise healthy tissues. In rare and unusual cases, neurological involvement has been described.We report a case of a 36-year-old man with a medical history of asthma and suspected seronegative rheumatoid arthritis/RP and panuveitis who was found deceased in his residence. Postmortem examination revealed cartilaginous destruction of the external ear and large airways and meningoencephalitis involving the left medial temporal lobe without an underlying infectious cause.Progressive destruction of airway tissue and increased susceptibility to pulmonary infection is the most common cause of death in RP. Central nervous system involvement is exceedingly rare, presenting with highly variable clinical and pathological manifestations. A review of RP and systemic manifestations will follow. Accurate recognition of this multisystem autoimmune disease as a cause of sudden and unexpected death is critical for proper death certification and to broaden our understanding of this disease.
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- 2022
13. [Recommendations of diagnosis and treatment of relapsing polychondritis in China]
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J, Xu, D D, Wang, G X, Shi, M Y J, Baima, X, Zhang, and Yan, Zhao
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China ,Humans ,Immunotherapy ,Polychondritis, Relapsing ,Prognosis - Abstract
Relapsing polychondritis is an immune mediated systemic inflammatory disease, involving the cartilaginous and proteoglycan rich structures. The characteristic manifestations were inflammation and deformity of ear and nasal cartilage. Here, Chinese Rheumatology Association summarized manifestations, diagnosis and disease activity index evaluation of relapsing polychondritis, standardized treatment regimens, to improve disease prognosis.复发性多软骨炎(RP)是一种免疫介导的全身炎症性疾病,主要累及软骨及富含蛋白聚糖成分的组织,特征性表现为耳和鼻软骨炎症、畸形。为提高临床医生对RP的认识和诊疗水平,中华医学会风湿病学分会在借鉴目前国内外经验和指南的基础上,制定了本规范,总结了RP的临床表现、诊断及疾病活动评估,旨在规范RP临床治疗方案,改善RP患者预后。.
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- 2022
14. B-cell lymphoma mimicking relapsing polychondritis
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Yachar Dawudi, Khadija Benali, Maxime Beydon, Muriel Hourseau, Karim Sacre, and Thomas Papo
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Diagnosis, Differential ,Lymphoma, B-Cell ,Humans ,Hematology ,Polychondritis, Relapsing - Published
- 2022
15. Relapsing polychondritis revealed by
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Ming, Ni, Qiang, Xie, Xingxing, Zhu, and Weifu, Lv
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Heterocyclic Compounds, 1-Ring ,Fluorodeoxyglucose F18 ,Positron Emission Tomography Computed Tomography ,Quinolines ,Humans ,Polychondritis, Relapsing - Published
- 2022
16. Relapsing Polychondritis in a Patient with Ankylosing Spondylitis under Two Different Tumor Necrosis Factor Alpha Inhibitors Treatment
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O C, İçaçan, M, Yalçın Mutlu, F, Yıldırım, and C, Bes
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Inflammation ,Tumor Necrosis Factor-alpha ,Humans ,Spondylitis, Ankylosing ,Tumor Necrosis Factor Inhibitors ,Polychondritis, Relapsing - Abstract
Relapsing polychondritis (RP) is a rare autoimmune disease char-acterized by multi-systemic involvement characterized by recurrent and progressive inflammation of the cartilaginous tissue. Auricular inflammation is a characteristic finding of RP. Anti-tumor necrosis fac-tor alpha (anti-TNF) is a highly effective drug used in the treatment of inflammatory arthritis. There are several case reports showing potential relationship between the RP development and anti-TNF treatment. Here, we present a case of RP in a patient with ankylosing spondylitis under the two different tumor necrosis factor alpha inhibitors therapy.
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- 2022
17. Methylprednisolone pulse therapy for relapsing polychondritis (RP) combined with heart block: myth or reality?
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M, Tang, Q-P, Xie, K, Zhu, and X-L, Fu
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Heart Block ,Hormone Replacement Therapy ,Humans ,Arrhythmias, Cardiac ,Polychondritis, Relapsing ,Methylprednisolone - Published
- 2022
18. Correlation between Computed Tomographic Analysis and Pulmonary Function Measurements in Patients with Relapsing Polychondritis
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Takeo Inoue, Hiroki Nishine, Tsuneo Yamashiro, Hiroshi Handa, Masamichi Mineshita, and Hajime Tsuruoka
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Adult ,Male ,Pulmonary and Respiratory Medicine ,Spirometry ,Pulmonary function testing ,Oscillometry ,medicine ,Humans ,Polychondritis, Relapsing ,Respiratory system ,Relapsing polychondritis ,Aged ,Aged, 80 and over ,medicine.diagnostic_test ,business.industry ,Middle Aged ,Airway obstruction ,medicine.disease ,Respiratory Function Tests ,Trachea ,Stenosis ,Impulse Oscillometry ,Female ,Tomography, X-Ray Computed ,Airway ,business ,Nuclear medicine ,Software - Abstract
Background: Relapsing polychondritis (RP) is a rare systemic disease of unknown origin, with cartilaginous involvement in multiple organs. Airway involvement is the most important prognostic factor in RP. Objectives: Spirometric measurements and minimum tracheal cross-sectional area (mtCSA) have been reported as useful to assess the degree of airway stenosis. Because the length and severity of tracheal involvement in RP can vary, mtCSA might not provide enough information to assess tracheal abnormalities. We introduced tracheal volume (TrV) as a new method to evaluate correlations between chest computed tomography (CT) measurements and pulmonary function tests, including impulse oscillometry (IOS). Method: We analyzed chest CT images, spirometry, and IOS collected at our institution from April 2004 to March 2019. We calculated correlations between chest CT measurements using software (TrV, TrV/tracheal length [TrV/TL], and mtCSA) and pulmonary function parameters. Results: Twenty-five of 73 clinically diagnosed patients with RP were included. Spirometric findings showed moderate airway obstruction. Peak flow (PEF) was strongly correlated with mtCSA, TrV, and TrV/TL (ρ = 0.74, p < 0.001). FEV1 was significantly correlated with mtCSA (ρ = 0.56, p = 0.004), TrV (ρ = 0.52, p = 0.007), and TrV/TL (ρ = 0.53, p = 0.006). Whereas respiratory resistance at 5 Hz (R5) and 20 Hz (R20) and resonant frequencies (RFs) were significantly correlated with TrV (ρ = −0.46, p = 0.021; ρ = −0.46, p = 0.046; and ρ = −0.42, p = 0.037, respectively), IOS parameters and mtCSA were not. Conclusions: In patients with RP, TrV and mtCSA were strongly correlated with spirometric measurements. Respiratory resistances assessed by IOS correlated only with TrV. This suggests TrV assessment reflects pulmonary function in patients with RP more appropriately than mtCSA.
