Treatment analysis of patients with iMCD
| Characteristic . | IL-6–targeted therapy* . | Other iMCD therapy† . | Corticosteroid monotherapy . | No treatment . | 
|---|---|---|---|---|
| Total n | 25 | 46 | 99 | 84 | 
| Sex | ||||
| Male | 14 (56.0) | 21 (45.7) | 45 (45.5) | 30 (35.7) | 
| Female | 11 (44.0) | 25 (54.3) | 54 (54.5) | 54 (64.3) | 
| Mean age ± SD, y | 47.9 ± 17.3 | 51.0 ± 15.8 | 53.8 ± 15.7 | 49.8 ± 17.8 | 
| Initial iMCD diagnosis setting | ||||
| Inpatient | 12 (48.0) | 17 (37.0) | 21 (21.2) | 23 (27.4) | 
| Outpatient | 13 (52.0) | 29 (63.0) | 78 (78.8) | 61 (72.6) | 
| Average follow-up after iMCD diagnosis ± SD, y | 2.0 ± 1.6 | 3.4 ± 3.0 | 3.7 ± 3.4 | 1.8 ± 2.1 | 
| Average n of iMCD minor criteria | 4.3 ± 2.0 | 4.2 ± 1.7 | 3.0 ± 1.0 | 3.0 ± 1.3 | 
| Distribution of minor criteria‡ | ||||
| Anemia | 19 (76) | 32 (69.6) | 64 (64.6) | 59 (70.2) | 
| Constitutional symptoms | 21 (84) | 42 (91.3) | 87 (87.9) | 76 (90.5) | 
| Edema and effusion | 17 (68) | 33 (71.7) | 52 (52.5) | 46 (54.8) | 
| Elevated CRP | 1 (4) | 3 (6.5) | 3 (3) | 2 (2.4) | 
| Elevated ESR | 1 (4) | 3 (6.5) | 3 (3) | 5 (6) | 
| Hemangiomatosis | 3 (12) | 4 (8.7) | 10 (10.1) | 6 (7.1) | 
| Hepatosplenomegaly | 12 (48) | 17 (37) | 19 (19.2) | 13 (15.5) | 
| Hypergammaglobulinemia | 2 (8) | 1 (2.2) | 6 (6.1) | 3 (3.6) | 
| Proteinuria | 7 (28) | 12 (26.1) | 9 (9.1) | 8 (9.5) | 
| Renal dysfunction | 12 (48) | 22 (47.8) | 30 (30.3) | 21 (25) | 
| Thrombocytopenia | 10 (40) | 17 (37) | 12 (12.1) | 9 (10.7) | 
| Thrombocytosis | 3 (12) | 7 (15.2) | 3 (3) | 3 (3.6) | 
| LIP | 0 | 0 | 0 | 0 | 
| Characteristic . | IL-6–targeted therapy* . | Other iMCD therapy† . | Corticosteroid monotherapy . | No treatment . | 
|---|---|---|---|---|
| Total n | 25 | 46 | 99 | 84 | 
| Sex | ||||
| Male | 14 (56.0) | 21 (45.7) | 45 (45.5) | 30 (35.7) | 
| Female | 11 (44.0) | 25 (54.3) | 54 (54.5) | 54 (64.3) | 
| Mean age ± SD, y | 47.9 ± 17.3 | 51.0 ± 15.8 | 53.8 ± 15.7 | 49.8 ± 17.8 | 
| Initial iMCD diagnosis setting | ||||
| Inpatient | 12 (48.0) | 17 (37.0) | 21 (21.2) | 23 (27.4) | 
| Outpatient | 13 (52.0) | 29 (63.0) | 78 (78.8) | 61 (72.6) | 
| Average follow-up after iMCD diagnosis ± SD, y | 2.0 ± 1.6 | 3.4 ± 3.0 | 3.7 ± 3.4 | 1.8 ± 2.1 | 
| Average n of iMCD minor criteria | 4.3 ± 2.0 | 4.2 ± 1.7 | 3.0 ± 1.0 | 3.0 ± 1.3 | 
| Distribution of minor criteria‡ | ||||
| Anemia | 19 (76) | 32 (69.6) | 64 (64.6) | 59 (70.2) | 
| Constitutional symptoms | 21 (84) | 42 (91.3) | 87 (87.9) | 76 (90.5) | 
| Edema and effusion | 17 (68) | 33 (71.7) | 52 (52.5) | 46 (54.8) | 
| Elevated CRP | 1 (4) | 3 (6.5) | 3 (3) | 2 (2.4) | 
| Elevated ESR | 1 (4) | 3 (6.5) | 3 (3) | 5 (6) | 
| Hemangiomatosis | 3 (12) | 4 (8.7) | 10 (10.1) | 6 (7.1) | 
| Hepatosplenomegaly | 12 (48) | 17 (37) | 19 (19.2) | 13 (15.5) | 
| Hypergammaglobulinemia | 2 (8) | 1 (2.2) | 6 (6.1) | 3 (3.6) | 
| Proteinuria | 7 (28) | 12 (26.1) | 9 (9.1) | 8 (9.5) | 
| Renal dysfunction | 12 (48) | 22 (47.8) | 30 (30.3) | 21 (25) | 
| Thrombocytopenia | 10 (40) | 17 (37) | 12 (12.1) | 9 (10.7) | 
| Thrombocytosis | 3 (12) | 7 (15.2) | 3 (3) | 3 (3.6) | 
| LIP | 0 | 0 | 0 | 0 | 
Data are presented as n (%) of patients unless otherwise indicated.
CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; LIP, lymphocytic interstitial pneumonitis.
Any duration of siltuximab and/or tocilizumab therapy.
Other iMCD therapy includes immunomodulators, chemotherapy, and rituximab. Patients could have no history of siltuximab or tocilizumab therapy.
There are 11 minor criteria for iMCD, with thrombocytopenia/thrombocytosis and elevated CRP/ESR each counting as a single criterion.