1/43. Vanishing pulmonary hypertension in mixed connective tissue disease.A 29-year-old woman with mixed connective tissue disease presented with signs of progressive pulmonary hypertension. After admission to the hospital her condition worsened rapidly and she developed a cardiac arrest resistant to cardiopulmonary resuscitation. Therefore, emergency extracorporeal assist was performed. No pulmonary embolism was found. Right heart catheterisation showed severe pulmonary hypertension, which was treated with nitric oxide ventilation. She was weaned from the extracorporeal assist with high doses of inotropic agents. Because of suspicion of exacerbation of her underlying disease, which led to pulmonary hypertension, immunosuppressive treatment was started with high doses of corticosteroids and plasma exchange. This resulted in slow recovery over the next four weeks. Control echocardiography showed complete normalisation of cardiac function without signs of pulmonary hypertension. Two months after admission she was discharged from the hospital in good condition.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
2/43. Pulmonary hypertension.A clinically useful, treatment-based classification of pulmonary hypertension divides the disease into 5 distinct categories: (1) pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia; (2) pulmonary venous hypertension; (3) chronic thromboembolic disease; (4) pulmonary arterial hypertension; and (5) pulmonary hypertension due to disorders directly affecting the pulmonary vasculature. Pulmonary arterial hypertension includes individuals with primary pulmonary hypertension, congenital heart disease, connective tissue disease, and liver disease. These heterogeneous diseases have similar characteristic pathological changes, including in situ thrombosis, smooth muscle hypertrophy, and intimal proliferation. Right heart catheterization is essential to confirm diagnosis, determine prognosis, and assign therapy. A minority of patients have a favorable response to an acute vasodilator trial and long-term benefit with calcium channel blocker therapy. Continuous intravenous epoprostenol improves symptoms and survival in patients with advanced primary pulmonary hypertension and has potential benefit in other forms of pulmonary arterial hypertension. lung transplantation remains an important option for individuals in whom maximal medical therapy fails. The recent discovery of the gene for familial primary pulmonary hypertension and the increase in new drugs undergoing clinical trials are encouraging developments.- - - - - - - - - - ranking = 0.2keywords = connective (Clic here for more details about this article) |
3/43. Renal crisis due to intimal hyperplasia in a patient with mixed connective tissue disease (MCTD) accompanied by pulmonary hypertension.We report the case of a young female patient with mixed connective tissue disease (MCTD). She had marked pulmonary hypertension (PH) without lung fibrosis. She developed renal crisis after delivery by caesarean section. Renal biopsy revealed severe renal intimal hyperplasia with mild glomerular changes. The combination of intravenous pulse high-dose corticosteroid and cyclophosphamide (CPA) infusion and subsequent corticosteroid oral administration rescued her from renal crisis. This suggests that the possibility of co-incident renal intimal hyperplasia should be considered in patients with MCTD accompanied by PH. In addition, there might be some clinical benefit in combining high-dose corticosteroid with CPA infusion in the treatment of renal crisis due to intimal hyperplasia in MCTD.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
4/43. Cardiac valvular disease in chronic inflammatory disorders of connective tissue. Factors influencing survival after surgery.Three cases of cardiac valvular disease complicating chronic inflammatory disorders of connective tissue are described. Each underwent surgical valve replacement. The outcome in the first case, a patient with aortitis complicating Reiter's syndrome and in good health otherwise, has been excellent. The other two patients, one with rheumatoid arthritis and vasculitis, the other with systemic lupus erythematosus and nephritis, died after operation as the result of their systemic disease. The importance of systemic features in assessing this type of patient is emphasized.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
5/43. Scleroderma renal crisis and concurrent isolated pulmonary hypertension in mixed connective tissue disease and overlap syndrome: report of two cases.We here report on scleroderma renal crisis (SRC) appearing concurrently with isolated pulmonary hypertension (IPHT), that is, pulmonary hypertension without interstitial lung disease with fibrosis, in a patient with mixed connective tissue disease (MCTD) and in one with overlap syndrome or undifferentiated connective tissue disease (UCTD). To the best of our knowledge there are only five previous reports on SRC in MCTD and UCTD. The unexpected appearance of SRC in the setting of concomitant IPHT and limited or absent scleroderma skin changes is discussed.- - - - - - - - - - ranking = 1.2keywords = connective (Clic here for more details about this article) |