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- 2021
19. Sequential Involvement of Oculomotor Nerve and Optic Nerve Sheath in Relapsing Polychondritis
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Ken Fukuda, Hirotaka Yamamoto, Chie Sotozono, Toshihide Ikeda, Takeshi Yoshida, Masatsugu Hashida, and Kimiaki Urabe
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Optic nerve sheath ,business.industry ,Oculomotor nerve ,Optic Nerve ,Anatomy ,medicine.disease ,Diagnosis, Differential ,Ophthalmology ,Oculomotor Nerve ,Humans ,Medicine ,Polychondritis, Relapsing ,Neurology (clinical) ,Tomography, X-Ray Computed ,business ,Relapsing polychondritis - Published
- 2021
20. Labyrinthine Hemorrhage in Relapsing Polychondritis
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Arnaud Attyé, Gabor Michael Halmagyi, and Griff Richards
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medicine.medical_specialty ,business.industry ,Hemorrhage ,medicine.disease ,Dermatology ,Sensory Systems ,Otorhinolaryngology ,Ear, Inner ,Humans ,Medicine ,Polychondritis, Relapsing ,Neurology (clinical) ,business ,Relapsing polychondritis - Published
- 2020
21. Unusual cause of bloody stool with earache and saddle-nose deformity
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Xiaolei Liu, Yongquan Shi, Zhenzhen Liu, Min Chen, and Jie Liang
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Adult ,0301 basic medicine ,medicine.medical_specialty ,immunoregulation ,IBD ,Colonoscopy ,Inflammation ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Deformity ,Humans ,Polychondritis, Relapsing ,Anti-neutrophil cytoplasmic antibody ,medicine.diagnostic_test ,biology ,business.industry ,C-reactive protein ,Ear Deformities, Acquired ,Nose Deformities, Acquired ,Sigmoid colon ,medicine.disease ,Bloody ,030104 developmental biology ,medicine.anatomical_structure ,IBD clinical ,Earache ,biology.protein ,Colitis, Ulcerative ,Female ,030211 gastroenterology & hepatology ,Editor’s quiz: GI snapshot ,medicine.symptom ,Gastrointestinal Hemorrhage ,business ,Vasculitis - Abstract
A 41-year-old female patient was admitted to hospital with bloody stool and diarrhoea for 2 months, accompanied with fever, left earache, obvious saddle-nose deformity and red eyes for 2 weeks. She had history of repeated multiple sore joints for 6 months and hearing impairment in the left ear for 4 months. Laboratory tests showed elevated white cell count (WBC) of 15.57×10ˆ9/L, neutrophil of 76.8% and C reactive protein (CRP) of 95.4 mg/L. Haemoglobin was decreased at 94 g/L. Antineutrophil cytoplasmic antibody was negative. Colonoscopy showed diffuse inflammation and ulcers in the left-sided colon (figure 1). Histological findings from sigmoid colon showed chronic inflammation without vasculitis appearance. CT enterography revealed left-sided colon inflammation (figure 2). MRI of …
- Published
- 2020
22. A man in his sixties with chondritis and bone marrow failure
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Øyvind, Midtvedt, Asbjørg, Stray-Pedersen, Helena, Andersson, Ragnar, Gunnarsson, Kristian, Tveten, Maryan Mohamed, Ali, and Geir E, Tjønnfjord
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Inflammation ,Male ,Pancytopenia ,Myelodysplastic Syndromes ,Giant Cell Arteritis ,Humans ,Polychondritis, Relapsing ,Ubiquitin-Activating Enzymes ,Bone Marrow Failure Disorders ,Aged - Abstract
VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic syndrome) first described in 2020, is caused by a limited repertoire of somatic mutations in UBA1, a gene involved in the initiation of ubiquitination. Ubiquitination, adding an ubiquitin protein to a substrate protein, can have various effects on the substrate. Disruption of UBA1 function results in diverse clinical manifestations, mimicking a variety of disorders.A man in his sixties presented with fever, chest pain, fatigue, pulmonary infiltrates and elevated acute phase reactants. Initially he was thought to have extra-cranial giant cell arteritis. When he developed ear and nose chondritis, a revised diagnosis of relapsing polychondritis was made. Subsequently he developed macrocytic anaemia and thrombocytopenia. His condition remained resistant to medical therapy and he died eight years after disease onset. Analysis of stored DNA revealed a somatic mutation in UBA1 confirming the diagnosis of VEXAS syndrome.VEXAS syndrome is a newly identified inflammatory disorder due to an acquired mutation in haematopoietic bone marrow cells in older men. The syndrome may be misdiagnosed as treatment-refractory relapsing polychondritis, polyarteritis nodosa, Sweet syndrome or giant cell arteritis. We describe the first individual with molecularly confirmed VEXAS syndrome in Norway.
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- 2022
23. Biologic therapy in relapsing polychondritis: navigating between options
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Roberto Padoan, Debora Campaniello, Luca Iorio, Andrea Doria, and Franco Schiavon
- Subjects
Pharmacology ,Biological Products ,anti-TNFα ,Tumor Necrosis Factor-alpha ,Clinical Biochemistry ,Abatacept ,Biological Therapy ,tocilizumab ,Biological Factors ,Interleukin 1 Receptor Antagonist Protein ,anakinra ,biologic therapy ,biological agents ,infliximab ,relapsing polychondritis ,rituximab ,Drug Discovery ,Humans ,Polychondritis, Relapsing ,Rituximab ,Glucocorticoids ,Immunosuppressive Agents - Abstract
Relapsing polychondritis (RP) is a rare systemic inflammatory disease of unknown etiology, primarily affecting cartilaginous tissue and proteoglycan-rich structures. Clinical manifestations vary from mild symptoms to occasional organ or life-threatening complications. Treatment can be challenging and is mostly based on experience or case reports/series.There is growing literature investigating the role of biologics in the management of RP. TNFα antagonists, abatacept, tocilizumab, rituximab, anakinra and tofacitinib have been prescribed in several RP patients, mainly as second-line treatment, after conventional immunosuppressive agents' failure.Glucocorticoids represent the gold standard treatment of RP. Conventional immunosuppressants should be administered in refractory patients or when a glucocorticoid-sparing effect is needed. Biologic therapy should be used after failure of conventional treatments or in severe manifestations. TNFα inhibitors are the most prescribed biologic agent, with partial or complete response in several cases, but loss of efficacy may occur over time. Infliximab and adalimumab should be preferred among TNFα antagonists. Abatacept and tocilizumab proved to be effective as second-line biologic agents, but infections are reported with the former. Data on anakinra and rituximab are controversial, therefore they are not recommended as first-line biologic drugs. The use of JAK inhibitors is still anecdotal.
