Octreotide treatment of chronic intestinal pseudoobstruction secondary to connective tissue diseases

Treatment of pulmonary hypertension secondary to connective tissue diseases.

Pulmonary hypertension has been identified as one of the well known life threatening complications of connective tissue diseases. In patients with connective tissue diseases pulmonary hypertension occurs with little or no evidence of parenchymal lung disease. As in primary pulmonary hypertension, the pathophysiology is unknown but the clinical course and pathological findings are similar. Scler...

A Case of Dermatomyositis and Anti-EJ Autoantibody with Chronic Intestinal Pseudoobstruction Successfully Treated with Octreotide

Chronic intestinal pseudoobstruction (CIPO) is a serious complication in patients with connective tissue disease (CTD) and is sometimes life-threatening or fatal despite intensive medical treatment. Here, we report a patient with dermatomyositis (DM) and anti-EJ autoantibody who developed CIPO that was improved by octreotide. Because her abdominal pain and bloatedness were so severe and persist...

Response to octreotide of intestinal pseudoobstruction and pneumatosis cystoides intestinalis associated with progressive systemic sclerosis.

Intestinal pseudoobstruction and pneumatosis cystoides intestinalis are uncommon complications of progressive systemic sclerosis. We report a 26-year-old woman with this disorder who responded poorly to conventional treatment. Subcutaneous administration of octreotide, a long-acting somatostatin analogue, at a dose of 50 micrograms/day for 3 weeks, relieved symptoms such as nausea and bloating....

Connective tissue diseases.

Undiagnosed connective tissue diseases

Among different subgroups of pulmonary arterial hypertension (PAH), those associated with connective tissue diseases (CTDs) have distinct hemodynamic and prognostic features; a correct etiologic diagnosis is thus mandatory.To estimate frequency and prognosis of previously undiagnosed CTDs in a suspect idiopathic (i) PAH cohort.Consecutive patients with PAH confirmed by right heart catheterizati...