Intermittent fever and cough in a 56-year-old patient: Relapsing polychondritis and extranodal NK/T-cell lymphoma
We report a case of a 56-year-old female subject presented as intermittent fever and cough, who was found obvious luminal stenosis and intense 18F-fluorodeoxyglucose (FDG) uptake in her larynx and trachea via bronchoscopy and FDG positron emission tomography/computed tomography (PET/CT). The auricular cartilage biopsy demonstrated chondritis. At first she was diagnosed as RP and treated by glucocorticoid and methotrexate, leading to completely response. Fever and cough recurred after 18 months, and FDG PET/CT were performed again and targeted a newfound nasopharyngeal lesion, where the biopsy proved to be an extranodal nat...
Source: Cell Research - May 4, 2023 Category: Cytology Authors: Qin Huang Danyu Cui Jianhui Chen Hao Ren Min Yang Source Type: research
Clinical and genetic features of Japanese cases of MDS associated with VEXAS syndrome
Int J Hematol. 2023 Apr 17. doi: 10.1007/s12185-023-03598-8. Online ahead of print.ABSTRACTVEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a new disease entity with autoinflammatory disorders (AID) driven by somatic variants in UBA1 that frequently co-exists with myelodysplastic syndromes (MDS). Clinicopathological and molecular features of Japanese cases with VEXAS-associated MDS remain elusive. We previously reported high prevalence of UBA1 variants in Japanese patients with relapsing polychondritis, in which 5 cases co-occurred with MDS. Here, we report clinicopathological and variant profil...
Source: International Journal of Hematology - April 16, 2023 Category: Hematology Authors: Hiroyoshi Kunimoto Ayaka Miura Ayaka Maeda Naomi Tsuchida Yuri Uchiyama Yosuke Kunishita Yuki Nakajima Kaoru Takase-Minegishi Ryusuke Yoshimi Takuya Miyazaki Maki Hagihara Etsuko Yamazaki Yohei Kirino Naomichi Matsumoto Hideaki Nakajima Source Type: research
Clinical and genetic features of Japanese cases of MDS associated with VEXAS syndrome
Int J Hematol. 2023 Apr 17. doi: 10.1007/s12185-023-03598-8. Online ahead of print.ABSTRACTVEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a new disease entity with autoinflammatory disorders (AID) driven by somatic variants in UBA1 that frequently co-exists with myelodysplastic syndromes (MDS). Clinicopathological and molecular features of Japanese cases with VEXAS-associated MDS remain elusive. We previously reported high prevalence of UBA1 variants in Japanese patients with relapsing polychondritis, in which 5 cases co-occurred with MDS. Here, we report clinicopathological and variant profil...
Source: International Journal of Hematology - April 16, 2023 Category: Hematology Authors: Hiroyoshi Kunimoto Ayaka Miura Ayaka Maeda Naomi Tsuchida Yuri Uchiyama Yosuke Kunishita Yuki Nakajima Kaoru Takase-Minegishi Ryusuke Yoshimi Takuya Miyazaki Maki Hagihara Etsuko Yamazaki Yohei Kirino Naomichi Matsumoto Hideaki Nakajima Source Type: research
Clinical and genetic features of Japanese cases of MDS associated with VEXAS syndrome
Int J Hematol. 2023 Apr 17. doi: 10.1007/s12185-023-03598-8. Online ahead of print.ABSTRACTVEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a new disease entity with autoinflammatory disorders (AID) driven by somatic variants in UBA1 that frequently co-exists with myelodysplastic syndromes (MDS). Clinicopathological and molecular features of Japanese cases with VEXAS-associated MDS remain elusive. We previously reported high prevalence of UBA1 variants in Japanese patients with relapsing polychondritis, in which 5 cases co-occurred with MDS. Here, we report clinicopathological and variant profil...
