Long-term treatment with human immunoglobulin for antisynthetase syndrome-associated interstitial lung disease. - 2019

BACKGROUND: Interstitial lung disease-associated antisynthetase syndrome (AS-ILD) carries significant morbidity and mortality. Corticosteroids and immunosuppressive drugs are the mainstay of treatment. Human immunoglobulin (IVIg), an immunomodulator without immunosuppressive properties, is effective in myositis but the evidence supporting its use in ILD is scarce. CONCLUSIONS: IVIg may be a useful complementary therapy in active progressive AS-ILD but is associated with potential side effects. Fssssurther investigation is required to determine the value of IVIg as an initial treatment in AS-ILD. Copyright (c) 2019. Published by Elsevier Ltd. METHODS: Retrospective analysis of AS-ILD patients. Linear mixed models using restricted maximum likelihood estimation was used to estimate the change in lung function and corticosteroid dose over time. OBJECTIVE: To describe clinical outcomes of AS-ILD patients receiving IVIg. RESULTS: Data from 17 patients was analyzed. Median follow-up was 24.6 months. Fourteen patients had refractory disease. The mean percent-predicted forced vital capacity (FVC%) (p=0.048) and percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%) (p=0.0223) increased over time, while the mean prednisone dose (p<0.001) decreased over time. Seven patients achieved a >10% increase in FVC%, including two who used IVIg as initial treatment. Five patients showed a >10% increase in DLCO% and TLC%. Nine (53%) patients experienced side effects.


English

0954-6111

10.1016/j.rmed.2019.05.012 [doi] S0954-6111(19)30164-7 [pii]


*Immunoglobulins, Intravenous/tu [Therapeutic Use]
*Immunologic Factors/tu [Therapeutic Use]
*Lung Diseases, Interstitial/th [Therapy]
*Myositis/th [Therapy]
Administration, Intravenous
Adrenal Cortex Hormones/tu [Therapeutic Use]
Adult
Aged
Carbon Monoxide/me [Metabolism]
Female
Follow-Up Studies
Humans
Immunoglobulins, Intravenous/ae [Adverse Effects]
Immunosuppressive Agents/tu [Therapeutic Use]
Lung Diseases, Interstitial/co [Complications]
Lung Diseases, Interstitial/mo [Mortality]
Lung/pp [Physiopathology]
Male
Middle Aged
Myositis/co [Complications]
Myositis/mo [Mortality]
Prednisone/tu [Therapeutic Use]
Pulmonary Diffusing Capacity/de [Drug Effects]
Retrospective Studies
Treatment Outcome
Vital Capacity/de [Drug Effects]


MedStar Washington Hospital Center


Medicine/Internal Medicine


Journal Article