- Published
- 2022
24. Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients
- Author
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Mohamed-Yacine Khitri, Alexis F Guedon, Sophie Georgin-Lavialle, Benjamin Terrier, David Saadoun, Julie Seguier, Maelle le Besnerais, Claire De Moreuil, Guillaume Denis, Mathieu Gerfaud-Valentin, Jean Sebastien Allain, Alexandre Maria, Laurence Bouillet, Vincent Grobost, Joris Galland, Olivier Kosmider, Anael Dumont, Mathilde Devaux, Benjamin Subran, Jean Schmidt, Paola Marianetti-Guingel, Sylvain Audia, Sylvain Palat, Marielle Roux-Sauvat, Vincent Jachiet, Pierre Hirsch, Olivier Fain, and Arsène Mekinian
- Subjects
Adult ,Cohort Studies ,Male ,Rheumatology ,Myelodysplastic Syndromes ,Immunology ,Immunology and Allergy ,Humans ,Female ,Polychondritis, Relapsing ,Glucocorticoids ,Retrospective Studies - Abstract
ObjectiveA new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic). We aimed to compare the clinical characteristics, the laboratory features and the outcomes between idiopathic-relapsing polychondritis (I-RP) and VEXAS-relapsing polychondritis (VEXAS-RP).MethodsPatients from French retrospective multicentre cohort of RP were separated into two groups: a VEXAS-RP and an I-RP.ResultsCompared with patients with I-RP (n=40), patients with VEXAS-RP (n=55) were men (96% vs 30%, pConclusionWe report the largest cohort of VEXAS-RP, characterised by high prevalence of male sex, fever, skin lesion, ocular involvement, pulmonary infiltration, heart involvement, older age and MDS association.
- Published
- 2022
25. Comparison of Relapsing Polychondritis Patients with and Without Respiratory Involvement Based on Chest Computed Tomography: A Retrospective Cohort Study
- Author
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Dong, Wang, Lujia, Guan, Xin, Dong, Xiaofan, Zhu, and Zhaohui, Tong
- Subjects
Cohort Studies ,Trachea ,Pulmonary and Respiratory Medicine ,Humans ,Chest computed tomography ,Respiratory involvement ,Relapsing polychondritis ,Polychondritis, Relapsing ,Tomography, X-Ray Computed ,Retrospective Studies - Abstract
Background Relapsing polychondritis (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 and a non-respiratory involvement subgroup according to chest computed tomography. Method We performed a retrospective cohort study collecting RP patients hospitalized at the Beijing Chao-Yang Hospital between January 2012 and August 2021. Results Respiratory involvement affected 59.7% of patients in our cohort. The incidence of costochondritis was more common in RP patients with respiratory involvement (p = 0.03); the incidence of inflammatory eye disease (p = 0.001) and auricular chondritis (p = 0.001) was less frequent in RP respiratory involvement patients.. Compared with the non-respiratory involvement subgroup the incidence of pulmonary infection marginally increased in the respiratory involvement subgroup (p = 0.06). Inflammatory indexes except for C-reactive protein to albumin ratio (CAR) were significantly higher in the respiratory involvement subgroup; analysis revealed no significant relationship between inflammatory indexes and pulmonary infection. Conclusion RP patients with respiratory involvement had a greater incidence of costochondritis and pulmonary infectionand lesser incidence of inflammatory eye diseases and auricular chondritis compared to non-respiratory involvement. Increasing inflammatory indexes suggests that patients with respiratory involvement had a higher disease activity index of RP. The difference in probability of survival was insignificant between subgroups.
- Published
- 2022
26. Nationwide cross-sectional survey of patients with relapsing polychondritis in 2019 demonstrates reduction of airway involvement compared with that in 2009
- Author
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Jun Shimizu, Yoshihisa Yamano, Kimito Kawahata, and Noboru Suzuki
- Subjects
Adult ,Male ,Multidisciplinary ,Science ,Respiratory Tract Diseases ,Diseases ,Middle Aged ,Article ,Infliximab ,Cross-Sectional Studies ,Methotrexate ,Rheumatology ,Japan ,Surveys and Questionnaires ,Azathioprine ,Medicine ,Humans ,Female ,Polychondritis, Relapsing ,Signs and symptoms ,Cyclophosphamide ,Immunosuppressive Agents ,Retrospective Studies - Abstract
We conducted retrospective cohort studies of patients with relapsing polychondritis (RP) twice in 2009 and 2019, using a physician questionnaire. We compared the patients’ clinical statuses between the years. Age and gender were comparable between the two surveys. Mean disease duration was longer in 2019 survey (8.3 years) than that in 2009 survey (4.8 years, P
- Published
- 2022
27. A 68-Year-Old Man With Skin Rash and a Pleural Effusion
- Author
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Oliver H. Chang, Tyler J. Albert, April Schachtel, Heidi Hermes Shantz, Sarah Bastawrous, Marat Gadzhiev, Gustavo Matute-Bello, and Robin H. Stiller
- Subjects
Male ,Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Triamcinolone acetonide ,Pleural effusion ,Critical Care and Intensive Care Medicine ,Azithromycin ,03 medical and health sciences ,0302 clinical medicine ,Prednisone ,Humans ,Medicine ,Polychondritis, Relapsing ,030212 general & internal medicine ,Relapsing polychondritis ,Aged ,business.industry ,Exanthema ,medicine.disease ,Sweet Syndrome ,Dermatology ,Rash ,Infliximab ,Pleural Effusion ,Pneumonia ,030228 respiratory system ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,medicine.drug - Abstract
Case Presentation A 68-year-old man developed an erythematous, papular, pruritic rash on his right thigh 1 month prior to presentation. It subsequently spread to his other extremities and trunk. He also endorsed fevers of > 38.3°C, night sweats, fatigue, shortness of breath, and a dry cough. He was prescribed triamcinolone 0.1% cream for his rash and azithromycin for presumed community-acquired pneumonia, with no improvement in symptoms. He had a history of relapsing polychondritis for which he was prescribed infliximab and low-dose prednisone. He had never smoked tobacco, did not use alcohol or illicit substances, and had no significant travel history.
- Published
- 2020
28. Relapsing Polychondritis with a Cobble-stone Appearance of the Tracheal Mucosa, Preceded by Posterior Reversible Encephalopathy Syndrome
- Author
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Takahide Ikeda, Hiroyuki Morita, Motochika Asano, Yuichi Hayashi, Yoshihiko Kitada, K. Taguchi, and Kazuo Kajita
- Subjects
Adult ,posterior reversible encephalopathy syndrome ,Pathology ,medicine.medical_specialty ,Case Report ,Tracheal mucosa ,Bronchoscopy ,Autoimmune vasculitis ,relapsing polychondritis ,Internal Medicine ,medicine ,Humans ,Polychondritis, Relapsing ,tracheal stenosis ,Relapsing polychondritis ,Mucous Membrane ,medicine.diagnostic_test ,business.industry ,Brain ,Magnetic resonance imaging ,Posterior reversible encephalopathy syndrome ,General Medicine ,medicine.disease ,Magnetic Resonance Imaging ,Tracheal Stenosis ,Trachea ,Steroid pulse ,Female ,Posterior Leukoencephalopathy Syndrome ,Tomography, X-Ray Computed ,business - Abstract
A 25-year-old woman had convulsions and disturbance of consciousness. Head magnetic resonance imaging (MRI) showed punctate areas in the occipital lobes with increased signals on T2-weighted imaging. The MRI abnormalities responded well to steroid pulse therapy, so we made a diagnosis of posterior reversible encephalopathy syndrome (PRES). Three months later, she developed a fever and dyspnea. Chest computed tomography revealed marked thickness of the tracheal and bronchial wall, and bronchoscopy showed a cobble-stone appearance of the tracheal mucosa, indicative of relapsing polychondritis (RPC). We consider that PRES had developed due to autoimmune vasculitis in the brain with RPC.