Source: International Journal of Hematology - April 16, 2023 Category: Hematology Authors: Hiroyoshi Kunimoto Ayaka Miura Ayaka Maeda Naomi Tsuchida Yuri Uchiyama Yosuke Kunishita Yuki Nakajima Kaoru Takase-Minegishi Ryusuke Yoshimi Takuya Miyazaki Maki Hagihara Etsuko Yamazaki Yohei Kirino Naomichi Matsumoto Hideaki Nakajima Source Type: research
SAPHO syndrome complicated with relapsing polychondritis: A case report
AbstractSynovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic inflammatory disease. The main clinical manifestation of SAPHO syndrome is an osteoarthropathy with cutaneous involvement. Relapsing polychondritis (RP) characterized by chronic inflammation and cartilage degeneration is a rare systematic autoimmune disease. Here we report a RP case in a SAPHO syndrome patient, in which auricularitis happened 10 years after the diagnosed as SAPHO syndrome. Tofacitinib treatment can alleviate the symptoms. (Source: International Journal of Rheumatic Diseases)
Source: International Journal of Rheumatic Diseases - April 7, 2023 Category: Rheumatology Authors: Fengjiao Cao,
Xiujuan Hou,
Tianlun Kang,
Xiaojun Shi,
Weiguo Ma,
Yuan Zhang,
Chen Li Tags: CASE REPORT Source Type: research
Pre-treatment bronchoscopic evaluation in a case of relapsing polychondrits
Relapsing polychondritis (RP) is a chronic and recurrent inflammatory disease of the cartilage tissues in the body. The cause of RP is unknown, and since it is a rare disease with symptoms that affect multiple... (Source: BMC Pulmonary Medicine)
Source: BMC Pulmonary Medicine - April 3, 2023 Category: Respiratory Medicine Authors: Kosumi Kumagai, Hajime Tsuruoka, Kei Morikawa, Hiroshi Handa and Masamichi Mineshita Tags: Case report Source Type: research
Systemic Disease Associations in a Cohort of Hispanic Patients with Scleritis
Conclusion: Rheumatoid arthritis was the most common systemic autoimmune disease association, while syphilis was the most common infectious disease associated with scleritis patients. Our study suggests that patients with nodular scleritis have a lower risk of having an associated immune-mediated disease.PMID:36902755 | DOI:10.3390/jcm12051969 (Source: Herpes)
Source: Herpes - March 11, 2023 Category: Infectious Diseases Authors: Cristina Arruza Guillermo A Requejo Figueroa Javier Col ón Estefania Ramirez Marquez Guillermo Puebla Daniela Ortega Mariella Pappaterra Rodriguez Jos é López Fontanet Alexandra Col ón Sof ía C Ayala Rodríguez Erick Rivera Grana Frances Marrero Emil Source Type: research
Systemic Disease Associations in a Cohort of Hispanic Patients with Scleritis
Conclusion: Rheumatoid arthritis was the most common systemic autoimmune disease association, while syphilis was the most common infectious disease associated with scleritis patients. Our study suggests that patients with nodular scleritis have a lower risk of having an associated immune-mediated disease.PMID:36902755 | DOI:10.3390/jcm12051969 (Source: Herpes)
Source: Herpes - March 11, 2023 Category: Infectious Diseases Authors: Cristina Arruza Guillermo A Requejo Figueroa Javier Col ón Estefania Ramirez Marquez Guillermo Puebla Daniela Ortega Mariella Pappaterra Rodriguez Jos é López Fontanet Alexandra Col ón Sof ía C Ayala Rodríguez Erick Rivera Grana Frances Marrero Emil Source Type: research
The ocular manifestations of relapsing polychondritis
ConclusionMany patients with RPC will be seen by an ophthalmologist during the course of their disease. Knowledge and awareness of RPC and its ocular manifestations is therefore essential to enable the ophthalmologist to make the diagnosis. (Source: International Ophthalmology)
Source: International Ophthalmology - March 1, 2023 Category: Opthalmology Source Type: research
Ear Swelling of Relapsing Polychondritis
(Source: Journal of General Internal Medicine)
Source: Journal of General Internal Medicine - February 28, 2023 Category: Internal Medicine Source Type: research
Relationship between histopathological features of non-infectious aortitis and the results of pre-operative 18F-FDG-PET/CT: a retrospective study of 16 patients
CONCLUSIONS: In histologically proved aortitis, 18FDG-PET/CT before surgery did not detect vascular inflammation in 31% patients, and PETVAS score correlated with the degree of adventitial histopathologic inflammation.PMID:36762741 | DOI:10.55563/clinexprheumatol/5mevq4 (Source: Clinical and Experimental Rheumatology)
Source: Clinical and Experimental Rheumatology - February 10, 2023 Category: Rheumatology Authors: Simon Parreau Olivier Espitia Michael S Bold Livia M Frota Lima Guillaume Lades M élanie Bois Morgane Assaraf David Saadoun Matthew J Koster Kim-Heang Ly Cornelia M Weyand Kenneth J Warrington Eric Liozon Source Type: research
