sildenafil-citrate and Sarcoidosis

Development of sarcoidosis-associated pulmonary hypertension (SAPH) significantly worsens prognosis in sarcoidosis patients. Unfortunately, there is no treatment of proven benefit for this condition. Medications used for treatment of pulmonary arterial hypertension are of great interest in this respect. Here, we report a case of a patient with severe SAPH treated with sildenafil. A significant, but only temporary improvement in functional status was observed, and the patient died of gradually progressing heart and respiratory failure while awaiting for lung transplantation.

Topics: Female; Humans; Hypertension, Pulmonary; Middle Aged; Sarcoidosis; Sarcoidosis, Pulmonary; Sildenafil Citrate; Vasodilator Agents

Sarcoidosis is a pleomorphic disease that can present with pulmonary hypertension (PH). What little information is available about the association of these two diseases comes mainly from small series of patients scheduled for transplant. We present 4 cases of mild pulmonary involvement in whom right catheterisation was performed and PH-specific therapy was administered. After obtaining written consent, a genetic study was performed that showed mutations in PH-related genes in 3 of the patients. This is the first study of its kind to yield genetic information for this type of PH.

Topics: Bone Morphogenetic Protein Receptors, Type II; Bosentan; Disease Progression; Epoprostenol; Fatal Outcome; Female; Humans; Hypertension, Pulmonary; Kv1.5 Potassium Channel; Male; Middle Aged; Mutation; Phenylpropionates; Point Mutation; Pyridazines; Respiratory Function Tests; RNA, Messenger; Sarcoidosis; Sildenafil Citrate; Sulfonamides; Tadalafil; Treatment Outcome

Pulmonary hypertension (PH) is a common complication of sarcoidosis that is associated with increased mortality. The pathogenesis of PH in sarcoidosis is uncertain, and the role of pulmonary arterial hypertension (PAH)-specific therapies remains to be determined.. We conducted a retrospective study of patients with sarcoidosis and PH at two referral centers. New York Heart Association (NYHA) functional class, exercise capacity, hemodynamic data, pulmonary function tests, and survival were collected and analyzed.. Twenty-two sarcoidosis patients treated with PAH-specific therapies were identified. After a median of 11 months of follow-up, NYHA class was improved in nine subjects. Mean 6-min walk distance (n = 18) increased by 59 m (p = 0.032). Patients with a higher FVC experienced a greater increment in exercise capacity. Among 12 patients with follow-up hemodynamic data, mean pulmonary artery pressure was reduced from 48.5 +/- 4.3 to 39.4 +/- 2.8 mm Hg (p = 0.008). The 1- and 3-year transplant-free survival rates were 90% and 74%, respectively.. PAH-specific therapy may improve functional class, exercise capacity, and hemodynamics in PH associated with sarcoidosis. Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to verify this apparent benefit. Mortality among the study population was high, highlighting the need for urgent evaluation at a lung transplant center.

Topics: Administration, Inhalation; Administration, Oral; Aged; Antihypertensive Agents; Bosentan; Cohort Studies; Drug Therapy, Combination; Epoprostenol; Female; Follow-Up Studies; Hemodynamics; Humans; Hypertension, Pulmonary; Iloprost; Kaplan-Meier Estimate; Linear Models; Male; Middle Aged; Piperazines; Probability; Purines; Respiratory Function Tests; Retrospective Studies; Risk Assessment; Sarcoidosis; Severity of Illness Index; Sildenafil Citrate; Statistics, Nonparametric; Sulfonamides; Sulfones; Survival Rate; Treatment Outcome

Severe pulmonary sarcoidosis is often complicated by pulmonary hypertension (PH) caused by different pathophysiological mechanisms.. To assess the acute vasoresponsiveness in patients with sarcoidosis and PH and the relation to the therapeutic effect of sildenafil.. A retrospective chart review of 25 patients with recalcitrant pulmonary sarcoidosis being evaluated for lung transplantation at our centre. Haemodynamics were evaluated by right heart catheterisation in 24 patients of whom 19 had PH. Eight of the 19 patients received vasodilator challenge with inhaled nitric oxide (iNO).. The study group of eight patients (seven men) had a median age of 51 years (range 38 years-58 years). During iNO we observed a reduction in all patients' mean pulmonary arterial pressure (MPAP) of median 9 mmHg (range 1 mmHg-20 mmHg) (P = 0.01) and in all patients' pulmonary vascular resistance of median 2.0 Wood Units (0.7 Wood Units-5.8 Wood Units) (P = 0.01). Acute vasoresponsiveness defined as reduction in MPAP of >or=10 mmHg to a MPAP of

Topics: Administration, Inhalation; Administration, Oral; Adult; Bronchodilator Agents; Chi-Square Distribution; Female; Hemodynamics; Humans; Hypertension, Pulmonary; Male; Middle Aged; Nitric Oxide; Piperazines; Purines; Respiratory Function Tests; Retrospective Studies; Sarcoidosis; Sildenafil Citrate; Statistics, Nonparametric; Sulfones; Treatment Outcome; Vasodilator Agents