6/43. Severe erythroderma as a complication of continuous epoprostenol therapy.epoprostenol is a vasodilator that is produced by vascular endothelial cells and is currently the "gold standard" therapy for patients with severe primary pulmonary hypertension or pulmonary hypertension secondary to collagen vascular disease. hypersensitivity to the drug has not been reported. We report a case of a patient with pulmonary hypertension and undifferentiated connective tissue disease who, after 2 months of treatment with epoprostenol, presented with rapidly progressive erythema, scaling, nausea and vomiting, and fever. Test results from a skin biopsy specimen were consistent with a drug reaction. The patient' condition improved after rapid tapering of her epoprostenol and administration of corticosteroids. epoprostenol may be associated rarely with severe erythroderma.- - - - - - - - - - ranking = 0.2keywords = connective (Clic here for more details about this article) |
7/43. Recovery from pulmonary hypertension in an adolescent with mixed connective tissue disease.This paper describes the case of an 11 year old girl who presented with mixed connective tissue disease which was complicated by the development of pulmonary hypertension. This case is unique with respect to the young age of onset, the serial non-invasive method used to follow the disease process, and the favourable response to treatment with vasodilator and anti-inflammatory drugs.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
8/43. Primary pulmonary hypertension after amfepramone (diethylpropion) with BMPR2 mutation.Primary pulmonary hypertension (PPH) is characterised by sustained elevations of pulmonary arterial pressure without a demonstrable cause, leading to right ventricular failure and death. Hereditary mutations in the bone morphogenetic protein receptor type II (BMPR2) gene result in familial PPH transmitted as an autosomal dominant trait, albeit with low penetrance. The causes in cases without a BMPR2 mutation are unknown, but a syndrome of pulmonary arterial hypertension (PAH) similar to hereditary PPH is associated with systemic connective tissue disease, congenital heart disease, portal hypertension, and human immunodeficiency virus infection, or with the use of appetite-suppressant drugs. The authors identified a BMPR2 gene mutation in a 27-yr-old female who developed PAH after a short course of the appetite-suppressant drug amfepramone (diethylpropion). This allowed molecular genetic counselling and prevention of potentially harmful drug exposure in the patient's son treated for attention deficit disorder with methylphenidate, an amphetamine-related drug. No BMPR2 mutation was found in four additional, unrelated patients with appetite suppressant-related PPH. The findings provide strong evidence that amfepramone can trigger primary pulmonary hypertension in a bone morphogenetic protein receptor type II gene mutation carrier, and indicate that other genes are probably implicated in genetic susceptibility to appetite suppressants.- - - - - - - - - - ranking = 0.2keywords = connective (Clic here for more details about this article) |
9/43. Severe pulmonary hypertension associated with primary sjogren's syndrome.Severe pulmonary hypertension is one of the fetal complications in various connective tissue diseases. We report a case of severe pulmonary hypertension associated with primary sjogren's syndrome. In a lung biopsy specimen, there were findings of intimal and medial hypertrophy with narrowing vessel lumina and plexiform lesions. Moreover, deposits of immunoglobulin m, immunoglobulin a and complement protein C1q were found in the pulmonary arterial walls. Although pulmonary hypertension was refractory to oral prostacyclin, steroid therapy improved the clinical and hemodynamic conditions. In the present case, the immunological etiology may be related to the mechanisms of pulmonary hypertension associated with sjogren's syndrome.- - - - - - - - - - ranking = 0.2keywords = connective (Clic here for more details about this article) |
10/43. Ten year survival of a patient with advanced pulmonary hypertension and mixed connective tissue disease treated with immunosuppressive therapy.Pulmonary hypertension is the most frequent cause of death in patients with mixed connective tissue disease. The pulmonary hypertension is progressive and difficult to treat effectively. These patients experience considerable morbidity secondary to diminished cardiac function. We describe a case in which sequential administration of cyclophosphamide and cyclosporine A favorably altered the clinical and hemodynamic course of this serious complication.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
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