- Published
- 2020
29. Genetic basis of relapsing polychondritis revealed by family‐based whole‐exome sequencing
- Author
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Xinghua Guo, Junmei Feng, Gui Lian, Linkai Fang, Zhiming Lin, Zuo Xiaoyu, Jieruo Gu, Jun Qi, Xi Zhang, Yanli Zhang, and Qing Lv
- Subjects
Adult ,Male ,Candidate gene ,Heredity ,DNA Mutational Analysis ,Population ,Single-nucleotide polymorphism ,medicine.disease_cause ,Polymorphism, Single Nucleotide ,03 medical and health sciences ,symbols.namesake ,0302 clinical medicine ,Rheumatology ,Predictive Value of Tests ,Exome Sequencing ,medicine ,Humans ,Genetic Predisposition to Disease ,Polychondritis, Relapsing ,030212 general & internal medicine ,Folliculin ,education ,Gene ,Exome sequencing ,030203 arthritis & rheumatology ,Genetics ,Sanger sequencing ,Mutation ,education.field_of_study ,business.industry ,Pedigree ,Phenotype ,symbols ,Female ,business - Abstract
AIM Genetic factors are believed to be implicated in the pathogenesis of relapsing polychondritis (RP). However, the molecular genetic determinants remain to be elucidated. This study aimed to detect the susceptibility genes of RP with whole-exome sequencing (WES) in a Chinese family and deepen our understanding of the pathogenesis of RP thereafter. METHOD A 32-year-old Chinese female proband with RP and her family including her mother with RP were enrolled in the study. The genomic DNA of 6 human subjects was extracted from peripheral blood and then gene allele mutations were identified using WES. Candidate variants with low frequency (
- Published
- 2020
30. Relapsing polychondritis in children: A review
- Author
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Jawaher R Alshanwani and Jubran Theeb Alqanatish
- Subjects
Pediatrics ,medicine.medical_specialty ,Delayed Diagnosis ,Adolescent ,Azathioprine ,Disease ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,Adrenal Cortex Hormones ,Humans ,Medicine ,Polychondritis, Relapsing ,030212 general & internal medicine ,Child ,Relapsing polychondritis ,030203 arthritis & rheumatology ,business.industry ,Incidence (epidemiology) ,medicine.disease ,Therapeutic modalities ,Methotrexate ,Clinical research ,Antirheumatic Agents ,business ,Antirheumatic drugs ,medicine.drug - Abstract
Relapsing polychondritis (RP) is a rare auto-immune disease that causes progressive destruction of cartilaginous structures. Most cases of pediatric-onset RP were published as a single case report or hand-full case series although the prevalence of RP is unknown. This review aimed to describe the characteristics of pediatric-onset RP in order to provide a comparison between childhood and adulthood features of the disease and to review the experiences of biological agents used in children with RP. In children, the diagnosis of RP is either delayed or overlooked due to low incidence and variability in clinical features. Treatment of RP is challenging due to the recurrent episodic nature of the disease. Different immunosuppressive medications, including steroid and steroid-sparing disease-modifying antirheumatic drugs (DMARDs), such as methotrexate or azathioprine, are used to treat RP. There is no rigorous clinical research to support the use of new therapeutic modalities, including biological agents. It is challenging to protocolize the treatment of pediatric-onset RP due to the rarity of the disease. Corticosteroids are the primary form of therapy. However, DMARDs and biological agents may have a role in treating patients with sustained or refractory disease.
- Published
- 2020
31. Aortic involvement in relapsing polychondritis: case-based review
- Author
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Vedat Hamuryudan, Gulen Hatemi, Sinem Nihal Esatoglu, and Mustafa Erdogan
- Subjects
Adult ,Aortic valve ,medicine.medical_specialty ,Computed Tomography Angiography ,Prednisolone ,Immunology ,Methylprednisolone ,Asymptomatic ,03 medical and health sciences ,Aortic aneurysm ,0302 clinical medicine ,Rheumatology ,Fluorodeoxyglucose F18 ,Positron Emission Tomography Computed Tomography ,medicine.artery ,medicine ,Humans ,Immunology and Allergy ,Polychondritis, Relapsing ,030212 general & internal medicine ,Cyclophosphamide ,Aortitis ,Relapsing polychondritis ,030203 arthritis & rheumatology ,Aortic dissection ,Aorta ,business.industry ,Abdominal aorta ,medicine.disease ,Infliximab ,Aortic Aneurysm ,Surgery ,Treatment Outcome ,medicine.anatomical_structure ,Aortic Valve ,cardiovascular system ,Female ,medicine.symptom ,Tomography, X-Ray Computed ,business - Abstract
Relapsing polychondritis is a systemic inflammatory disease that mainly affects ears, nose, eyes, joints, and large airway. Relapsing polychondritis may also affect cardiac valves and large vessels with the aorta being most frequently involved. We conducted a systematic literature review to delineate the clinical characteristics, treatment, and outcome of relapsing polychondritis patients with aortic involvement including thoracic and abdominal aorta, aortic valve, and coronary arteries. 113 patients reported in 85 manuscripts were retrieved through the systematic literature search and references of the selected manuscripts. With the addition of a patient from our center, a total of 114 patients were included in the analyses. Aortic vessel involvement was the predominant type of involvement that was identified in 93 (82%) patients, while aortic valve involvement was identified in 41 patients (36%). The median age at aortic involvement was 37 years [IQR: 30-53] with a delay of 5 years [IQR: 1-8] between first relapsing polychondritis symptom and aortic involvement. Nineteen percent of the patients were asymptomatic at the time of aortic involvement diagnosis. The initial treatment was immunosuppressives in 41 patients (56%) and surgery in 28 patients (38%). The mortality ratio was 27% in a 24 month follow-up [IQR: 7.5-54 months]. Aortic dissection or rupture was the most frequent causes of mortality. Concomitant coronary artery involvement suggested a worse outcome. Aortic involvement in relapsing polychondritis is a mortal complication despite medical and surgical treatments. It may be asymptomatic in 19% of the patients which warrants the importance of screening.
- Published
- 2019
32. P63‐related disorders: Dermatological characteristics in 22 patients
- Author
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Frédéric Caux, Eric E. Gabison, Serge Doan, Smail Hadj-Rabia, Caroline Alby, Christine Bodemer, and Alexia Maillard
- Subjects
Adult ,Male ,0301 basic medicine ,medicine.medical_specialty ,Adolescent ,Cleft Lip ,Erythroderma ,Dermatology ,Biochemistry ,Young Adult ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Ectodermal Dysplasia ,TP63 ,medicine ,Humans ,Eye Abnormalities ,Polychondritis, Relapsing ,Dermatoglyphics ,Child ,Molecular Biology ,Tooth Abnormalities ,business.industry ,Tumor Suppressor Proteins ,High-Throughput Nucleotide Sequencing ,Infant ,medicine.disease ,Phenotype ,Cleft Palate ,stomatognathic diseases ,030104 developmental biology ,Child, Preschool ,Nipples ,Urogenital Abnormalities ,Mutation (genetic algorithm) ,Eyelid Diseases ,Female ,Symptom Assessment ,business ,Pigmentation Disorders ,Dermatitis, Exfoliative ,Hair ,Transcription Factors - Abstract
In P63-related ectodermal dysplasias (ED), the clinical characteristics focus on extra-cutaneous manifestations. The dermatological phenotype remains incompletely characterized. We report the dermatological features of 22 patients carrying a TP63 mutation. Erosions, erythroderma and pigmentary anomalies are characteristics of P63-related ED. Our data suggest that patients might be classified into two major P63-related disorders: AEC and EEC. RHS and ADULT represent mild AEC and EEC forms, respectively.
