Table 2 sHLH/MAS therapeutics reported in the literature.
Target | Drugs | Combined drugs | Condition | Trigger | Clinical and laboratory criteria | Results | Ref. |
|---|---|---|---|---|---|---|---|
IL-1 | Anakinra | Methylprednisolone, Ciclosporin, Prednisolone | sJIA | Methylprednisolone | ↑Fever, ↑Splenomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑Fibrinogen, ↑sCD25 | Ferritin/PLT/AST/Fibrinogen normal range | |
IVIG, Aspirin, Infliximab, Methylprednisolone, Prednisolone | KD, HLH | Methylprednisolone, Prednisolone | ↑Ferritin, ↑WBC, ↑PLT, ↑AST, ↑ALT, ↑TG | Inflammatory marker recovery | |||
Methylprednisolone, Ciclosporin, Prednisolone, Dexamethasone | AOSD, HLH | Methylprednisolone | ↑Fever, ↑Hepatomegaly, Cervical lymphadenopathy, ↑Neutrophilic leukocytosis, ↑Ferritin, ↑AST, ↑TG, ↑Hematopoietic cells | Ferritin/Inflammatory marker improvement | |||
Methylprednisolone, Corticosteroids, Tocilizumab | AOSD, HLH | Anakinra (100 mg/day), Methylprednisolone, Tocilizumab | ↑Fever, ↑Hepatomegaly, ↑Splenomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑ALT, ↓Leucocyte count, lymphopenia, ↑Hematopoietic cells | Ferritin/systemic inflammation and cytolysis improvement | |||
Methylprednisolone, Infliximab, Prednisolone, MTX, Dexamethasone | sJIA | Infliximab, Prednisolone, MTX | ↑Fever, ↑Splenomegaly, ↑Ferritin, ↓PLT | Ferritin/WBC/PLT normal range | |||
Prednisone, Adalimumab | axSpA | Adalimumab | ↑Fever, ↑Splenomegaly, Lymphopenia, ↓PLT, ↑AST, ↑ALT, ↑Ferritin | ESR/CRP/ferritin/fibrinogen/inflammatory marker improvement | |||
Canakinumab | Prednisone, Methylprednisolone, Anakinra, Cyclosporine A | sJIA | Prednisone, Methylprednisolone, Anakinra | ↑Fever, ↑Ferritin, ↑PLT, ↑AST, ↑TG, ↑Fibrinogen | Ferritin decrease, AST normal range | ||
Methylprednisolone, Glucocorticoid, Cyclosporine A, Anakinra | sJIA | Prednisone, Thrombophlebitis | ↑Fever, ↑Ferritin, ↓PLT, ↑AST, ↑ALT, ↑TG, ↓Fibrinogen | Improvements in all MAS biomarkers | |||
IL-2 | Daclizumab | Corticosteroids | HLH | - | ↑Fever, ↑Ferritin, ↓WBC, ↓PLT, ↑AST, ↑ALT | Fever/Ferritin normal range | |
IL-6 | Tocilizumab | Methotrexate, Prednisolone, Cyclosporine, Omalizumab, Anakinra | AOSD, HLH | EBV, Dexamethasone | ↑Fever, ↑Ferritin, ↑Leukocytosis, ↓PLT, ↑AST, ↑TG, ↓ Fibrinogen | AOSD and idiopathic urticaria nonrecurrence | |
Prednisolone, Ganciclovir, Sulfamethoxazole, Trimethoprim | AOSD | Prednisolone, Sulfamethoxazole, Trimethoprim, CMV | ↑Fever, ↑Hepatomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑ALT, ↑TG, ↓ Fibrinogen | Fever/skin rash improvement | |||
IFN-γ | Emapalumab | Tocilizumab, Anakinra, Prednisolone, Corticosteroids, Alemtuzumab | sJIA | Parainfluenza 3 virus, Anakinra, | ↑Fever, ↑Hepatomegaly, ↑Splenomegaly, ↑Ferritin, ↓WBC, ↓PLT, ↑AST, ↑ALT, ↑TG, ↑sCD25 | Inflammatory marker/Ferritin/CXCL9 improvement | |
