| ALL | Acute lymphoblastic leukemia |
| ADA2 | Adenosine deaminase 2 |
| AOSD | Adult-onset Still’s disease |
| AGEs | Advanced glycation end products |
| AHSP | Alpha hemoglobin-stabilizing protein |
| ACR | American College of Rheumatology |
| ACR 50 | American College of Rheumatology score = at least 50% improvement in disease activity |
| ANK | Anakinra |
| ANAs | Antinuclear antibodies |
| Arg-1 | Arginase-1 |
| AST | Aspartate aminotransferase |
| ADs | Autoimmune diseases |
| AID | Autoinflammatory disease |
| bDMARDs | Biological disease-modifying anti-rheumatic drugs |
| S100A12 | Calgranulin C |
| CP, or S100A8/A9, or MRP8/14 | Calprotectin |
| CAN | Canakinumab |
| CASP8 | Caspase-8 |
| CCL23 | C-C motif chemokine ligand 23 |
| CDC | Centers for Disease Control and Prevention |
| CNS | Central nervous system |
| CXCL1…11 | Chemokine (C-X-C motif) ligand 1…11 |
| CCL2 | Chemokine (C-C motif) ligand 2 |
| CXCL9 | Chemokine (C-X-C motif) ligand 9 |
| CXCL10 | Chemokine (C-X-C motif) ligand 10 |
| CX3CR1 | Chemokine (C-X3-C motif) receptor 1 |
| CID | Clinically inactive disease |
| CD | Cluster of differentiation |
| CD163 | Cluster of differentiation 163 |
| CLPs | Common lymphoid progenitors |
| CBC | Complete blood count |
| CTD | Connective tissue disease |
| cNK | Conventional NK |
| csDMARDs | Conventional synthetic disease-modifying anti-rheumatic drugs |
| CS | Corticosteroid |
| CRP | C-reactive protein |
| CSS | Cytokine storm syndrome |
| DAMP | Damage-associated molecular pattern |
| DNA | Deoxyribonucleic acid |
| DEGs | Differentially expressed genes |
| DMARDs | Disease-modifying anti-rheumatic drugs |
| EHEC | Enterohemorrhagic Escherichia coli |
| ELISA | Enzyme-linked immunosorbent assay |
| EBV | Epstein–Barr virus |
| EBV-HLH | Epstein–Barr-virus-induced hemophagocytic lymphohistiocytosis |
| ESR | Erythrocyte sedimentation rate |
| fHLH | Familial hemophagocytic lymphohistiocytosis |
| FMF | Familial Mediterranean fever |
| FasL | Fas ligand |
| FUO | Fever of unknown origin |
| Fg | Fibrinogen |
| FSTL-1 | Follistatin-like protein 1 |
| FDA | Food and Drug Administration |
| CFA | Freund’s complete adjuvant |
| GEO | Gene Expression Omnibus |
| G-CSF | Granulocyte colony-stimulating factor |
| GM-CSF | Granulocyte–macrophage colony-stimulating factor |
| GZMK | Granzyme K |
| HCs | Healthy controls |
| HSC | Hematopoietic stem cell |
| HLH | Hemophagocytic lymphohistiocytosis |
| HMGB1 | High-mobility group box-1 |
| hsCRP | High-sensitivity C-reactive protein |
| HLA | Human leukocyte antigen |
| Igs | Immunoglobulins |
| ILCs | Innate lymphoid cells |
| ILC1 | “Unconventional” NK cells |
| ICAM-5 | Intercellular adhesion molecule 5 |
| IFN-α | Interferon-alpha |
| IFN-γ | Interferon-gamma |
| IRF5 | Interferon regulatory factor 5 |
| IL-1, IL-6, IL-18 | Interleukin 1, 6, 18 |
| IL-18Rβ | IL-18β receptor |
| IL-1 alpha, IL-1α; IL-1 beta, IL-1β | Interleukin 1 alpha, beta |
| IL-1Ra | Interleukin-1 receptor antagonist |
| IL10RA | Interleukin-10 receptor, alpha subunit |
| IL6ST | Interleukin-6 signal transducer |
| IL-18BP | IL-18-binding protein |
| ICAM-1 | Intracellular adhesion molecule 1 |
| IV | Intravenous |
| JDM | Juvenile dermatomyositis |
| JIA | Juvenile idiopathic arthritis |
| JADAS | Juvenile Arthritis Disease Activity Score |
| KD | Kawasaki disease |
| LDH | Lactate dehydrogenase |
| LRG | Leucine-rich α2-glycoprotein |
| LRG1 | Leucine-rich α-2 glycoprotein 1 |
| LPSs | Lipopolysaccharides |
| CD62L | L-selectin |
| LCLs | Lymphoblastoid cell lines |
| M | Macrophage |
| M1 | M1 macrophages (encourage inflammation) |
| M2 | M2 macrophages (decrease inflammation and encourage tissue repair) |
| MAS | Macrophage activation syndrome |