- Published
- 2019
33. Teaching NeuroImage: Seizures as the Initial Symptom of Relapsing Polychondritis
- Author
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Yujie Meng, Lin Sun, Yingying Xu, Ping Wang, and Shunliang Xu
- Subjects
medicine.medical_specialty ,business.industry ,Seizures ,Medicine ,Humans ,Neurology (clinical) ,Polychondritis, Relapsing ,business ,medicine.disease ,Dermatology ,Relapsing polychondritis - Published
- 2021
34. When You 'Can't See' a Case of Relapsing Polychondritis
- Author
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Varun Bali, Sabetian Katayon, Arash Heidari, David Contreras, Bao Quynh, Navpreet Dhillon, and Rupam Sharma
- Subjects
Adult ,medicine.medical_specialty ,Medicine (General) ,pleocytosis ,genetic structures ,Epidemiology ,Case Report ,glucocorticosteroids ,Young Adult ,R5-920 ,Blurred vision ,Sulfasalazine ,medicine ,Pathology ,relapsing polychondritis ,RB1-214 ,Humans ,Meningitis, Aseptic ,Polychondritis, Relapsing ,Safety, Risk, Reliability and Quality ,Pleocytosis ,Relapsing polychondritis ,medicine.diagnostic_test ,McAdam criteria ,Lumbar puncture ,business.industry ,Aseptic meningitis ,Brain ,Magnetic resonance imaging ,medicine.disease ,Dermatology ,Magnetic Resonance Imaging ,eye diseases ,aseptic meningitis ,Methotrexate ,Female ,medicine.symptom ,business ,Safety Research ,Immunosuppressive Agents ,medicine.drug - Abstract
Relapsing polychondritis (RP) is a rare and, if not treated, potentially lethal autoimmune disorder. Involvement of central nervous system (CNS) in RP is rare and, when present, makes it extremely difficult to diagnose. In this report, we present a case of a 22-year-old Hispanic woman who presented with sudden onset of headache and blurred vision. Magnetic resonance imaging (MRI) of her brain and orbit showed leptomeningeal enhancements in addition to asymmetrical thickening and enhancement of globes. Her lumbar puncture was consistent with aseptic meningitis picture, and she was placed on empirical treatment for presumptive CNS tuberculosis. Her vision deteriorated, and she was diagnosed with RP with CNS and ocular involvement and placed on high-dose steroids with dramatic rapid response. She has been on immunosuppressive treatment, including Sulfasalazine and Methotrexate, since then and her disease has been under control with decreased need for ophthalmic steroid drops. There have been only 19 previous cases found in literature reporting an association of RP with CNS involvement.
- Published
- 2021
35. Vitamin D insufficiency is very frequent and linked to inflammatory biomarkers in relapsing polychondritis
- Author
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J, Freire de Carvalho, M F, Vicente Matias, L M, Carvalho Bastos, and R P, Correia de Araujo
- Subjects
Adult ,Inflammation ,Male ,Skin Cream ,Humans ,Female ,Blood Sedimentation ,Polychondritis, Relapsing ,Middle Aged ,Vitamin D Deficiency ,Biomarkers - Published
- 2021
36. A case of VEXAS syndrome manifesting as Kikuchi-Fujimoto disease, relapsing polychondritis, venous thromboembolism and macrocytic anaemia
- Author
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Bingwen Eugene Fan, Joel Hua-Liang Lim, Joyce Siong See Lee, Shang Ming Samuel Lee, Li Wearn Koh, and Liuh Ling Goh
- Subjects
Male ,medicine.medical_specialty ,Kikuchi-Fujimoto Disease ,business.industry ,Hereditary Autoinflammatory Diseases ,Venous Thromboembolism ,Macrocytic anaemia ,medicine.disease ,Dermatology ,Diagnosis, Differential ,Rheumatology ,medicine ,Humans ,Pharmacology (medical) ,Anemia, Macrocytic ,Polychondritis, Relapsing ,business ,Histiocytic Necrotizing Lymphadenitis ,Venous thromboembolism ,Relapsing polychondritis ,Aged - Published
- 2021
37. Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis
- Author
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Nasam Alfraji and Deborah Alpert
- Subjects
medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Arthritis ,HIV ,Immunosuppression ,HIV Infections ,General Medicine ,Disease ,Articles ,Middle Aged ,medicine.disease ,Dermatology ,Arthritis, Reactive ,Autoimmune Diseases ,medicine ,Etiology ,Humans ,Chondritis ,Reactive arthritis ,Female ,Polychondritis, Relapsing ,business ,Relapsing polychondritis ,Scleritis - Abstract
Patient: Female, 58-year-old Final Diagnosis: Relapsing polychondritis Symptoms: Ear pain • eye pain Medication: — Clinical Procedure: — Specialty: Rheumatology Objective: Rare disease Background: Relapsing polychondritis (RP) is an uncommon autoimmune condition that impacts cartilaginous structures involving the ears, nose, respiratory tract, and joints. Its etiology is unknown; however, it may be associated with other systemic autoimmune diseases, malignancy, and rarely with human immunodeficiency virus (HIV) infection. RP has a variable pattern at presentation and may be associated with constitutional symptoms such as fever and arthralgia, in addition to various auricular, ocular, respiratory, and cardiovascular manifestations. Auricular and ocular signs are the most common presenting features; however, idiopathic orbital inflammatory syndrome is considered a rare manifestation of the disease. Systemic corticosteroids are the mainstay of treatment, but immunomodulatory therapy may be required for refractory cases. Case Report: We present a challenging case of RP in a 58-year-old woman with HIV controlled by highly active anti-retroviral therapy (HAART) and stable chronic reactive arthritis on sulfasalazine who developed unilateral auricular chondritis associated with contralateral idiopathic orbital inflammation and scleritis as well as worsening arthralgia. She was initially treated empirically with antibiotics, without clinical improvement. The infectious diseases work-up was unrevealing, and other diagnostic possibilities were meticulously excluded. Clinical suspicion for RP ultimately led to appropriate therapy with corticosteroids and subsequent immunosuppression with methotrexate, resulting in clinical improvement and allowing for gradual tapering of steroids. Conclusions: RP is an uncommon multisystem disorder that can occur in the setting of other underlying chronic illnesses, as seen in our patient. It has a variable presentation and course, with no diagnostic laboratory tests; therefore, clinical suspicion is imperative for appropriate diagnosis and management.
