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Table 2 Summary of the current management of IL-1 mediated SAIDs

From: Pathophysiology, clinical manifestations and current management of IL-1 mediated monogenic systemic autoinflammatory diseases, a literature review

SAIDs

current management

Recommendation Index

MKD

canakinumab

Recommendation A, approved by the FDA, strong evidence for the effectiveness [10, 11]

anakinra

Recommendation B, canakinumab is unavailable [9]

tocilizumab

Recommendation C, IL-1 blockade fails or is unavailable [13]

etanercept

Recommendation C, IL-1 blockade fails or is unavailable [12]

NSAIDS or glucocorticoids

Recommendation D, mildly affected patients

allogeneic stem cell transplantation

Severest cases [17]

hemodialysis, kidney transplantation

AA-amyloidosis, kidney failure [15]

FMF

Colchicine

Recommendation A, early use after diagnosis [20]

canakinumab

Recommendation B: the second line: colchicine resistance or intolerance to colchicine, approved by the FDA [23]

anakinra

Recommendation C [22]

TRAPS

canakinumab

Recommendation A, approved by the FDA [31]

NSAIDS or glucocorticoids

Mildly affected patients [27]

etanercept

Case report [28]

anakinra

Case report [29]

tocilizumab

Case report [30]

hemodialysis, kidney transplantation

AA-amyloidosis, kidney failure [34]

PAAND

anakinra

Systemic and/or gastrointestinal inflammation [35]

adalimumab

Cutaneous and/or articular manifestations [35]

isotretinoin

Cutaneous component [35]

canakinumab

Systemic and/or gastrointestinal inflammation [35]

colchicine and low-dose prednisolone

Case report [36]

PAPA

NSAIDS or glucocorticoids

Mildly affected patients [41]

etanercept, adalimumab and infliximab

Case report [42]

anakinra

Case report [41]

Canakinumab

Case report [43]

retinoids

Case report [41]

CAPS

anakinra

Approved by the FDA and EMA [49]

canakinumab

Approved by the FDA and EMA [52]

rilonacept

Approved by the FDA [51]

NSAIDS or glucocorticoids

Combination with biological agents [53, 54]

tocilizumab

Case report [55]

DIRA

anakinra

Case report [60, 61]

canakinumab

Case report [62]

rilonacept

Case report [61]

Majeed syndrome

NSAIDS or glucocorticoids

The first-line [70]

anakinra

Case report [67]

canakinumab

Case report [67]

TNF-α blockers or bisphosphonates

Refractory cases [70]

NAIAD

corticosteroids

Case report [72]

acitretin

Case report [71]

etanercept or anakinra

Case report [71]

NLRC4-MAS

IL-1 blockade

The first-line [74]

PFIT

hematopoietic stem cell transplantation

Case report [78]

APIAID

Glucocorticoids

Case report [79]

IVIG

Case report [79]

adalimumab

Case report [79]

IL-1 blockers

Case report [84]