ABSTRACT
BACKGROUND: Intravenous periodic Iloprost is proven effective in the treatment of Raynaud phenomenon (RP) related to connective tissue disorder (CTD). It's well known that synthetic prostaglandins are effective drugs for the treatment of pulmonary arterial hypertension (PAH), and that PAH is frequently associated with CTD. OBJECTIVE: The aim of the study is to evaluate in the chronic effect of cyclic intravenous Iloprost on pulmonary arterial pressure. METHODS: We studied 17 consecutive patients with CTD (14 systemic sclerosis, 3 mixed CTD) and RP, at the entry and after at least 6months of treatment of RP with cyclic Iloprost. On both occasions, in all patients we performed transthoracic Doppler echocardiography and we determined NT-proBNP plasma levels, NYHA functional class, 6 Minute-Walk Distance (6MWD). RESULTS: At follow-up (8.2±1.9months; range 6-12) mean values of pulmonary arterial systolic pressure (PASP) significantly decreased (from 32.2±9.2 to 29.2±7.6mmHg, p<0.04) and mean values of 6MWD significantly increased (from 407.5±101.5 to 448.3±89.9m, p<0.01). Moreover, we observed a significant direct correlation between PASP and NT-proBNP values and a significant inverse correlation both between NT-proBNP and 6MWD values and between PASP and 6MWD values. CONCLUSION: Our results suggest that cyclic intravenous Iloprost may protect against the development or worsening of PAH in patients with CTD and RP.
Subject(s)
Connective Tissue Diseases/complications , Hypertension, Pulmonary/prevention & control , Iloprost/administration & dosage , Pulmonary Wedge Pressure/drug effects , Adult , Aged , Cardiovascular Agents/administration & dosage , Cardiovascular Agents/therapeutic use , Connective Tissue Diseases/physiopathology , Dose-Response Relationship, Drug , Familial Primary Pulmonary Hypertension , Female , Follow-Up Studies , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Iloprost/therapeutic use , Infusions, Intravenous , Male , Middle Aged , Pulmonary Wedge Pressure/physiology , Retrospective Studies , Time Factors , Treatment OutcomeABSTRACT
Interferon (IFN) therapy is nowadays widely used in clinical practice. In the literature, there are very few reports of the association between IFN therapy and pulmonary arterial hypertension (PAH), and current guidelines do not mention IFNs as a risk factor for PAH. We describe a patient with multiple sclerosis who developed severe PAH after treatment with IFN-ß-1a and the clinical response to sildenafil. Furthermore, we stress the need to further investigate the link between IFNs and PAH.
Subject(s)
Adjuvants, Immunologic/adverse effects , Antihypertensive Agents/therapeutic use , Hypertension, Pulmonary/chemically induced , Interferon-beta/adverse effects , Piperazines/therapeutic use , Sulfones/therapeutic use , Familial Primary Pulmonary Hypertension , Female , Humans , Hypertension, Pulmonary/drug therapy , Interferon beta-1a , Middle Aged , Multiple Sclerosis/drug therapy , Purines/therapeutic use , Sildenafil CitrateABSTRACT
The authors discuss the diagnostic and therapeutic implication regarding the clinical case of a young woman with hypertension due to atypical coarctation type Takayasu arteritis with impressive stenosis of renal arteries, of the proximal segment of celiac tripod and of superior mesenteric artery.
Subject(s)
Takayasu Arteritis/complications , Adult , Amlodipine/administration & dosage , Amlodipine/therapeutic use , Angiotensin-Converting Enzyme Inhibitors/administration & dosage , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Anti-Inflammatory Agents/administration & dosage , Anti-Inflammatory Agents/therapeutic use , Antihypertensive Agents/administration & dosage , Antihypertensive Agents/therapeutic use , Female , Hospitalization , Humans , Hypertension, Renovascular/etiology , Prednisone/administration & dosage , Prednisone/therapeutic use , Radiography, Abdominal , Radiography, Thoracic , Ramipril/administration & dosage , Ramipril/therapeutic use , Renal Artery Obstruction/complications , Renal Artery Obstruction/etiology , Takayasu Arteritis/diagnostic imaging , Takayasu Arteritis/drug therapy , Time Factors , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Pulmonary arterial hypertension (PAH) is a devastating disease with significant disability and mortality, and it has much higher prevalence than previously thought. During the past 15 years, we have witnessed remarkable advances in our understanding of pathogenesis, in diagnostic process and in the development of disease-specific treatments for PAH. Nowadays, the diagnosis is more clearly defined, non-invasive markers of disease severity can be widely applied, and finally we can adopt evidence-based treatment. Newer drugs availability has resulted in radical change in the management of this disease. The article reviews established approaches to evaluation and treatment of this disorder.