- Published
- 2021
38. Relapsing polychondritis: state-of-the-art review with three case presentations
- Author
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Hamza Tariq, Jacob Mitchell, Bogna Grygiel-Górniak, Azad Mohammed, and Włodzimierz Samborski
- Subjects
Male ,Systemic disease ,medicine.medical_specialty ,MEDLINE ,Disease ,Nose Diseases ,medicine ,Prevalence ,Humans ,Polychondritis, Relapsing ,Intensive care medicine ,Ear Diseases ,Relapsing polychondritis ,Autoimmune disease ,Biological Products ,business.industry ,General Medicine ,State of the art review ,Middle Aged ,medicine.disease ,Clinical trial ,Treatment Outcome ,Etiology ,Female ,Tumor Necrosis Factor Inhibitors ,sense organs ,Symptom Assessment ,business ,Immunosuppressive Agents - Abstract
BACKGROUND Relapsing polychondritis (RPC) is a complex immune-mediated systemic disease affecting cartilaginous tissue and proteoglycan-rich organs. The most common and earliest clinical features are intermittent inflammation involving the auricular and nasal regions, although all cartilage types can be potentially affected. The life-threatening effects of rpc involve the tracheobronchial tree and cardiac connective components. Rpc is difficult to identify among other autoimmune comorbidities; diagnosis is usually delayed and based on nonspecific clinical symptoms with limited laboratory aid and investigations. Medications can vary, from steroids, immunosuppressants, and biologics, including anti-tnf alpha antagonist drugs. METHOD Information on updated etiology, clinical symptoms, diagnosis, and treatment of rpc has been obtained via extensive research of electronic literature published between 1976 and 2019 using PubMed and medline databases. English was the language of use. Search inputs included 'relapsing polychondritis,' 'polychondritis,' 'relapsing polychondritis symptoms,' and 'treatment of relapsing polychondritis.' Published articles in English that outlined and reported rpc's clinical manifestations and treatment ultimately met the inclusion criteria. Articles that failed to report the above and reported on other cartilaginous diseases met the exclusion criteria. RESULT Utilizing an extensive overview of work undertaken in critical areas of RPC research, this review intends to further explore and educate the approach to this disease in all dimensions from pathophysiology, diagnosis, and management. CONCLUSION RPC is a rare multi-systemic autoimmune disease and possibly fatal. The management remains empiric and is identified based on the severity of the disease per case. The optimal way to advance is to continue sharing data on RPC from reference centers; furthermore, clinical trials in randomized control groups must provide evidence-based treatment and management. Acquiring such information will refine the current knowledge of RPC, which will improve not only treatment but also diagnostic methods, including imaging and biological markers.
- Published
- 2021
39. Mechanical model of steady-state and inflammatory conditions in patients with relapsing polychondritis: A review
- Author
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Jun Shimizu and Noboru Suzuki
- Subjects
Interleukin-6 ,Interleukins ,Interleukin-1beta ,Leukocytes, Mononuclear ,Cytokines ,Humans ,Matrix Metalloproteinase 3 ,General Medicine ,Metagenomics ,Polychondritis, Relapsing ,RNA, Messenger ,Fatty Acids, Volatile ,T-Lymphocytes, Regulatory - Abstract
Relapsing polychondritis (RP) is a multisystem inflammatory disorder, considered to associate with immune aberration.Increased T helper type-1 cell-related cytokines were reported in RP patients. mRNA expressions of a regulatory T cell cytokine interleukin (IL)-10 increased, whereas pro-inflammatory cytokines IL1β and IL6 mRNA expressions decreased in freshly isolated peripheral blood mononuclear cells of RP patients compared with those in healthy individuals. Upon in vitro stimulation with mitogen, IL10 mRNA expressions decreased, and IL1β and IL6 mRNA expressions increased in RP patients.This short-time dynamic change of gene expressions from anti-inflammatory to pro-inflammatory features of immune cells may be associated with the "relapsing" disease course of patients with RP. IL1β mRNA expressions of peripheral blood mononuclear cells exhibited positive correlations with serum matrix metalloproteinase (MMP)-3 concentrations in patients with respiratory involvement. Such positive correlation was not found in those without respiratory involvement.In a metagenomic analysis, an altered composition of gut microbes was found, suggesting that microbe metabolites such as short-chain fatty acids may affect T cell responses of the patients.In this review, the relationships among RP-related inflammatory molecules were summarized. The data support a hypothesis that the immune conditions are different between steady-state and inflammation in RP patients.
- Published
- 2021
40. Co-occurrence of relapsing polychondritis and autoimmune thyroid diseases
- Author
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Toshiki Nakajima, Hajime Yoshifuji, Yoshihisa Yamano, Kimiko Yurugi, Yasuo Miura, Taira Maekawa, Tsuneyasu Yoshida, Hiroshi Handa, Koichiro Ohmura, Tsuneyo Mimori, and Chikashi Terao
- Subjects
Autoimmune thyroid disease ,Relapsing polychondritis ,Humans ,Human Leucocyte Antigen ,Pharmacology (medical) ,Genetic Predisposition to Disease ,General Medicine ,Hashimoto Disease ,Polychondritis, Relapsing ,Genetics (clinical) ,Alleles ,Graves Disease ,Autoimmune Diseases - Abstract
[Background] Relapsing polychondritis (RP) is a rare inflammatory disease characterized by recurrent inflammation and destruction of cartilaginous tissues. RP has characteristics of autoimmune disease and some reports have noted co-occurrence with autoimmune thyroid disease (AITD), consisting of Graves’ disease (GD) and Hashimoto thyroiditis (HT). However, there have been no detailed studies on the co-occurrence of RP and AITD. In this study, we aimed to determine whether patients with RP tend to be complicated with AITD. We also analyzed the clinical and genetic profiles of patients in whom these diseases co-occur. [Methods] We recruited 117 patients with RP and reviewed their medical records. Furthermore, we genotyped Human Leucocyte Antigen (HLA)-A, B Cw, DRB1, DQB1, and DPB1 alleles for 93 of the 117 patients. The prevalence of AITD among the patients with RP was compared with that among the general Japanese population. We also analyzed the clinical and genetic features of the patients with both RP and AITD. [Results] The prevalence of GD among the patients with RP was 4.3% (5 among 117 patients), significantly higher than that among Japanese (0.11%) (p = 2.44 × 10–7, binomial test). RP patients with GD tended to have nasal involvement (p = 0.023) (odds ratio (OR) 2.58) and HLA-DPB1*02:02 (p = 0.035, OR 10.41). We did not find significant enrichment of HT in patients with RP. [Conclusions] Patients with RP appear to be at elevated risk of GD. Nasal involvement and HLA-DPB1*02:02 characterize the subset of RP patients with GD, which may guide attempts to characterize a distinct subtype of RP for precision medicine., 再発性多発軟骨炎はバセドウ病を合併しやすい --統計学的解析から特定の病変・遺伝子型との関係を解明--. 京都大学プレスリリース. 2022-05-10.
