Lymphocyte-Directed Immunomodulation Remits Thymoma-Associated Autoimmune Pneumonitis.
Humans
Thymoma
/ immunology
Female
Immunomodulation
Male
Rituximab
/ therapeutic use
Autoantibodies
/ immunology
Middle Aged
Thymus Neoplasms
/ immunology
Pneumonia
/ etiology
Autoimmune Diseases
/ immunology
Adult
Azathioprine
/ therapeutic use
B-Lymphocytes
/ immunology
Treatment Outcome
T-Lymphocytes
/ immunology
Azathioprine
Bronchiectasis
KCNRG
Pneumonitis
Rituximab
Thymoma
Journal
Journal of clinical immunology
ISSN: 1573-2592
Titre abrégé: J Clin Immunol
Pays: Netherlands
ID NLM: 8102137
Informations de publication
Date de publication:
01 Jul 2024
01 Jul 2024
Historique:
received:
08
04
2024
accepted:
25
06
2024
medline:
2
7
2024
pubmed:
2
7
2024
entrez:
2
7
2024
Statut:
epublish
Résumé
Thymoma presents with several autoimmune manifestations and is associated with secondary autoimmune regulator (AIRE) deficiency. Pneumonitis has recently been described as an autoimmune manifestation associated with thymoma presenting with similar clinical, radiographic, histological, and autoantibody features as seen in patients with inherited AIRE deficiency who suffer from Autoimmune PolyEndocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED) syndrome. To treat two patients with biopsy-proven thymoma-associated pneumonitis with lymphocyte-directed immunomodulation. Two patients with thymoma were enrolled on IRB-approved protocols at the NIH Clinical Center. We performed history and physical examination; laboratory, radiographic, histologic and pulmonary function evaluations; and measurement of the lung-directed autoantibodies KCNRG and BPIFB1 prior to and at 1- and 6-months following initiation of lymphocyte-directed immunomodulation with azathioprine with or without rituximab. Combination T- and B-lymphocyte-directed immunomodulation resulted in improvement of clinical, functional, and radiographic parameters at 6-month follow-up evaluations in both patients with sustained remission up to 12-36 months following treatment initiation. Lymphocyte-directed immunomodulation remitted autoimmune pneumonitis in two patients with thymoma.
Sections du résumé
BACKGROUND
BACKGROUND
Thymoma presents with several autoimmune manifestations and is associated with secondary autoimmune regulator (AIRE) deficiency. Pneumonitis has recently been described as an autoimmune manifestation associated with thymoma presenting with similar clinical, radiographic, histological, and autoantibody features as seen in patients with inherited AIRE deficiency who suffer from Autoimmune PolyEndocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED) syndrome.
OBJECTIVES
OBJECTIVE
To treat two patients with biopsy-proven thymoma-associated pneumonitis with lymphocyte-directed immunomodulation.
METHODS
METHODS
Two patients with thymoma were enrolled on IRB-approved protocols at the NIH Clinical Center. We performed history and physical examination; laboratory, radiographic, histologic and pulmonary function evaluations; and measurement of the lung-directed autoantibodies KCNRG and BPIFB1 prior to and at 1- and 6-months following initiation of lymphocyte-directed immunomodulation with azathioprine with or without rituximab.
RESULTS
RESULTS
Combination T- and B-lymphocyte-directed immunomodulation resulted in improvement of clinical, functional, and radiographic parameters at 6-month follow-up evaluations in both patients with sustained remission up to 12-36 months following treatment initiation.
CONCLUSION
CONCLUSIONS
Lymphocyte-directed immunomodulation remitted autoimmune pneumonitis in two patients with thymoma.
Identifiants
pubmed: 38954150
doi: 10.1007/s10875-024-01760-3
pii: 10.1007/s10875-024-01760-3
doi:
Substances chimiques
Rituximab
4F4X42SYQ6
Autoantibodies
0
Azathioprine
MRK240IY2L
Types de publication
Journal Article
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
156Informations de copyright
© 2024. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.
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