Table 2.

Causes of death in 17 patients with juvenile idiopathic inflammatory myopathies^*

System	Diagnosis	Myositis Autoanti-bodies	Gender/Race	Age at Diagnosis (years)	Disease Duration at Death (years)	Cause of Death
Pulmonary	JCTM†||	none	Female/White	15	0.3	Interstitial lung disease, progressive respiratory failure, acute cardiac failure‡
	JDM	PL-12	Female/White	15	1.4	Respiratory failure, pulmonary fibrosis§
	JDM	PL-12	Female/White	10	5.6	Interstitial lung disease, chronic interstitial pneumonitis‡§
	JCTM||	Ku	Female/White	8	6.6	Interstitial lung disease (diffuse alveolar damage with marked interstitial fibrosis)‡§
	JDM	p155/140	Female/White	12	7.5	Interstitial lung disease, secondary pulmonary hypertension‡
	JCTM¶	Jo-1, Ro, La	Female/Black	17	9.5	Interstitial lung disease‡
	JCTM||	none	Female/Other	5	12.6	Pulmonary hypertension; multiple spontaneous pneumothoraces; secondary scleroderma, severe malnutrition, superior mesenteric artery syndrome‡
Gastrointestinal	JPM	Ro	Male/White	1	0.5	Intestinal perforation‡
	JCTM†||	Sm	Male/Black	15	4.0	Colon perforation resulting in E. coli sepsis‡
	JDM	p155/140	Female/White	7	10.3	Gastrointestinal hemorrhage‡
Multisystem	JPM	none	Female/White	16	0.1	Myocarditis, tachyarrhythmias, respiratory failure, pneumonia‡
	JDM	none	Female/Black	16	2.0	Acute respiratory distress syndrome; sepsis; congestive heart failure; intracranial hemorrhage‡
	JCTM#	none	Female/White	16	3.5	Liver failure, renal failure§
Unknown	JCTM||	PM-Scl	Female/White	16	16.7	Unknown
	JDM	MJ	Female/White	14	31.7	Unknown
	JDM	none	Male/White	10	35.1	Unknown
	JDM	p155/140	Female/White	7	38.5	Unknown

Abbreviations. JDM = juvenile dermatomyositis; JPM = juvenile polymyositis; JCTM = juvenile myositis associated with an underlying connective tissue disease; PL-12=Anti-alanyl-tRNA synthetase autoantibodies; Ku=Anti-Ku autoantibodies; p155/140=Anti-p155/140 autoantibodies; Jo-1=Anti-histidyl tRNA synthetase autoantibodies; Ro=Anti-Ro autoantibodies; La=Anti-La autoantibodies; Sm=Anti-Sm autoantibodies; PM-Scl=Anti-PM-Scl autoantibodies; MJ=Anti-MJ autoantibodies.

^*Patients were diagnosed between 1981 and 2002 and were a median of 18.4 years (25^th, 75^th percentile 16.6, 27.4 years) at the time of death

^†Overlap of JDM and systemic lupus erythematosus.

^‡Information from physician or medical record.

^§Information from death certificate.

^||Overlap of JDM and systemic sclerosis.

^¶Overlap of JPM and Sjögren syndrome.

^#Overlap of JPM and systemic lupus erythematosus.