- Published
- 2021
41. Relapsing polychondritis after treatment with PD-1 blockade
- Author
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Toyohiro Hirai, Hironori Yoshida, Masanobu Mizuta, and Tatsuya Ogimoto
- Subjects
Male ,medicine.medical_specialty ,medicine.medical_treatment ,Prednisolone ,Programmed Cell Death 1 Receptor ,Gastroenterology ,Fluorodeoxyglucose F18 ,Internal medicine ,Positron Emission Tomography Computed Tomography ,medicine ,Nasal septum ,Humans ,Pharmacology (medical) ,Polychondritis, Relapsing ,Adverse effect ,Nose ,Relapsing polychondritis ,Aged ,Pharmacology ,business.industry ,Cancer ,medicine.disease ,Radiation therapy ,Radiation Pneumonitis ,medicine.anatomical_structure ,Nivolumab ,Oncology ,business ,medicine.drug - Abstract
Nivolumab, a programmed death 1 blockade drug, is used in various types of cancers and can cause a unique immune-related adverse event (irAE). Relapsing polychondritis (RP) is a rare autoimmune disease that mainly involves inflammation of the auricle, nose and airway cartilage. A 72-year-old man with mandibular cancer received nivolumab after surgery for the primary lesion and radiation therapy for lung metastases. He then developed radiation pneumonitis, and prednisolone (PSL) was started. During the tapering of PSL, he developed exertional dyspnea and cough. The condition of mandibular cancer and radiation pneumonitis had not deteriorated. Fluorodeoxyglucose (FDG)-PET/CT showed a thickening of and abnormal FDG uptake in the tracheobronchial and nasal septum cartilage. These characteristic findings were not observed before nivolumab was initiated; thus, we clinically diagnosed the patient as having RP induced by nivolumab. Since the symptoms were mild, the patient's condition was carefully managed with inhaled corticosteroids, and the RP has not progressed thus far. Physicians should be aware that RP can occur as an irAE because RP may progress to serious respiratory symptoms.
- Published
- 2021
42. [Rapid Growing Thoracic Aortic Aneurysm in a Patient with Relapsing Polychondritis]
- Author
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Takahiro, Yamamoto, Koichi, Adachi, Daijiro, Hori, Tomonari, Fujimori, Manabu, Shiraishi, Naoyuki, Kimura, and Atsushi, Yamaguchi
- Subjects
Adult ,Male ,Blood Vessel Prosthesis Implantation ,Aortic Aneurysm, Thoracic ,Thoracotomy ,Humans ,Aorta, Thoracic ,Polychondritis, Relapsing ,Aorta - Abstract
A 26-year-old man with relapsing polychondritis was admitted for the treatment of multiple thoracic aortic aneurysms in the ascending and descending aorta. Descending thoracic aortic aneurysm showed rapid expansion, therefore, the patient underwent an extended thoracic aortic repair from the ascending aorta to the descending aorta via anterolateral thoracotomy and partial sternotomy. Although postoperative course was uneventful, aortic root enlargement and severe aortic insufficiency progressed over the next two years. He and his family refused redo surgical intervention and the patient died of heart failure. Careful perioperative follow-up may be mandatory in a patient with relapsing polychondritis complicated by cardiovascular disease.
- Published
- 2021
43. Characteristics and Clinical Outcomes of 295 Patients With Relapsing Polychondritis
- Author
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Yi Zheng and Nan Chen
- Subjects
Adult ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Immunology ,Airway obstruction ,Middle Aged ,medicine.disease ,Laryngeal Obstruction ,Tracheotomy ,Tracheostomy ,Rheumatology ,Respiratory failure ,Internal medicine ,Lower respiratory tract infection ,medicine ,Immunology and Allergy ,Humans ,Polychondritis, Relapsing ,business ,Airway ,Respiratory Tract Infections ,Relapsing polychondritis ,Cause of death ,Retrospective Studies - Abstract
ObjectiveThis study analyzes the clinical features of Chinese patients with relapsing polychondritis (RP).MethodsThe clinical data of 295 patients with RP at Beijing Tongren Hospital were retrospectively analyzed.ResultsThe mean age of onset was 41.0 ± 15.0 years. The sex ratio was 1:1. Up to 70.5% of the patients had airway involvement during the disease course; among them, the larynx was most commonly affected (82.2%). One-quarter (25.7%) of the patients with laryngeal involvement underwent tracheotomy as a result of progressive dyspnea or acute laryngeal obstruction. Younger age at onset and respiratory symptoms at initial presentation were independent risk factors for tracheotomy in patients with RP with laryngeal involvement. The risk of tracheotomy in patients who presented with respiratory symptoms was 2.35 times higher than that of patients who presented with other symptoms (HR 2.35, 95% CI 1.23–4.50,P= 0.01). The risk of tracheotomy increased by 4.8% for every 1-year decrease in the age at onset (HR 0.95, 95% CI 0.93–0.97,P< 0.001). The incidence of lower respiratory tract infection was much higher in patients with airway involvement than in those without airway involvement. The main cause of death was respiratory failure as a result of airway obstruction.ConclusionThere is a high prevalence of airway involvement in Chinese patients with RP. Laryngeal involvement is associated with a high risk of death. More attention should be paid to patients with RP with laryngeal involvement who are young at disease onset and present with respiratory symptoms.
