Acthar Gel for Active Systemic Lupus Erythematosus (SLE)

PGA is a 100 mm visual analogue scale where higher scores indicate more severe disease activity. Lower scores indicate improvement.

BILAG records disease activity occurring over the past 4 weeks, and is used to determine whether different course of treatment is required. The BILAG-2004 index covers 97 signs/symptoms across 9 organ systems. Each question is answered as 0-not present, 1-improving, 2-same, 3-worse, or 4-new.

The BILAG-2004 index categorizes disease activity in each organ system into five different levels from A to E. Grade A represents very active disease, Grade B represents moderate disease activity, Grade C indicates mild stable disease, and grade D implies no disease activity, but suggests the organ system had previously been affected. Grade E indicates no current or previous disease activity. A score is applied to each grade of each organ system using coding scheme of A=12, B=8, C=1, and D/E=0 and is summarized as a total score ranging 0-108. Higher scores indicate more severe disease activity.

The SLEDAI-2K is a modified version of a composite score based on the presence or absence of clinical signs, clinical symptoms, and immunologic laboratory results taken within 10 days of the evaluations. Each of the descriptors has a weighted score and the total score of SLEDAI-2K is the sum of all 24 descriptor scores. The total SLEDAI-2K score falls between 0 and 105, with higher scores representing higher disease activity. Decrease from baseline indicates improvement.

Among some adults, having a period of SLE symptoms-called flares-may happen every so often, sometimes even years apart, and go away at other times-called remission. The SFI categorizes SLE flares as mild, moderate or severe.

The CLASI total activity score reflects ongoing inflammation that can be treated, with points given for the presence of erythema, scale, mucous membrane lesions, recent hair loss, and inflammatory alopecia. Mild, moderate, and severe disease correspond with CLASI activity score ranges of 0 to 9, 10 to 20, and 21 to 70, respectively. Higher scores indicate more disease activity, lower scores indicate improvement.

The 28 Joint Count includes assessment of swelling and tenderness in the shoulders, elbows, wrists, metacarpophalangeal joints, proximal interphalangeal joints and knees. The investigator counts how many of the 28 joints are swollen or tender at the given week.