Table 2.

Reports of vasodilator therapy in sarcoidosis

	No. treated	FVC%*	mPAP * (mmHg)	Treatment (number)	Follow up (mo)	Outcome	Observations
Preston, 2001 [44]	8	48	55	iNO (5) CCB (2)	6–24	5/8 subjects died in 0–18 months	Acute vasodilator response in 7/8 subjects
Culver, 2005 [84]	11	57	76†	EPO (4) EPO + Bos (4) Bos (3)	2–30	8/11 subjects died or required lung transplant Short-term hemodynamic or functional benefit observed in all subjects	Hemodynamic response most evident in patients with FVC ≥70%
Foley, 2005 [85]	1	64	55	Bosentan	24	mPAP decreased to 23 mmHg at six months	Improved functional class (NYHA IV to II)
Sharma, 2005 [86]	1	40	78+	Bosentan	12	Improved six minute walk distance	Improved functional class (NYHA IV to II)
Nunes, 2006 [40]	1	N/A	N/A	Iloprost (1)	4	Died awaiting lung transplant
Baughman, 2006 [29]	7	64	83	EPO (1) EPO + Bos (1) Bos (4) CCB (1)	6	Significant decrease in mPAP after 6 months in 5/7 patients	Immunosupressive therapy was increased
Fisher, 2006 [83]	7	59	57	EPO (7)	0–49	The majority of patients responded to EPO therapy; 4/7 patients alive & without transplant	One episode of pulmonary edema; One sudden death 4 hours after EPO initiation
Milman, 2008 [30]	12	41	36	Sildenafil	1–12	mPAP decreased >20% in 50% of subjects; cardiac output improved in 86%	No benefit on 6 minute walk test

CXR= chest x-ray; FVC=mean forced vital capacity; mPAP= average mean pulmonary artery pressure; iNO=inhaled nitric oxide; CCB=calcium channel blocker; EPO= epoprostenol; Bos=bosentan; NYHA=New York Heart Association

^*Mean or median value for the entire reported population, including those treated with vasodilators

^†Right ventricular systolic pressure estimate by echocardiogram

⁺Systolic pulmonary artery pressure