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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Embolization of cholesterol crystals from ulcerated atheromatous lesions can produce distinct syndromes that mimic more common disease processes. Cholesterol emboli can present as renal failure, hypertension, spells of numbness, abdominal pain, and myocardial infarction, or as a multisystem disease that closely approximates the presentation, clinical course, and even biopsy picture of polymyositis or periarteritis nodosa. A review of this problem with particular attention to the clinical presentations should help in the early diagnosis and treatment of cholesterol emboli and avoid unnecessary and inappropriate therapies.
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PMID:Cholesterol embolism: the great masquerader. 37 Oct 3

Polymyositis-dermatomyositis (PM-DM) is an inflammatory disease of muscle and skin mediated by autoimmune and cellular events. Most typically, muscle weakness is the usual presentation. This review emphasizes that often the systemic components of this disease may mask the usual presentation and actually may be the presenting and only manifestations; more often than not they are the causes of increased morbidity and mortality. In particular, the cardiopulmonary manifestations may dominate the disease course. Cardiac complications include congestive heart failure resulting from a primary cardiomyopathy, disrhythmias and atrioventricular conduction disturbances, sick sinus syndrome, and cor pulmonale either secondary to interstitial lung disease (ILD) or primary pulmonary artery hypertension. Recurrent aspiration pneumonia results from pharyngeal muscle involvement by the myositic process. Several histologic patterns of ILD can emerge with varying outcomes and responses to immunosuppresive therapy. Involvement of the muscles of respiration can lead to hypercapnic respiratory failure, diaphragmatic dysfunction, hypostatic pneumonia, and restrictive lung disease.
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PMID:Pulmonary and cardiac manifestations of polymyositis-dermatomyositis. 157 25

Patients with mixed connective tissue disease (MCTD) exhibit clinical features of systemic lupus erythematosus (SLE), progressive systemic sclerosis or scleroderma (PSS), and polymyositis-dermatomyositis (PM-DM). In their sera is an unusually high titer of a circulating antinuclear antibody with specificity for a nuclear ribonucleoprotein antigen. Pleuropulmonary manifestations are common in MCTD and the incidence varies from 20% to 85%. The pleuropulmonary complications include pleural effusion, interstitial pulmonary processes, pulmonary arterial hypertension (PAH), pulmonary vasculitis, pulmonary thromboembolic phenomena, aspiration pneumonia, and hypoventilatory failure. Pulmonary vascular pathology with progressive PAH and cor pulmonale is the most serious complication of MCTD. The pleuropulmonary manifestations in MCTD are similar to the respiratory problems well recorded in SLE, PSS, and PM-DM. Even though the pleuropulmonary complications are common in MCTD, they may remain clinically inapparent until fatal complications ensue.
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PMID:Lungs in mixed connective tissue disease. 157 26

In this review, the cardiac lesions which develop in association with the various collagen-vascular diseases are described. In rheumatoid arthritis, the most frequent lesions are: fibrous obliterative pericarditis, with pericardial deposits of calcium, fibrin, cholesterol, and rheumatoid granulomas; granulomatous or nonspecific myocarditis; valvulitis, vasculitis, and amyloid deposits. In ankylosing spondylitis, the lesions involve mainly the valves (aortic and mitral valves) and the aorta. In systemic lupus erythematosus, the predominant cardiovascular lesions are: pericarditis, Libman-Sacks endocarditis, nonspecific myocarditis, vasculitis with fibrinoid necrosis, and acceleration of atherosclerosis. In scleroderma, the main cardiac lesion is fibrosis with only scanty inflammatory cells; pericarditis and nonbacterial thrombotic endocarditis also occur. In dermatomyositis/polymyositis, fibrous or fibrinous pericarditis can occur, as well as myocarditis with infiltrates of lymphocytes and plasma cells and with degeneration and necrosis of myocytes; valvulitis is uncommon except when the disease is related to mucinous adenocarcinoma. In polyarteritis nodosa, various stages of necrotizing vasculitis involve all layers of the arterial walls; foci of myocardial necrosis of various sizes can occur in association with these lesions; cardiac hypertrophy related to hypertension and pericarditis related to uremia, may also be found. In Wegener's granulomatosis, pericarditis, inflammatory infiltrates, necrotizing granulomas, and vasculitis have been observed in the heart.
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PMID:Cardiovascular lesions in collagen-vascular diseases. 391 76

