Details

Autor(en) / Beteiligte

• Kosinski, Slawomir M.
• Nachajon, Roberto V.
• Milman, Edward

Titel

Rituximab as a single agent for successful treatment of granulomatous and lymphocytic interstitial lung disease in a pediatric patient with common variable immunodeficiency

Ist Teil von

• The journal of allergy and clinical immunology in practice (Cambridge, MA), 2022-03, Vol.10 (3), p.876-878.e1

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2022

Quelle

MEDLINE

Beschreibungen/Notizen

• In deciding on how to manage our patient, we considered the reported reduction of life expectancy and the possibility of progression of pulmonary fibrosis in patients with GLILD.1,5 Our patient had been asymptomatic, but a repeat thoracic HRCT 4 months after a diagnosis showed resolution of some, but also the emergence of new lesions. [...]we decided to start the least aggressive treatment, and we received innovative consent to try RTX alone. Pulmonary functions tests (PFTs) are not considered to have good sensitivity for screening for GLILD. [...]despite finding on 2 occasions a decrease in inspiratory capacity (83% and 69% of predicted), and a decrease in diffusion capacity for carbon monoxide DLco (71% and 73% of predicted), we did not interpret these findings as significant. [...]both these measures completely normalized after the treatment (see Table E2 in this article’s Online Repository at www.jaci-inpractice.org). [...]it may be reasonable to consider that such modest, but reproducible changes in the PFTs in children at risk for GLILD warrant a follow-up with a thoracic HRCT.