Successful treatment of sarcoidosis-associated pulmonary hypertension with corticosteroids

We report a case of sarcoidosis presenting with cor pulmonale of a greater severity than would be expected from the degree of hypoxia and pulmonary fibrosis. Right heart catheterization revealed that mean pulmonary artery pressure was markedly increased (42 mm Hg), was not reduced by supplemental oxygen, but was reduced significantly (25 mm Hg) after 16 weeks of therapy with corticosteroids. Improvement in symptoms and pulmonary function was maintained for an 18-month period of observation after cessation of corticosteroid therapy.     

Successful treatment of sarcoidosis-associated pulmonary hypertension with bosentan

Sarcoidosis is a systemic granulomatous disease of unknown etiology, in which the lungs and intrathoracic lymph nodes are predominant sites of involvement. Pulmonary hypertension is a known complication of sarcoidosis. Treatment of sarcoidosis-associated pulmonary hypertension has traditionally focused on the initiation of systemic corticosteroids, but has had inconsistent results. We present a patient with sarcoidosis-associated pulmonary hypertension who achieved substantial clinical improvement with the dual endothelin receptor antagonist bosentan.     

Exercise-induced pulmonary hypertension

Exercise stress tests of the pulmonary circulation show promise for the detection of early or latent pulmonary vascular disease and may help us understand the clinical evolution and effects of treatments in patients with established disease. Exercise stresses the pulmonary circulation through increases in cardiac output and left atrial pressure. Recent studies have shown that exercise-induced increase in pulmonary artery pressure is associated with dyspnea-fatigue symptomatology, validating the notion of exercise-induced pulmonary hypertension. Exercise in established pulmonary hypertension has no diagnostic relevance, but may help in the understanding of changes in functional state and the effects of therapies.     

Primary pulmonary hypertension

Primary pulmonary hypertension (PPH) is a rare disorder characterised by raised pulmonary-artery pressure in the absence of secondary causes. Precapillary pulmonary arteries are affected by medial hypertrophy, intimal fibrosis, microthrombosis, and plexiform lesions. Most individuals present with dyspnoea or evidence of right heart failure. Echocardiography is the best non-invasive test to screen for suspected pulmonary hypertension. The discovery of mutations in the coding region of the gene for bone morphogenetic protein receptor 2 in patients with familial and sporadic PPH may help not only to elucidate pathogenesis but also to direct future treatment options. The pathogenesis is not completely understood, but recent investigations have revealed many possible candidate modifier genes. Without treatment, the disorder progresses in most cases to right heart failure and death. With current therapies such as epoprostenol, progression of disease is slowed, but not halted. Many promising new therapeutic options, including prostacyclin analogues, endothelin-1-receptor antagonists, and phosphodiesterase inhibitors, improve clinical function and haemodynamic measures and may prolong survival.     

Primary pulmonary hypertension

Primary pulmonary hypertension has become a very treatable disease given the recent advances in medical therapy. Any patient with unexplained right ventricular enlargement or pulmonary hypertension needs a thorough workup to determine the cause. Patients with primary pulmonary hypertension should be referred to a specialized "Center of Excellence" to evaluate potential therapies on a case-by-case basis. Warfarin anticoagulation is generally recommended in all patients. Some patients (approximately 20%) will have a dramatic response to high doses of calcium channel blockers; the remainder are generally helped by the use of continuous intravenous prostacyclin therapy. Lung transplantation remains the final treatment option for patients in whom medical therapy fails.     

Secondary pulmonary hypertension

Opinion statement  The diagnosis of pulmonary hypertension first requires a clinical suspicion, as symptoms are often nonspecific. After the diagnosis is made, appropriate classification into the various categories of pulmonary hypertension is essential in order to manage the patient’s disease and symptoms appropriately. Therapy is targeted at the underlying cause of the pulmonary hypertension, as well as its effects on the cardiovascular system. Until recently, the treatment of both primary and secondary pulmonary arterial hypertension was limited to supportive therapy alone. With the advent of novel therapeutic agents, more focused therapies designed to treat the pulmonary vasculopathy have become available. These include pulmonary vasodilators such as continuous intravenous prostacyclin, and experimental agents currently undergoing clinical trials. For patients with pulmonary hypertension secondary to pulmonary venous hypertension, therapies differ. In cases where there is left-sided heart disease leading to pulmonary venous hypertension, treatment is aimed at repairing or ameliorating the underlying heart disease. Patients with pulmonary venous hypertension due to extrinsic compression of the central pulmonary veins, or pulmonary veno-occlusive disease have few options, and treatment is generally palliative. In patients with pulmonary hypertension that is associated with disorders of the respiratory system or hypoxemia, the pulmonary hypertension is due to a reactive pulmonary vasoconstriction. Reversal of this vasoconstriction with pulmonary vasodilators can be harmful because of the risk of increasing perfusion to nonventilated lung units. Pulmonary hypertension due to chronic thrombotic or embolic disease can be treated surgically, if the obstructive thrombi are proximal enough for the surgeon to resect them. More distal pulmonary emboli, however, cannot be resected, but there is emerging evidence that the chronic administration of pulmonary vasodilators can be effective in treating this form of the disease.     

Primary pulmonary hypertension

Primary Pulmonary Hypertension (PPH) is a rare disease that progressively increases pulmonary arterial pressure and pulmonary vascular resistance to the point of right heart failure, in the absence of secondary causes of the disease. The following specific risk factors that can trigger PPH have been identified and examined: appetite depressant drugs, oral contraceptives and hyperuricaemia. Familial PPH transmitted by an incomplete penetrance dominant autosomic mechanism is responsible for about 6% of PPH cases. Recent research groups have identified the gene responsible for familial PPH. Since pulmonary vasoconstriction is the prime cause of PPH, vasodilation of the pulmonary arterial circulation system (using prostacyclin in the form of epoprostenol or iloprost) is the main aim of treatment.     

Primary pulmonary hypertension

Primary pulmonary hypertension is a rare disease of unknown etiology, which mainly affects people in the third and fourth decades. Although the clinical cause is highly variable and spontaneous regression has been described, survival rates are usually poor, averaging only three years after diagnosis. Treatment with vasodilator agents, especially calcium channel blockers and prostaglandins, usually improves symptoms, but have not been shown to improve chances for survival. In a few selected patients who continue to deteriorate despite medical treatment, lung transplantation may be the only option for improving function and survival.