Collagen vascular disease-associated interstitial lung disease

Table 1 Summary of key features of collagen vascular-interstitial lung disease

	Prevalence	Histology	Other pulmonary findings	Treatment
Scleroderma	> 70%	NSIP	Pulmonary arterial hypertension	1Cyclophosphamide[22,29]
				Mycophenolate, azathioprine used for maintenance
Dermatomyositis/Polymyositis	20%-30%	NSIP	Diaphragmatic weakness	Corticosteroids
				Azathioprine
				Mycophenolate
				Calcineurin inhibitors
				Rituximab
				IVIG
Mixed connective tissue disease	30%-60%	NSIP	Diaphragmatic weakness	Corticosteroids ± cyclophosphamide
Sjogren syndrome	25%-40%	NSIP, UIP, OP, LIP		Corticosteroids ± cyclophosphamide
Rheumatoid arthritis	10%-20%	UIP	May have obstructive lung disease,	Corticosteroids ± cyclophosphamide or azathioprine
			necrobiotic nodules, pulmonary arterial hypertension
Systemic lupus erythematosus	5%	NSIP, OP	Pulmonary arterial hypertension,	Corticosteroids ± cyclophosphamide or azathioprine
			Pleural effusion
ANCA-associated vasculitis	7%-47%	UIP	Pulmonary hemorrhage	Induction with corticosteroids + cyclophosphamide or rituximab[84-86]
				Maintenance with methotrexate or azathioprine
				Plasmapheresis for pulmonary hemorrhage

¹Use in collagen vascular disease-associated- interstitial lung disease supported by multicenter clinical trials.