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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cogan's syndrome is a rare idiopathic chronic inflammatory disease of the eye and the inner ear in young adults. Cogan's syndrome can be associated with large vessel vasculitis. We report a young female, with a history of interstitial keratitis and audiovestibular disease, who presents with large vessel vasculitis with left main coronary artery occlusion and develops heart failure. Cogan's syndrome was diagnosed on the basis of the presence of large vessel vasculitis with the typical inner ear and ocular involvement.
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PMID:Cogan's syndrome with left main coronary artery occlusion. 1995 Jan 3

Churg-Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.
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PMID:An aggressive and lethal course of Churg-Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy. 2002 52

Idiopathic inflammatory myopathies (IIMs) represent a heterogeneous group of autoimmune systemic diseases characterized by chronic muscle weakness and inflammatory cell infiltrates in skeletal muscle. The most frequent IIMs, such as adult-onset polymyositis and dermatomyositis, display a wide range of clinical manifestations other than myositis, including skin changes, Raynaud's phenomenon and interstitial lung disease. Cardiac involvement is now well recognized as a clinically important manifestation in patients with polymyositis or dermatomyositis, although its actual frequency is still uncertain. Cardiovascular complications represent one of the most frequent causes of death in myositis, apart from cancer and lung involvement. Despite the fact that clinical manifestations are relatively rare, asymptomatic cardiovascular features are frequently reported in patients with polydermatomyositis and dermatomyositis. They are characterized by isolated electrocardiographic changes, valve disease, coronary vasculitis, ischemic abnormalities, heart failure and myocarditis. Chronic inflammation producing myocyte degeneration, tissues fibrosis and vascular alterations can explain the majority of reported cardiac features in myositic patients. Although previous works reported an association between heart involvement and some myositis-specific autoantibodies (namely anti-signal recognition particle), electrocardiography, echocardiography and, where necessary, heart magnetic resonance remain the mainstay for diagnosing and monitoring myocardial inflammation in these diseases. Anyway, a complete multiorgan assessment and a careful analysis of autoantibodies should be performed in every patient in order to define any possible distinct disease entities with different prognosis within the spectrum of IIMs.
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PMID:Cardiological features in idiopathic inflammatory myopathies. 2062 8

There are many cause of cholinesterase deficiency, including drugs, liver disease, chronic anemia, malignant states, cardiac failure, severe acute infection, surgical shock, severe burn, collagen disease and vasculitis syndromes. Vasculitis syndromes are relatively rare, and among them, Churg-Strauss syndrome (CSS) is even rarer. We report here on a case of a patient with CSS who underwent endoscopic sinus surgery under general anesthesia.
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PMID:A patient with Churg-Strauss syndrome who underwent endoscopic sinus surgery under general anesthesia -A case report-. 2065 99

In this review of the gastrointestinal (GI) and hepatic manifestations of systemic lupus erythematosus (SLE), 180 articles from the English literature, found using a medline search from January 1965 to December 2010, were examined. Vasculitis may cause ulcerations, bleeding, stricture formation, and perforation from ischemia and infarction. Otherwise, GI symptoms, occurring in about 50% of patients, are usually mild. Esophageal dysmotility may result in heartburn, regurgitation, and dysphagia. Occasionally, pneumatosis cystoides intestinalis may develop, sometimes associated with benign pneumoperitoneum. Patients are prone to salmonella bacteremia, presenting more commonly with fever and abdominal pain than with diarrhea. Intestinal pseudoobstruction usually is found with active lupus serology, preferentially involving small rather than the large bowel. Protein-losing enteropathy, characterized by diarrhea, edema, and hypoalbuminemia, can be the initial presentation of SLE. Malabsorption with a prevalence of 9.5% is occasionally associated with celiac disease. Pancreatitis, with an annual incidence of 0.4 to 1/1000, has an overall mortality of 27% that is decreased with corticosteroid therapy. Acute and chronic ascites may be due to lupus peritonitis or to associated diseases, such as pancreatitis, nephrotic syndrome, heart failure, or infections. Abnormal liver function tests may be due to steatosis from lupus or from corticosteroid therapy. Only about 10% of patients with autoimmune hepatitis have lupus. Up to 4.7% of patients with SLE have chronic active hepatitis correlating strongly with the presence of antibody to ribosomal P protein. SLE can involve the entire GI tract and the liver. Treatment with corticosteroids, cytotoxic agents, and/or immunosuppressants is often successful.
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PMID:Gastrointestinal and hepatic manifestations of systemic lupus erythematosus. 2142 47

