Použití CXCL9 jako biomarkeru Acthar účinnosti (Acthar)

Experimentální: Gel Acthar

Po 4týdenním období základního sledování bude Acthar gel podáván intramuskulární nebo subkutánní injekcí. Počáteční dávkování bude 40 U každých 72 hodin (nebo dvakrát týdně) po dobu 4 týdnů. Dávka se poté zvýší na 80 U s podobnou frekvencí po dobu 8 týdnů až 16 týdnů.

Lék: Gel Acthar

Acthar gel podávaný IM nebo SQ podle příbalového letáku

Ostatní jména:

• Acthar

Number of Participants That Show a Decrease in Blood CXCL9 Levels by 50%

Quantitative polymerase chain reaction assay was used to measure the gene expression for the gene CXCL9 using whole blood samples. The change in cycle threshold from before starting medication to the end of treatment was calculated. From this calculation, the number of participants that met the criteria of a 50% or greater drop in CXCL9 levels is reported.

Participants With Improvement in Forced Vital Capacity (FVC) Liters by 5 Percent or More

Participants With Improvement in Dyspnea Score

The University of California San Diego Shortness of Breath Questionnaire (UCSD SOBQ) is a 24-item patient-reported outcome measure that assesses the severity of dyspnea during activities of daily living. Participants rate shortness of breath for each activity on a 6-point scale from 0 ("not at all") to 5 ("maximally or unable to do because of breathlessness"). Item scores are summed to generate a total score ranging from 0 to 120. Higher total scores represent worse dyspnea severity, while lower scores indicate less dyspnea. The SOBQ does not contain separate subscales; all 24 items are equally weighted and combined by simple summation to calculate the total score. If a participant does not routinely perform an activity, they are instructed to estimate anticipated dyspnea, minimizing missing data.