Table 1.

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

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
Psoriasis