Pulmonary-renal syndromes are a group of disorders characterised by necrotising glomerulonephritis and pulmonary haemorrhage. Small vessel systemic vasculitis is the most common cause of pulmonary-renal syndromes presenting to respiratory physicians. Rarer causes include systemic lupus erythematosus and connective tissue diseases though severe pneumonia or cardiac failure may mimic their presentation. Some forms of small vessel vasculitides have a predilection for the pulmonary and renal vascular beds and if left untreated can result in fulminant organ failure. Whilst the aetiology of these syndromes remains unclear, much is known about the disease mechanisms including the pathogenic role of autoantibodies, immune-complex mediated inflammation and microangiopathic in-situ thrombosis. Despite established treatments achieving successful remission induction, patient tolerability and side effect profiles have limited their use which has led to searches for more targeted treatments. Consequently newer biological therapies have gained wider acceptance despite little being known about their long term safety and efficacy. The European Vasculitis Study Group (EUVAS) have recently formulated guidelines to provide consensus on diagnosis and management in this area and work to define survival rates in these conditions with longer term follow-up studies is ongoing. This review summarises the current aetiopathogenesis thought to underlie these complex diseases, the diagnostic definitions and classification criteria currently in use and the evidence base for modern therapies. Though unusual for respiratory specialists to coordinate overall management of these patients, an update on their current management is regarded as important to their practice given the recently changing trends in treatments.
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PMID:Pulmonary-renal syndromes: an update for respiratory physicians. 2168 32

Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a medium vessel vasculitis, most commonly involving the coronary arteries. Though subclinical myocarditis is rather common in KD, symptomatic congestive heart failure is extremely uncommon. The authors report a 9-y-old boy who developed heart failure (ejection fraction 28%) in the acute phase of KD. He was initially treated with intravenous immunoglobulin (2 g/kg) without much clinical improvement. He was then given 5 daily pulses of intravenous methylprednisolone followed by tapering doses of oral prednisolone. The child showed prompt clinical recovery and remains well on follow-up. The present case serves to highlight the fact that methylprednisolone can be considered upfront as rescue therapy in children with KD who have symptomatic congestive cardiac failure during the acute stage of the disease.
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PMID:Symptomatic myocarditis in Kawasaki disease. 2186 18

Kawasaki disease constitutes an acute febrile vasculitis of unknown aetiology. It is considered the most common cause of acquired cardiac failure in children. Although standard treatment comprises intravenous immunoglobulin and aspirin, some children exhibit refractory disease, necessitating the use of alternative therapies such as corticosteroids and anti-tumour necrosis factor-alpha. For these cases, few controlled data are available. This report focuses on an extremely refractory classical Kawasaki disease with coronary artery aneurysms and ongoing inflammation. We discuss the therapeutic approaches and the potential pitfalls undertaken, which led to an unfavourable clinical outcome.
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PMID:A girl with extremely refractory Kawasaki disease: an instructive case with unusual course and outcome. 2193 60

Takayasu's arteritis is an inflammatory vasculitis that primarily affects the aorta and its major branches. Involvement of the thoracic and abdominal aortas, although rare, causes marked hypertension and may lead to severe heart failure. We report the improvement of cardiac function after axillofemoral bypass grafting in a 59-year-old woman who had this condition.
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PMID:Axillofemoral Bypass to Treat Severe Heart Failure Caused by Takayasu's Arteritis. 2250 Feb 84

The investigation of relatively rare affections in familial Mediterranean fever--cardiac and lung lesions and pathogenesis of myocardium infarction in background of cardiac lesions is actual. Clinical-morphological analysis of 68 autopsy cases was done. The investigation data observes that cardiac amyloidosis as a dominated morphological manifestation in FMF can leads to heart failure and death. Macroscopically cardiac lesions as a cardiomegaly was observe. The morphological manifestation of cardiac affections in FMF was amyloidosis of the vessels and myocardium stroma. Amyloidosis of the heart valves leads to deformity and clinical-morphological picture of heart defect perform. The large amyloid areas of myocardium leading to the heart insufficiensy according to clinical and instrumental data as pseudoinfarctions were manifested. Myocardium infarction develops in background of cardiac lesions in FMF--amyloid angiopathias, which were more expressed in arteriolar walls, with narrowing or obstructing of lumina, and accompanied with them--coronary vasculitis. The main predisposing pathogenic factors for myocardial infarction can be atherosclerotic changes of the vessels also, which were complicated by amyloid depositions of the vascular walls. Cardiac failure can develop before renal amyloidosis and uremia.
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PMID:Myocardial infarction in patients with familial Mediterranean fever and cardiac lesions. 2257 51


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