A case of localized tracheobronchial relapsing polychondritis with positive matrilin-1 staining.
Matrilin-1
Relapsing polychondritis
Tracheobronchial
Journal
BMC rheumatology
ISSN: 2520-1026
Titre abrégé: BMC Rheumatol
Pays: England
ID NLM: 101738571
Informations de publication
Date de publication:
2020
2020
Historique:
received:
03
06
2019
accepted:
30
10
2019
entrez:
5
2
2020
pubmed:
6
2
2020
medline:
6
2
2020
Statut:
epublish
Résumé
Relapsing polychondritis (RPC) is a rare progressive autoimmune disease characterized by inflammation in the cartilage of multiple organs. Tracheobronchial involvement appears in nearly half of RPC patients during the course of their disease and represents the main cause of death. Localized tracheobronchial RPC is much rarer, and the pathogenesis remains unclear. Matrilin-1 is a non-collagenous cartilage matrix protein and has been suggested to be a potent autoantigen that induces the airway disease of RPC in animal models. However, the expression of matrilin-1 in tracheobronchial tissue in human remains unclear. Therefore, we examined the expression of matrilin-1 in the tracheal and auricular tissues in a localized tracheobronchial RPC patient. A 62-year-old man with systemic sclerosis presented with cough and dyspnea on exertion. The lung function test showed an expiratory flow limitation and chest computed tomography showed diffuse thickness from the trachea to the bronchiole. No other tests showed abnormal findings. To evaluate further, bronchoscopy was performed and endobronchial ultrasonography showed thickness in the fourth-marginal echo layer suggesting inflammation of the cartilage. However, the tracheal biopsy showed no specific findings. The subsequent surgical tracheal biopsies showed inflammatory cell infiltration with destruction of the cartilage. Neither auricular nor nasal deformity, except for a tracheobronchial lesion, was detected. Biopsy from the left auricular cartilage also did not show any inflammatory changes. Finally, we diagnosed the patient with localized tracheobronchial RPC. To address the hypothesis that autoimmunity against matrilin-1 is involved in the pathogenesis of localized tracheobronchial RPC, we evaluated the expression level of matrilin-1 in a tracheal and auricular specimen from this patient. Immunohistochemical staining with anti-matrilin-1 antibody showed matrilin-1 in the tracheal but not in the auricular cartilage. We first demonstrated the expression of matrilin-1 in tracheal but not in auricular cartilage in a localized tracheobronchial RPC patient. This result supports the possibility that matrilin-1 is involved in the pathogenesis of localized tracheobronchial RPC. However, this is only one case report and further observations will be needed to confirm this result.
Sections du résumé
BACKGROUND
BACKGROUND
Relapsing polychondritis (RPC) is a rare progressive autoimmune disease characterized by inflammation in the cartilage of multiple organs. Tracheobronchial involvement appears in nearly half of RPC patients during the course of their disease and represents the main cause of death. Localized tracheobronchial RPC is much rarer, and the pathogenesis remains unclear. Matrilin-1 is a non-collagenous cartilage matrix protein and has been suggested to be a potent autoantigen that induces the airway disease of RPC in animal models. However, the expression of matrilin-1 in tracheobronchial tissue in human remains unclear. Therefore, we examined the expression of matrilin-1 in the tracheal and auricular tissues in a localized tracheobronchial RPC patient.
CASE PRESENTATION
METHODS
A 62-year-old man with systemic sclerosis presented with cough and dyspnea on exertion. The lung function test showed an expiratory flow limitation and chest computed tomography showed diffuse thickness from the trachea to the bronchiole. No other tests showed abnormal findings. To evaluate further, bronchoscopy was performed and endobronchial ultrasonography showed thickness in the fourth-marginal echo layer suggesting inflammation of the cartilage. However, the tracheal biopsy showed no specific findings. The subsequent surgical tracheal biopsies showed inflammatory cell infiltration with destruction of the cartilage. Neither auricular nor nasal deformity, except for a tracheobronchial lesion, was detected. Biopsy from the left auricular cartilage also did not show any inflammatory changes. Finally, we diagnosed the patient with localized tracheobronchial RPC. To address the hypothesis that autoimmunity against matrilin-1 is involved in the pathogenesis of localized tracheobronchial RPC, we evaluated the expression level of matrilin-1 in a tracheal and auricular specimen from this patient. Immunohistochemical staining with anti-matrilin-1 antibody showed matrilin-1 in the tracheal but not in the auricular cartilage.
CONCLUSIONS
CONCLUSIONS
We first demonstrated the expression of matrilin-1 in tracheal but not in auricular cartilage in a localized tracheobronchial RPC patient. This result supports the possibility that matrilin-1 is involved in the pathogenesis of localized tracheobronchial RPC. However, this is only one case report and further observations will be needed to confirm this result.
Identifiants
pubmed: 32016169
doi: 10.1186/s41927-019-0103-6
pii: 103
pmc: PMC6988282
doi:
Types de publication
Case Reports
Langues
eng
Pagination
1Informations de copyright
© The Author(s) 2020.
Déclaration de conflit d'intérêts
Competing interestsThe authors declare that they have no competing interests.
Références
Arthritis Rheum. 1995 Feb;38(2):294-6
pubmed: 7848324
Eur Respir J. 2006 Sep;28(3):666-9
pubmed: 16946098
Lancet. 2015 Jan 3;385(9962):88
pubmed: 25592904
Radiol Med. 2007 Dec;112(8):1132-41
pubmed: 18074199
J Clin Invest. 1999 Sep;104(5):589-98
pubmed: 10487773
N Engl J Med. 2008 Jan 31;358(5):536-7
pubmed: 18234765
Tissue Antigens. 1984 May;23(5):263-9
pubmed: 6589808
J Autoimmun. 2014 Feb-Mar;48-49:53-9
pubmed: 24461536
Biomedicines. 2018 Aug 02;6(3):null
pubmed: 30072598
Int J Biochem Cell Biol. 2011 Mar;43(3):320-30
pubmed: 21163365
Respiration. 2000;67(3):320-2
pubmed: 10867603
Autoimmun Rev. 2014 Feb;13(2):90-5
pubmed: 24051104
Medicine (Baltimore). 1976 May;55(3):193-215
pubmed: 775252
Medicine (Baltimore). 2001 May;80(3):173-9
pubmed: 11388093
Arthritis Rheum. 2001 Oct;44(10):2402-12
pubmed: 11665983
Am J Pathol. 2004 Mar;164(3):959-66
pubmed: 14982849
Chest. 2003 Dec;124(6):2393-5
pubmed: 14665528
Eur J Med Res. 1996 Nov 25;1(12):554-8
pubmed: 9438161
Arthritis Rheum. 1989 Sep;32(9):1080-6
pubmed: 2775318
Ann Intern Med. 1986 Jan;104(1):74-8
pubmed: 3484422
Adv Drug Deliv Rev. 2003 Nov 28;55(12):1569-93
pubmed: 14623402