| sJIA-MAS | Macrophage activation syndrome in sJIA |
| MIF | Macrophage migration inhibitory factor |
| MHC | Major histocompatibility complex |
| MMP1 | Matrix metalloproteinase 1 |
| MMP3 | Matrix metalloproteinase 3 |
| MEFV | Mediterranean fever |
| MTX | Methotrexate |
| MAPKs | Mitogen-activated protein kinases |
| MCP1 | Monocyte chemoattractant protein 1 |
| MIS-C | Multisystem inflammatory syndrome in children |
| MPO–DNA complexes | Myeloperoxidase–DNA complexes |
| MRP | Myeloid-related protein |
| MRP8 | Myeloid-related protein 8 |
| MRP14 | Myeloid-related protein 14 |
| NCR2 or NKp44 | Natural cytotoxicity-triggering receptor 2 |
| NK | Natural killer |
| NKT | Natural killer T |
| NCAM or CD56 | Neural cell adhesion molecule, also called CD56 |
| NETs | Neutrophil extracellular traps |
| NETosis | NET activation and release |
| NLR | NOD-like receptor |
| NSAIDs | Non-steroidal anti-inflammatory drugs |
| nsJIA | Non-systemic forms of JIA |
| NFIL3 | Nuclear factor, interleukin 3-regulated gene |
| NF-κB or NF-kappaB | Nuclear factor kappa-light-chain-enhancer of activated B cells |
| NOD | Nucleotide-binding oligomerization |
| NLRP | Nucleotide-binding oligomerization domain, leucine-rich repeat and Pyrin domain |
| NLRP3 | Nucleotide-binding oligomerization domain-like receptor pyrin domain-containing protein 3 |
| NLRs | Nucleotide-binding oligomerization domain-like receptors = NOD-like receptors |
| No. | Number; Latin “numero”, the ablative case of “numerus” |
| PAMP | Pathogen-associated molecular pattern |
| PRRs | Pattern-recognition receptors |
| PRINTO | Pediatric Rheumatology International Trials Organization |
| PRF1 | Perforin 1 |
| PBMC | Peripheral blood mononuclear cell |
| PMLs or PMNLs | Polymorphonuclear leukocytes |
| PMN | Polymorphonuclear neutrophil |
| + | Present |
| ± | Present/absent |
| pHLH | Primary hemophagocytic lymphohistiocytosis |
| PCT | Procalcitonin |
| PD-L1 | Programmed death ligand-1 |
| PKC | Protein kinase C |
| qRT-PCR | Quantitative reverse-transcription polymerase chain reaction |
| ROS | Reactive oxygen species |
| TNFSF11 | Receptor activator of nuclear factor-κB ligand, RANKL |
| RAGE | Receptor for advanced glycation end products |
| rIL-1Ra | Recombinant IL-1 receptor antagonist |
| rr | Reference range |
| R | Response |
| RMD | Rheumatic and musculoskeletal disease |
| RF | Rheumatoid factor |
| S100A9 | S100 calcium-binding protein A9 |
| MS score | Score for sJIA-MAS |
| sec-HLH | Secondary hemophagocytic lymphohistiocytosis |
| SAA | Serum amyloid A |
| sCP | Serum calprotectin |
| sPCT | Serum procalcitonin |
| SIF | Severe infections |
| SNP | Single-nucleotide polymorphism |
| sCD163 | Soluble cluster of differentiation 163 |
| sCD25 or sIL2RA | Soluble IL-2 receptor alpha chain, or soluble IL2RA |
| sRAGE | Soluble receptor for advanced glycation end products |
| sTNFR-I | Soluble tumor necrosis factor receptor I |
| sTNFR-II | Soluble tumor necrosis factor receptor II |
| sTNF-R II/I | Soluble TNF receptor II/I |
| SF | Synovial fluid |
| sJIA | Systemic juvenile idiopathic arthritis |
| SLE | Systemic lupus erythematosus |
| TCR | T-cell receptor |
| Th cells, CD4+ or CD4-positive | T-helper cells, or CD4+ cells, or CD4-positive cells |
| trNK | Tissue-resident (tr)NK |
| TWEAK | TNF-like weak inducer of apoptosis |
| TCZ | Tocilizumab |
| TLR | Toll-like receptor |
| TGF-β | Transforming growth factor beta |
| TAMs | Tumor-associated macrophages |
| TNF-α | Tumor necrosis factor alpha |
| TNFR1 | Tumor necrosis factor receptor 1 |
| FcγRIII | Type III Fcγ receptor |
| WGCNA | Weighted gene co-expression network analysis |
| WT | Wild type |
| Increasing/high | ↑ |
| Very high | ↑↑ |
| Decreasing/low | ↓ |
| Very low | ↓↓ |