Table 2.
The immune deficiency and dysregulation activity (IDDA2.1) score
| Parameters (for grading 0–4°, see below1; line titles serve as examples) | |
|---|---|
| 1 | Autoimmune (AI) cytopenia |
| 2 | Hemophagocytosis | HLH (according to clinical AND lab criteria of the HS) |
| 3 | Enteropathy | IBD |
| 4 | Lymphoproliferation | splenomegaly | hepatomegaly |
| 5 | Parenchymal lung disease | LIP | GLILD |
| 6 | Skin or eye manifestations | eczema, uveitis, alopecia, vitiligo, other |
| 7 | Granulomatous disease in any organ (except GLILD) |
| 8 | Endocrinopathy | IDDM, thyreoiditis, other |
| 9 | Arthritis | other musculoskeletal |
| 10 | AI-hepatitis | cholangitis | pancreatitis |
| 11 | Glomerulonephritis | nephropathy, tubulopathy |
| 12 | Neurologic manifestations of immune dysregulation | CNS autoimmunity, inflammation, vasculitis |
| 13 | Failure to thrive | malresorption, wasting |
| 14 | Severe infections | opportunistic (excl. chronic infestation, see below) |
| Other factors and symptoms (will multiply or add to the IDDAscore)2 | |
| 15 | Karnovsky / Lansky Performance Scale (%) |
| 16 |
Hospitalization (days out of 100 days; including day clinic stays, excl. intensive care unit [ICU]) |
| 17 |
Mechanical ventilation or other ICU measures (days out of 100 days; except elective procedures) |
| 18 | Immunoglobulin substitution therapy | hypogammaglobulinemia |
| 19 | Any relevant chronic or recurring infestation/infection (e.g., Norovirus, EBV…) |
| 20 | Any other organ dysfunction / malady (e.g., cardiomyopathy, kidney failure, allergies…) |
| 21 | Nutrition / dietary status and habits |
| 22 | Malignancy (separately noted, not added to numeric score) |
|
Formula for IDDA2.1 score total (Excel® format) = (SUM(line1:line14) + IF(line16 < 40;line16*0.1;4) + IF(line17 < 10;line17*0.8;8) + SUM(line18:line21))*IF(line15 > 29;150/line15;6) | |
1, grading for lines 1–14: 0, absent; 1, mild, transient, not requiring treatment; 2, moderate, intermittent therapy needed; 3, severe, continuous therapy needed; 4, life-threatening, refractory, irreversible
2, lines 15–17 are percentages, lines 18–21 are scored as follows: line 18 (0, no; 2, sporadic; 3[iv], regularly IVIG; 3[sc], regularly SCIG); line 19 (0, no; 1, asymptomatic infestation; 2, oligosymptomatic recurring infection; 3, recurring symptomatic infection requiring on/off treatment; 4, chronic infection requiring permanent treatment or refractory infection, only score worst if more than one microbial agents are relevant); line 20 (e.g., hepatopathy, cardiomyopathy, kidney failure; please quantify if possible: 0, no organopathy; 1, mild transient dysfunction; 2, chronic mild dysfunction; 3, moderate-severe dysfunction; 4, clinically compromising dysfunction requiring treatment or replacement therapy, only score worst if more than one organ is involved); line 21 (0, normal; 1, modified disease-adjusted; 2, part-formula medically advised; 3, tube-feeding and/or full-formula or partial parenteral nutrition (irregularly); 4, total parenteral nutrition)