In this study we have investigated the efficacy and safety of cyclosporin A (CyA) in a group of pediatric patients with juvenile chronic arthritis (JCA, 9 cases) and polymyositis-dermatomyositis (PM-DM, 3 cases). Of the 9 JCA patients, 7 had the systemic and 2 the polyarticular form of the disease. All of the patients received CyA after the failure of corticosteroids and/or cytotoxic drugs. CyA was administered for 9 to 48 months at a mean dose of 5 mg/kg/day. Ten patients received corticosteroids with CyA. The results of CyA treatment were satisfactory overall, both in the PM-DM patients and in the JCA patients, as assessed by clinical and laboratory evaluation. CyA-related side effects included alopecia, hypertension, hypertrichosis, tremors, and hirsutism. One JCA patient developed polyserositis with hypoproteinemia of unknown origin while receiving CyA. CyA blood levels did not correlate with clinical efficacy and/or side effects. This study suggests that CyA represents a promising agent for the treatment of JCA and childhood PM-DM.
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PMID:Cyclosporin A in the treatment of juvenile chronic arthritis and childhood polymyositis-dermatomyositis. Results of a preliminary study. 850 52

A 65-year-old woman was given a diagnosis of polymyositis in April 1991. She was treated with prednisolone until December 1993, at which time muscle strength had increased and high blood pressure had developed. In May 1994 she was hospitalized for muscle weakness and mild liver dysfunction. Prednisolone was given and the levels of hepatobiliary enzymes decreased. Immunological examination revealed strongly positive results for anti-mitochondria antibody and M-2 antibody, which lead to the diagnosis of primary biliary cirrhosis. administration of ursodeoxycholic acid in addition to prednisolone was followed by normalization of liver function and a decrease in the production of the autoantibodies. Although polymyositis can be complicated by autoimmune diseases, reports of complication by primary biliary cirrhosis are rare, here we report that treatment with the combination of ursodeoxycholic acid and prednisolone was successful in a patient with liver dysfunction and primary biliary cirrhosis.
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PMID:[Primary biliary cirrhosis with polymyositis successfully treated with prednisolone and ursodeoxycholic acid]. 895 42

A 60-year-old man with dermatomyositis was admitted to our hospital because of dyspnea and hypertension. He had high fever and convulsive seizures after admission. Laboratory examinations showed hemolytic anemia, thrombocytopenia, and renal failure. A clinical diagnosis of thrombotic thrombocytopenic purpura (TTP) was made. He failed to respond to plasma exchange therapy, pulse therapy with methylprednisolone, high-dose gamma-globulin therapy, and antiplatelet therapies with ticlopidine, dipyridamole and a prostacyclin analog of beraprost sodium. He died on his 17th day in hospital. Autopsy examination revealed widespread microthrombi in his kidneys, lungs, spleen, and intestine. Only seven cases of dermatomyositis or polymyositis complicated by TTP have been cited in the literature. TTP was fatal in 6 of these 7 cases. Early diagnosis and prompt treatment may improve the outcome of TTP patients with dermatomyositis. Dermatologists should keep in mind that TTP occasionally arises as a serious complication of dermatomyositis.
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PMID:A case of dermatomyositis complicated by thrombotic thrombocytopenic purpura. 903 97

