Table 1.

Studies reporting the occurrence of PAP in sJIA.

Source	Study Design n: Number PAP-sJIA	Frequency PAP-SJIA	Delayed Onset of PAP after the Start of sJIA	Risk Factors	Treatment	Median Follow-Up	Evolution
Schulert (2019) [3]	Prospective study n: 5	6.8%	Not available	Age < 2 years	Anti-IL-1 and anti-IL-6 biologic drugs	12 months	50% improvement associated with disease control
				Tocilizumab reaction
				Early disease onset MAS
				IL-18 elevation
Saper (2019) [10]	Retrospective study n: 61	12.90%	Median time 18 months	Young age	Biological anti cytokine drugs	19 months	Mortality 159/1000 person years
				Anaphylaxis tocilizumab
				Trisomy 21
				Onset MAS at the- beginning of the disease
De Jesus (2020) [13]	Prospective study n: 8	No data	Variable between 6 and 24 months	IL-18 elevation	Anti-IL-1 biologic drugs Ciclosporin Tacrolimus Tofacitinib	24 months	25% death
				IFN positive signature elevation
				Elevated IgE
Chen (2022) [16]	Retrospective study n: 24	No data	No data	IL-18 elevation	No data	No data	No data
				ICAM5 elevation
				MMP7 elevation
				CCL11 elevation
				Eotaxin1 elevation
Canna (2019) [14]	3 cases reports n: 3	No data	12 months	Elevation IL-18	JAK inhibitor Emapalumab	No data	No data
				Age of onset sJIA < 2 years
				MAS onset
				Anaphylactic reaction biological drug
Kimura (2013) [8]	Retrospective study n: 5	1.28%	No data	MAS	DMARD blocking IL1- IL6- TNF alpha Corticoids Cyclophosphamide	No data	No data
Rood (2022) [15]	1 case report n: 1	No data	6 years	IL-18 elevation	Anti IL1 biologic-drugs Methotrexate Cyclophosphamide Azathioprine MAS 825 (biological anti IL1B- anti IL18)	40 weeks	Removal of pulmonary lesions Improvement of clinical signs and complete regression of the inflammatory syndrome
				MAS