Purpose: To retrospectively determine the prevalence of expiratory computed tomographic (CT) abnormalities, including malacia and air trapping, in patients with relapsing polychondritis and to retrospectively determine the frequency with which expiratory abnormalities are accompanied by inspiratory abnormalities on CT scans.
Materials And Methods: Institutional review board approval was obtained, and informed consent was not required for this retrospective HIPAA-compliant study. A computerized hospital information system was used to identify all patients with clinically diagnosed or biopsy-proved relapsing polychondritis who were referred for CT airway imaging during a 17-month period. The study cohort comprised 18 patients (15 women, three men; mean age, 47 years; age range, 20-71 years). Multidetector helical CT was performed in all patients by using a standard protocol, which included end-inspiratory and dynamic expiratory volumetric imaging. Two observers who were blinded to the original scan interpretations simultaneously reviewed CT scans. Findings were recorded in consensus. Dynamic expiratory CT scans were assessed for malacia that involved the trachea and main bronchi (reduction in cross-sectional area of more than 50%) and for air trapping (failure of lung parenchyma to increase in attenuation during expiration). Air trapping was visually classified according to pattern and extent (lobular, segmental, lobar, or whole lung). Inspiratory CT scans were evaluated for tracheal and bronchial stenosis (>25% luminal diameter narrowing compared with a corresponding uninvolved segment), wall thickening (>2 mm), and calcification.
Results: Expiratory CT abnormalities were present in 17 (94%) of 18 patients and included malacia in 13 patients (72%) and air trapping in 17 patients (94%). Inspiratory CT abnormalities were found in eight (47%) of 17 patients who had expiratory CT abnormalities. Calcification of the airway walls was present in seven (39%) of 18 patients. All patients who had inspiratory CT abnormalities demonstrated expiratory CT abnormalities.
Conclusion: Expiratory CT abnormalities were present in the majority of patients with relapsing polychondritis who were referred for airway imaging, yet only half of these patients demonstrated abnormalities on routine inspiratory CT scans. Thus, dynamic expiratory CT should be a standard component of imaging assessment in patients with relapsing polychondritis.
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http://dx.doi.org/10.1148/radiol.2401050562 | DOI Listing |
Int J Med Sci
January 2025
Department of Rheumatism and Immunology, Tianjin First Central hospital, Tianjin, China.
Autoimmune inner ear disease (AIED) is a rare condition characterized by immune-mediated damage to the inner ear, leading to progressive sensorineural hearing loss (SNHL) and vestibular symptoms such as vertigo and tinnitus. This study investigates the pathogenesis and therapeutic strategies for AIED through the analysis of three cases with different underlying autoimmune disorders: rheumatoid arthritis, relapsing polychondritis, and IgG4-related disease. The etiology of AIED involves complex immunopathological mechanisms, including molecular mimicry and the "bystander effect," with specific autoantibodies, such as those against heat shock protein 70 (HSP70), playing a potential role in cochlear damage.
View Article and Find Full Text PDFInt J Dermatol
December 2024
Department of Dermatology, International University of Health and Welfare, Chiba, Japan.
Rev Med Interne
December 2024
Service de médecine interne, CHI Poissy-St Germain, 10, rue du Champs Gaillard, 78300 Poissy, France.
Introduction: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic), recently described, due to a somatic mutation of the UBA1 gene and often associated with hemopathy, is characterized by systemic symptoms close to those described in Still's disease or relapsing polychondritis. There are also patients with hemopathy, presenting inflammatory symptoms reminiscent of those of VEXAS syndrome but without mutation of the UBA1 gene.
Case/discussion: Two male patients consulted for general signs, dermatological symptoms, arthralgia, chondritis and venous thrombosis, like patients in the French cohort suffering from VEXAS syndrome.
Immunotherapy
December 2024
Department of Clinical Immunology and Allergy, Flinders Medical Centre, Bedford Park, South Australia.
Relapsing polychondritis is rare and affects non-synovial fibrocartilage. Currently, there is a paucity of treatment algorithms, especially for those with refractory disease. A middle-aged man presented with polychondritis affecting the nose, ears, joints, and larynx.
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