- Published
- 2021
44. A rare cause of multiple airways narrowing in a 15-year-old girl
- Author
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May El Hachem, Maria Giovanna Paglietti, Renato Cutrera, Fabrizio De Benedetti, Federica Porcaro, Nicola Ullmann, Sergio Ghirardo, and Maria Beatrice Chiarini Testa
- Subjects
Diagnostic Imaging ,Pulmonary and Respiratory Medicine ,Vital capacity ,Adolescent ,Biopsy ,Stridor ,Physical examination ,Diagnosis, Differential ,03 medical and health sciences ,FEV1/FVC ratio ,0302 clinical medicine ,Humans ,Medicine ,Polychondritis, Relapsing ,030212 general & internal medicine ,medicine.diagnostic_test ,business.industry ,Respiratory infection ,medicine.disease ,Antibiotic coverage ,Pulmonary hypertension ,Respiratory Function Tests ,030228 respiratory system ,Anesthesia ,Salbutamol ,Drug Therapy, Combination ,Female ,medicine.symptom ,business ,medicine.drug - Abstract
Sergio Ghirardo (SG): a 15-year-old girl was first admitted to our unit, with significant dyspnoea and fatigue at rest. Her clinical history was uneventful until she was 14 years old when she was hospitalised twice for respiratory symptoms, interpreted as post viral wheezing. Treatment with intravenous corticosteroids, salbutamol and azithromycin was of little clinical benefit. In the last 6 months, she complained of progressive exercise-induced dyspnoea, and her lung function showed a moderate-to-severe obstructive pattern (forced expiratory volume in one second, FEV1/forced vital capacity, FVC 56.6%), with no response to salbutamol. Her symptoms did not improve with inhaled corticosteroids, long-acting β2 agonists and montelukast treatment. Clinically, she reported moderate swelling of the nasal dorsum as a consequence of a blow with a ball received 5 months before admission. She had a bilateral transmissive mild hypoacusis and a significative weight loss of 18 kg in only 8 months. On initial physical examination she appeared pale and tired with a mild redness of the ear helix sparing the lobe and a mild swelling of the nasal dorsum. She was tachypnoeic (20 breaths/min), with normal levels of oxygen saturation. Mild supraclavicular retractions were present during inspiration. On chest auscultation stridor and mild bilateral wheezing were detected. Heart rate was 109 beats/min. The remaining physical examination was normal. Renato Cutrera (RC): we have a previously healthy female adolescent with a personal history of only partially remitting obstructive dyspnoea suggesting a chronic respiratory infection (like tuberculosis or pulmonary mycosis). However, the fatigue associated with significant weight loss should raise the suspicion of pulmonary hypertension. I suggest performing routine blood tests, chest X-ray, full blood count and cardiological evaluation. At the same time, I would administer oral corticosteroids and antibiotic coverage. Federica Porcaro (FP): blood tests showed a mild rise of C reactive protein (CRP) 7.2 mg/L (normal range 0–5 mg/L); whereas …
- Published
- 2020
45. Dapsone-induced methaemoglobinaemia in relapsing polychondritis
- Author
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Tomohiro Yoshida, Keisuke Nishimura, Hiroyuki Murabe, and Toshihiko Yokota
- Subjects
Humans ,Polychondritis, Relapsing ,General Medicine ,Methemoglobinemia ,Dapsone - Published
- 2022
46. Hidradenitis suppurativa and relapsing polychondritis: An uncommon association
- Author
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Nour El Imene Ouni, Nesrine Ben Salah, and Amina Aounallah
- Subjects
Rheumatology ,Humans ,Polychondritis, Relapsing ,Hidradenitis Suppurativa - Published
- 2022
47. Relapsing polychondritis occurring after esophageal cancer surgery: a case report with a literature review
- Author
-
Yuta Sato, Takeharu Imai, Takao Takahashi, Naoki Okumura, Yoshihiro Tanaka, Kazuhiro Yoshida, Junichi Mase, and Nobuhisa Matsuhashi
- Subjects
Male ,medicine.medical_specialty ,medicine.diagnostic_test ,Respiratory distress ,Esophageal Neoplasms ,business.industry ,Gastroenterology ,General Medicine ,Esophageal cancer ,medicine.disease ,Dysphagia ,Surgery ,Endoscopy ,medicine.anatomical_structure ,Cricoid cartilage ,Biopsy ,medicine ,Humans ,Polychondritis, Relapsing ,medicine.symptom ,Neoplasm Recurrence, Local ,business ,Relapsing polychondritis ,Abdominal surgery ,Aged - Abstract
Relapsing polychondritis is a relatively rare chronic inflammatory disease of unknown etiology. In this case the treatment for esophageal cancer may have triggered relapsing polychondritis. A 70-year-old man complained of dysphagia and weight loss. An upper gastrointestinal endoscopy revealed type 2 advanced esophageal cancer. A subtotal esophagectomy and three-region lymph node dissection were performed after chemotherapy. One month later, the patient developed respiratory distress accompanied by wheezing, dizziness, and hearing loss. The symptoms improved within a few days. The frequency of respiratory distress increased and the patient visited our department. Pharyngeal endoscopy revealed narrowing of the glottic space and a subglottic tumor. No malignant findings were found histopathologically on the biopsy specimens, but infiltration of inflammatory cells was observed. We diagnosed relapsing polychondritis based on the histopathological findings of the pharyngeal cartilage, in addition to the osteolytic changes of the cricoid cartilage on CT. The symptoms were relieved after the administration of oral steroids. Despite tapering of the steroids, no recurrence of relapsing polychondritis occurred. There was no evidence of esophageal cancer recurrence. Early diagnosis and treatment for relapsing polychondritis are necessary because this condition is often associated with airway lesions. Esophageal cancer treatment may trigger relapsing polychondritis.
- Published
- 2021
48. Efficacy of tocilizumab for refractory relapsing polychondritis with tracheal stenosis and respiratory distress
- Author
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Hsien-Tzung Liao
- Subjects
Adult ,medicine.medical_specialty ,Respiratory Distress Syndrome ,Respiratory distress ,business.industry ,medicine.disease ,Antibodies, Monoclonal, Humanized ,Tracheal Stenosis ,chemistry.chemical_compound ,Tocilizumab ,Rheumatology ,chemistry ,Refractory ,Internal medicine ,medicine ,Humans ,Pharmacology (medical) ,Female ,Polychondritis, Relapsing ,business ,Relapsing polychondritis - Published
- 2021
49. 'Cave Aurem'! Chondritis/Perichondritis in the Course of a Systemic Disease
- Author
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Galya, Tanay and Amir, Tanay
- Subjects
Vasculitis ,Urticaria ,Humans ,Female ,Polychondritis, Relapsing ,Syndrome ,Ear Cartilage ,Middle Aged ,Cartilage Diseases - Published
- 2021
50. Discordance in patient and physician global assessment in relapsing polychondritis
- Author
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Marcela A. Ferrada, Laurent Arnaud, Jeff Kim, Arlene Sirajuddin, Marcus Y. Chen, Kaitlin A. Quinn, Wendy Goodspeed, Clint T. Allen, Emily Rose, and Peter C. Grayson
- Subjects
Adult ,medicine.medical_specialty ,business.industry ,Concordance ,Outcome measures ,Geriatric assessment ,Disease ,Clinical Science ,medicine.disease ,Severity of Illness Index ,Rheumatology ,Interquartile range ,Internal medicine ,Physicians ,medicine ,Humans ,Pharmacology (medical) ,In patient ,Patient Reported Outcome Measures ,Polychondritis, Relapsing ,Prospective Studies ,business ,Prospective cohort study ,Relapsing polychondritis - Abstract
Objectives Relapsing polychondritis (RP) is a rare, heterogeneous, systemic inflammatory disease that targets cartilage. Patient-reported outcome measures may differ from physician assessment. This study compared patient global assessment (PtGA) and physician global assessment (PhGA) scores in a prospective cohort of patients with RP. Methods Adult patients with RP underwent a standardized comprehensive evaluation at ∼6 month intervals. At each visit, three physicians scored PhGA by consensus. The patient independently completed four patient-reported outcomes: PtGA, 36-item Short Form Health Survey (SF-36), Brief Illness Perception Questionnaire (BIPQ) and Multidimensional Fatigue Inventory (MFI). Patient–physician discordance was defined as a difference between PtGA and PhGA of ≥3 on a 0–10 scale. Results A total of 76 patients were evaluated over 154 visits. The median PhGA was 3 [interquartile range (IQR) 2–3] and the median PtGA was 5 (IQR 4–7). PtGA and PhGA were concordant in 66 visits (42.9%) and patients scored disease severity ≥3 points higher than physicians scored disease activity (positive discordance) in 84 visits (54.5%). Compared with visits with concordance, visits with positive discordance were associated with significantly worse scores on the MFI, BIPQ, SF-36 physical component score and SF-36 mental component score. Conclusion Patients with RP typically self-report high PtGA that does not align with PhGA. Discordance is likely driven by the high physical and psychological burden of illness experienced by patients. Multifaceted treatment approaches that address the burden of disease in RP from the patient perspective are needed.
- Published
- 2021
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