Table 3.

Clinical features at pulmonary disease diagnosis

Total N: 25
Age at start of pulmonary symptoms (years)*	11.7 ± 5.2 (3.5–18.8)
Disease duration at pulmonary diagnosis (months [mos])*	50.6 ± 44.6 (8–160)
Time between pulmonary symptoms to diagnosis (mos)*	3.1 ± 3.2 (0–10)
Time between pulmonary diagnosis to death (mos) (N=17)*	10.2 ± 13 (0–44)
Disease features at pulmonary disease diagnosis	N (%)
sJIA manifestations
Any systemic manifestation**	23 (92)
MAS (suspected or confirmed)	15 (60)
Pericarditis/serositis	11 (44)
Thrombotic thrombocytopenic purpura	1 (4)
Arthritis	16 (64)
Pulmonary symptoms
Shortness of breath	16 (64)
Dyspnea on exertion	18 (72)
Cough	11 (44)
Clubbing	10 (40)
Chest pain	5 (20)
Pulmonary diagnosis
Pulmonary artery hypertension	16 (64)
Alveolar proteinosis	5 (20)
Interstitial lung disease	7 (28)

^*Mean ± SD (range)

^**Includes patients with one or more of the following: fever, rash, lymphadenopathy, hepatomegaly, splenomegaly