Table 4.

Treatment analysis of patients with iMCD

CharacteristicIL-6–targeted therapy*Other iMCD therapyCorticosteroid monotherapyNo 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 
CharacteristicIL-6–targeted therapy*Other iMCD therapyCorticosteroid monotherapyNo 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 

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.

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