Abatacept Use Is Associated with Steroid Dose Reduction and Improvement in Fatigue and CD4-Dysregulation in CVID Patients with Interstitial Lung Disease
| Autor: | Klaus Warnatz, Sigune Goldacker, Sylvia Gutenberger, Constanze Echternach, Caroline von Spee-Mayer, Veronika Soetedjo, Prerana Agarwal |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
medicine.medical_specialty
Pilot Projects medicine.disease_cause Gastroenterology Abatacept 03 medical and health sciences 0302 clinical medicine Diffusing capacity Internal medicine medicine Clinical endpoint Immunology and Allergy Humans 030212 general & internal medicine Prospective Studies Fatigue Lung Drug Tapering business.industry Common variable immunodeficiency Interstitial lung disease Immune dysregulation medicine.disease medicine.anatomical_structure Common Variable Immunodeficiency 030228 respiratory system Rheumatoid arthritis Quality of Life Steroids business Lung Diseases Interstitial medicine.drug |
| Zdroj: | The journal of allergy and clinical immunology. In practice. 9(2) |
| ISSN: | 2213-2201 |
| Popis: | Background Interstitial lung disease (ILD) represents a severe clinical manifestation of systemic immune dysregulation in patients with common variable immunodeficiency (CVID). Its treatment often requires systemic immunosuppression beyond corticosteroids. Objective To assess the safety and efficacy of abatacept in patients with CVID and ILD. Methods Ten patients with confirmed diagnosis of CVID and ILD were included in a single-center, prospective, open-label, nonrandomized trial. Abatacept was administered subcutaneously at a dose of 125 mg/wk for 12 months. Results Abatacept was a safe treatment for ILD in CVID except for 1 case of bronchopulmonary aspergillosis. One additional patient terminated the trial prematurely because of recurrent bronchitis. Five of 8 patients treated per protocol benefited from the treatment according to American Thoracic Society/European Respiratory Society criteria. The primary end point of the study was met because single breath diffusing capacity of the lung for carbon monoxide was stable (62.5%) or improved (37.5%) in all patients treated per protocol. Although nodules (71%) and ground-glass opacities (57%) improved in most patients, other computed tomography pathologies were less responsive. Quality of life improved in 87.5% and fatigue in 57% of patients. Abatacept treatment was associated with significant improvement in CD4 T-cell dysregulation, signified by a decrease in serum soluble IL-2 receptor levels and of proliferating Ki67+ CD4 T cells, and a recovery of total lymphocytes, CD4+ T cells, and naive CD4 T cells. Conclusions Abatacept may represent a treatment option for CVID-associated ILD. This pilot study demonstrated a good safety profile, steroid-sparing effect, positive immune modulation, and overall positive treatment response especially in quality of life. Larger controlled studies are needed to confirm these findings. |
| Databáze: | OpenAIRE |
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