Prednisolone, Methylprednisolone, Anakinra, Corticosteroids, Dexamethasone, Acyclovir, Fluconazole | AOSD | Prednisolone, Methylprednisolone, Anakinra | ↑Fever, ↑Splenomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑ALT, ↑TG, ↑Fibrinogen, ↑sCD25 | Ferritin/PLT/Fibrinogen/TG improvement | |||
Etoposide, Dexamethasone, Rituximab, IVIG | HLH | EBV | ↑Fever, ↑Hepatomegaly, ↑Splenomegaly, ↓WBC, ↓PLT, ↑Ferritin, ↓Fibrinogen, ↑sCD25, | Clinical symptom/ Ferritin/sCD25 level improvement | |||
Canakinumab, Sulfamethoxazole Methylprednisolone, Trimethoprim | sJIA | EBV | ↑Fever, ↓PLT, Neutropenia, Lymphopenia, ↑Ferritin, ↑AST, ↓Fibrinogen, | Cytopenia/hypofibrinogenemia improvement | |||
TNF-α | Etanercept | Ibuprofen, Solumedrol, Steroid, Prednisolone | sJIA | Ibuprofen | ↑Fever, ↑Hepatomegaly, ↑Splenomegaly, ↑WBC, ↓PLT, ↑Ferritin, ↓PLT, ↑TG, ↓ Fibrinogen | Fever/Chest pain/Rash improvement | |
Etoposide, Methylprednisolone, Prednisolone, Tacrolimus, MTX, Infliximab | AOSD | Prednisolone | ↑Fever, ↑Ferritin, ↑WBC, ↓PLT, ↑AST, ↑TG, ↑Fibrinogen, ↑sCD25 | Fever and polyarthralgia improvement | |||
Infliximab | IVIG, Acetylsalicylic acid, Methylprednisolone, Cyclosporine A | KD | IVIG, Acetylsalicylic acid | ↑Fever, ↑Hepatomegaly, ↑Splenomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑TG, ↓ Fibrinogen | Hepatomegaly/Splenomegaly/Clinical and Biochemical parameter improvement | ||
Dexamethasone, Etoposide, Infliximab, Daclizumab, | HLH | EBV, CMV, HHV6 | ↑Fever, ↑Hepatomegaly, ↑Splenomegaly, ↓WBC, ↓PLT, ↑Ferritin, ↑sCD25, ↑TG, ↑AST, ↑ALT, | Clinical symptom improvement | |||
JAK 1/2 | Ruxolitinib | Prednisolone, Tocilizumab Methylprednisolone, Anakinra, | AOSD | – | ↑Fever, ↑Hepatomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑TG, ↓ Fibrinogen | Blood counts/Ferritin/CD25 levels normal range | |
Baricitinib | Glucocorticoid, Immunoglobulin, Cyclophosphamide, MTX, Prednisolone | Nodular panniculitis, HLH | Prednisolone | ↑Fever, ↑Hepatomegaly, ↑Splenomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑TG, ↓ Fibrinogen, ↑sCD25 | Abnormal laboratory all improvement | ||
CD20 | Rituximab | Metronidazole, Etoposide, Cyclosporine, Dexamethasone | SLE | – | ↑Fever, ↑Splenomegaly, ↓WBC, ↓PLT, ↑Ferritin, ↓PLT, ↑AST, ↑TG, ↓ Fibrinogen | Blood counts/Ferritin/TG all normal range | |
Methylprednisolone, Prednisone, Tacrolimus, Cyclosporine A | DM | Methylprednisolone | ↑Fever, ↑AST, ↑ALT, ↓WBC, ↓PLT, ↑Ferritin | Pancytopenia improvement | |||
Methylprednisolone, Immunoglobulin, rituximab, Prednisolone | XLP | EBV | ↑Fever, ↑Hepatomegaly, ↑Splenomegaly ↑WBC, ↑AST | Clinical symptom improvement | |||
CD28 | Abatacept | Anakinra, MTX, Corticosteroids, Methylprednisolone, IVIG, Cyclosporine | sJIA | Anakinra, Corticosteroids | Development of chronic low-grade MAS with concurrent cutaneous vasculitis | Fever improvement, ESR normal range |
