Systemic juvenile idiopathic arthritis–associated lung disease: A retrospective cohort study

Table 1 Correspondence of our patients to current[10] and provisional International League of Associations for Rheumatology criteria for systemic juvenile arthritis[11]

No. ID	Major criteria			Minor criteria				Overall correspondence ILAR2004 criteria	Overall correspondence ILAR2019 criteria
	Fever	Erythematous rash	Arthritis	Lymphadenopathy/hepatomegaly/splenomegaly	Serositis	Arthralgia	Leukocytosis as/mm3
1	Yes	Yes	No	Yes	Yes	No	53.300	No	Yes
2	Yes	Yes	No	Yes	Yes	Yes	15.100	No	Yes
3	Yes	Yes	Yes	Yes	Yes	Yes	47.200	Yes	Yes
4	Yes	Yes	Yes	Yes	Yes	Yes	30,820	Yes	Yes
5	Yes	Yes	Yes	Yes	Yes	No	2.300	Yes	Yes

ILAR: International League of Associations for Rheumatology.

Table 2 Demographic characteristics of the patients

No.	Sex	Age of onset in yr	Age of last follow-up visit in yr	Time to sJIA-LD	Concomitant disease
1	Male	1	10	3 yr	--
2	Female	2	11	3 yr	--
3	Female	10	17	1 month	--
4	Male	2	11	4 months	Atopic dermatitis
5	Female	2	7	4 yr	Trisomy 21 syndrome

sJIA-LD: Systemic juvenile arthritis with lung diseases.

Table 3 Clinical and radiological characteristics of the patients with systemic juvenile arthritis at the moment of diagnostics of lung involvement

No.	Rash	Hepatitis	Lymphadenopathy	Cough	Dyspnea	Clubbing	Respiratory failure	Infusion reaction on TCZ	PAH	MAS	hScorein points	Heart involvement	X-ray, CT, or MRI or US findings	Eosinophils as × 109/L
1	Yes	Yes	Yes	No	Yes	Yes	Yes	Yes	Yes	Yes	220	Pericarditis	ILD, pleurisy	0.63
2	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes	Yes	209	Myocarditis, Pericarditis	Interstitial lung disease with intralobular foci, pleurisy	0.19
3	Yes	Yes	No	No	Yes	No	No	No	No	Yes	224	N	Alveolitis, diffuse focal lesions, pleurisy	0.29
4	Yes	Yes	Yes	No	Yes	Yes	Yes	Yes	No	Yes	194	Pericarditis	ILD, atelectasis, pleurisy	0.4
5	Yes	Yes	Yes	No	Yes	No	Yes	No	No	Yes	220	Heart Failure	ILD, pleurisy	0.12

CT: Computed tomography; ILD: Interstitial lung disease, MAS: Macrophage activation syndrome; MRI: Magnetic resonance imaging; PAH: Pulmonary arterial hypertension; TCZ: Tocilizumab; US: Ultrasound.

Table 4 Main treatment outcomes of the patients with systemic juvenile arthritis and interstitial lung disease

No.	First biologic	Biologic at the ILD onset	Final therapy	Respiratory symptoms at the last follow-up visit	Dose reduction of non-biologic DMARDs	Dose reduction of BA	Discontinuation of GCS therapy	The outcome of sJIA-LD	The outcome of sJIA
1	TCZ	TCZ	CAN + ABT + CsA + GCS + SDF	No	No	No	No	Progression	Improvement
2	TCM	CAN	CAN + MMF + inhGS	No	Yes	No	Yes	Progression with the following improvement	Remission
3	CAN	CAN	TCZ + CsA	No	No	No	Yes	Improvement	Remission
4	TCZ	TCZ	CAN + MMF	No	No	No	Yes	Improvement	Improvement
5	TCZ	TCZ	CAN + GCS + IVIG	-	-	-	No	Death	Death

ABT: Abatacept; CAN: Canakinumab; CsA: Cyclosporine A; DMARD: Disease-modifying anti-rheumatic drug; GCS: Glucocorticosteroids; inhGCS: Inhaled glucocorticosteroids; IVIG: Intravenous immunoglobulin; ILD: Interstitial lung disease; SDF: Sildenafil; sJIA: Systemic juvenile idiopathic arthritis; TCZ: Tocilizumab.