Organ response. (A) Overall organ response. A cardiac response was defined as either a reduction in the NT-proBNP of >30% and at least a 300 pg/mL decrease from the baseline result if the NT-proBNP was ≥650 pg/mL at baseline, or improvement in New York Heart Association (NYHA) class by 1 class if NYHA class 1 or 2, and a 2-class improvement if NYHA class 3 at baseline.28 A renal response was defined as either a 30% decrease in urine total albumin or a decrease to <500 mg per 24 hours urine total protein without renal progression.30 A gastrointestinal response was defined as either a reduction in 24-hour fecal fat excretion by ≥50% in patients with steatorrhea or a ≥50% reduction in the number of loose stools per day in patients with diarrhea. A liver response was defined as either a 50% decrease in or normalization of the alkaline phosphatase level or a reduction in the span of the liver by at least 2 cm by radiographic determination. Of the 25 patients with cardiac involvement, 14 had a baseline NT-proBNP ≥650 pg/mL, and were evaluable for cardiac response based upon the NT-proBNP. One patient with an NT-proBNP <650 pg/mL was classified as a response based upon improvement in NYHA class. This patient is not depicted in the graph. (B) Graded cardiac/renal organ response. Cardiac and renal responses were retrospectively calculated. One patient with a renal response was not evaluable for grading given a baseline 24-hour proteinuria level <500 mg per 24 hours. The 14 patients with a NT-proBNP ≥650 pg/mL were evaluable for graded cardiac responses.33,34