Among connective tissue diseases, systemic lupus erythematosus is the illness that is most concerned by hormonal life events. The sex ratio is 9/1, and symptoms begin mostly during the third decade, sometimes during birth pill contraception or during pregnancy. As soon as systemic lupus is under control of an efficient treatment, pregnancy is no longer contra-indicated. A medical multidisciplinary surveillance is required. Complicated pregnancy concerns mother and baby. Lupus flares are more frequent during the second and third trimesters as well as during the post-partum period. Usually the intensity is moderate. Severe flares concern patients with renal involvement, hypertension and renal insufficiency and are mostly seen in patients with unplanified pregnancy and yet with still active lupus. Foetal death occurs in 10-30% of the cases, depending on the lupus activity and severity (renal lupus). Prematurity remains an important cause of morbidity (30% of live births). Foetal deaths and prematurity are even more frequent if the patient has an antiphospholipid syndrome. Neonatal cutaneous lupus and auriculo-ventricular congenital heart block is infrequent (1% of SLE patients with anti-Ro/SSA antibodies). Among other connective tissue diseases, polymyositis has a very severe obstetrical prognosis for both mother and foetus. Among primary vasculitis, polyarteritis nodosa, as found during pregnancy, can herald a very bad prognosis.
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PMID:[Hormonal life in systemic lupus and other connective tissue diseases]. 1449 21

The negative association between gout and rheumatoid arthritis is widely accepted, and gout is also speculated to be rare in systemic lupus erythematosus (SLE), as only a few sporadic cases have been reported. From 1985 to 2001 we encountered 15 lupus patients at Chang-Gung Memorial Hospital, including two with lupus-scleroderma and one with lupus-scleroderma-polymyositis overlap syndrome coexisting with gout. This study retrospectively analyses the clinical and laboratory characteristics of these patients. A lower female predominance is found, and most patients developed gout after the onset of SLE, although gout preceded SLE in two cases. Measurement of serum uric acid and 24-h urine uric acid found all of the patients to be hyperuricaemic and underexcretors of uric acid. Furthermore, most of the patients (14/15) were receiving diuretics. Also, many had hypertension and serious cardiovascular diseases. Renal impairment during gouty attacks seemed to be a predisposing factor for developing end-stage renal disease. Gouty arthritis usually occurred during relative SLE inactivity, podagra was frequent, and tophi were found in a few patients. Compared with the unselected population of SLE patients, the cases studied here had a higher incidence of chronic arthritis, malar rash, haematologic disorder, photosensitivity, serositis and neurologic disorder. Renal disease in the patients sampled was frequently membranous nephropathy.
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PMID:Gout in systemic lupus erythematosus and overlap syndrome - a hospital-based study. 1457 59

Connective tissue diseases represent a group of heterogeneous disorders that share certain common features, including inflammation of the skin, joints and other structures rich in connective tissue, as well as altered immunoregulation patterns, including autoantibody production and cell-mediated immunity abnormalities. While certain distinct clinical entities can be defined, manifestations can vary considerably from patient to patient, and the overlap of clinical features between and among specific diseases is common. Genetic, environmental and sex hormonal factors are likely to be of pathogenic importance. Among these diseases, systemic lupus erythematosus is the most frequent, followed by systemic sclerosis or scleroderma (SSc), Sjogren's disease, polymyositis and dermatomyositis and mixed connective tissue disease. Systemic sclerosis involves multiple organs in a process consisting of disseminated sclerosis affecting all compartments. Prominent vascular lesions typify the renal lesions. Scleroderma crisis is a major complication of SSc, characterized by severe hypertension, rapidly progressive renal failure, thrombotic microangiopathy with hemolitic anemia and low platelet count: the prompt use of angiotensin-converting enzyme (ACE) inhibitors significantly increased the 1-yr survival rate from 15 to 76%. Sjogren's syndrome is an immunologic disorder characterized by progressive lymphocytic destruction of the exocrine gland, frequently resulting in symptomatic eye and mouth dryness. The interstitial lesions constitute the principal renal manifestations: interstitial infiltrates and small lymphocytes, which can be homogenous and massive. In polymyositis and dermatomyositis renal involvement is rare.
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PMID:[Non SLE connective tissue diseases: general aspects and kidney]. 1641


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