Skip to main content

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

 

Psoriasis