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Table 1 Defined diagnoses of paediatric inflammatory rheumatic diseases (PiRD) for literature search

From: Biologic disease modifying antirheumatic drugs and Janus kinase inhibitors in paediatric rheumatology – what we know and what we do not know from randomized controlled trials

Indication Population
JIA Polyarticular rheumatoid factor positive/negative JIA (PJIA)
Persistent or extended oligoarticular JIA (OJIA)
Enthesitis-related juvenile idiopathic arthritis and juvenile ankylosing spondylitis, including sacroiliitis (ERA)
Psoriatic juvenile idiopathic arthritis (PsA)
Systemic JIA (SJIA)
Uveitis JIA-associated uveitis
Non-infectious uveitis
Autoinflammatory Diseases Familial Mediterranean Fever (FMF)
TNF receptor-1 associated periodic syndrome (TRAPS)
Cryopyrin-associated periodic syndromes (CAPS)
Mevalonate Kinase Deficiency (MKD)/Hyperimmunoglobulin D syndrome (HIDS)
Unclassified periodic fever syndromes
Chronic recurrent multifocal osteomyelitis (CRMO) and Majeed syndrome
Deficiency of the interleukin-1 receptor antagonist (DIRA)
A20 haploinsufficiency (HA20)
Sideroblastic anemia with B cell immunodeficiency, periodic fevers and developmental delay syndrome (SIFD)
Pyogenic arthritis, pyoderma gangraenosum and acne (PAPA)
Deficiency of the interleukin-36 receptor antagonist (DITRA)
Palmar plantar pustulosis (PPP)
Pyoderma gangraenosum
Interferonopathy Chronic atypical neutrophilic dermatitis with lipodystrophy and elevated temperature (CANDLE)
Stimulator of interferon genes-associated vasculopathy with onset in infancy (SAVI)
Vasculitis Takayasu arteritis
Leucocytoclastic vasculitis
Granulomatosis with polyangiitis (GPA), Wegener’s Granulomatosis
Polyarteritis nodosa
Microscopic polyangiitis (MPA)
Eosinophilic granulomatosis with polyangiitis
Kawasaki Disease (KD)
Behcet disease
Connective Tissue Diseases Systemic Lupus Erythematosus (SLE)
Juvenile Dermatomyositis (JDM)
Paediatric sarcoidosis
Systemic and Localized Scleroderma
Sjögren Syndrome
Mixed connective tissue diseases (MCTD)
Macrophage activation syndrome