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- Title
Improvement of Refractory Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease with Single-Agent Blockade of IL-1β and IL-18.
- Authors
Rood, Julia E.; Rezk, Ayman; Pogoriler, Jennifer; Finn, Laura S.; Burnham, Jon M.; Josephson, Maureen B.; Bar-Or, Amit; Behrens, Edward M.; Canna, Scott W.
- Abstract
Systemic juvenile idiopathic arthritis associated with interstitial lung disease (SJIA-LD) represents a highly morbid subset of SJIA for which effective therapies are lacking. We report the case of a patient with refractory SJIA-LD who underwent treatment with MAS-825, an investigational bispecific monoclonal antibody targeting IL-1β and IL-18. MAS-825 treatment was associated with a marked reduction in total IL-18 and free IL-18 in both serum and bronchoalveolar lavage fluid (BAL). Baseline oxygen saturation, exercise tolerance, and quality of life metrics improved after treatment with MAS-825, while pulmonary function testing remained stable. Following treatment, the BAL showed no evidence of pulmonary alveolar proteinosis and inflammatory infiltrates were markedly reduced, reflected by decreased numbers of CD4 T-cells, CD8 T-cells, and macrophages. The patient was able to wean entirely off systemic corticosteroids and other biologics after 10 months of treatment with MAS-825 and experienced no side effects of the drug. This case demonstrates improvement in pulmonary symptoms, lung inflammation, and burden of immunomodulatory therapy after treatment with MAS-825 and suggests that simultaneous targeting of both IL-1β and IL-18 may be a safe and effective treatment strategy in SJIA-LD.
- Subjects
INTERSTITIAL lung diseases; BISPECIFIC antibodies; PULMONARY alveolar proteinosis; JUVENILE idiopathic arthritis; LUNG diseases; EXERCISE tolerance
- Publication
Journal of Clinical Immunology, 2023, Vol 43, Issue 1, p101
- ISSN
0271-9142
- Publication type
Article
- DOI
10.1007/s10875-022-01353-y