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At least 19 recordsLinked to original sources
PubMed1992

[Mixed connective tissue disease].

Mixed connective tissue disease can be regarded as a distinct rheumatic disease syndrome. It can be differentiated from the other systemic rheumatic diseases and overlap syndromes, and has its own diagnostic criteria. The article presents symptoms, treatment, outcome, classification and diagnosis of mixed connective tissue disease. The most usual symptoms are Raynaud's phenomenon, swelling of the hands, polyarthritis, muscle weakness, oesophageal hypomotility and pulmonary disease. The recommended therapy should be based on patterns of involvement of organ systems and the severity of the disease. In severe cases the treatment is often a combination of corticosteroids and cytostatics. Due to severe impacts on lungs and kidneys it seems necessary to modify earlier predictions of a generally favourable outcome in patients with mixed connective tissue disease.

Diagnosis, Differential↗
PubMed2004

[Undifferentiated, overlapping and mixed connective tissue diseases].

Mixed connective tissue disease (MCTD), undifferentiated connective tissue disease and overlap syndromes are autoimmune systemic diseases that must be differentiated. Antibodies against the U1-ribonucleoprotein complex --spliceosome-- allows the diagnosis of mixed connective tissue disease. Links between the immunologic and clinical phenomena are emerging. Longitudinal studies of patients with MCTD highlight the impact of pulmonary hypertension and contribute to define the disease. Immunogenetic studies hold MCTD as an independent disease.

Antibodies↗
PubMed1999

Mixed connective tissue disease.

Mixed connective tissue disease deserves to be a distinct disease entity due to the persistent citation of this disease in the literature since the original description by Sharp in 1972, in spite of the presence of several criticisms against the independency of this disease. The characteristic features of mixed connective tissue disease are: 1) the presence of anti-U1snRNP antibody with high titers in sera, 2) an increased frequency of HLA-DR4 in the leukocytes, and 3) death due to pulmonary hypertension.

Autoantibodies↗
PubMed2003

Nucleoporin p62 antibodies in a case of mixed connective tissue disease.

Mixed connective tissue disease is an overlap syndrome characterized by features of different systemic autoimmune diseases and a high titer of U1-snRNP antibodies. We examine here the autoantibodies to nucleoporin p62 in a severe case of mixed connective tissue disease in a young male patient. Thus far, p62 antibodies have mainly been described in cases of primary biliary cirrhosis. We speculate that the presence of p62 antibodies is an indication of a poor prognosis in connective tissue disorders.

Adult↗
PubMedDate not supplied

Gastrointestinal systemic sclerosis in serologic mixed connective tissue disease.

Mixed connective tissue disease is a clinical entity defined by overlapping features of progressive systemic sclerosis, systemic lupus erythematosus, polymyositis, rheumatoid arthritis, and distinct serologic findings. Esophageal dilatation and dysmotility have been the only gastrointestinal manifestations reported. Three patients with serologic findings of mixed connective tissue disease and extensive gastrointestinal involvement compatible with the changes found in progressive systemic sclerosis are presented. Gastrointestinal manifestations of progressive systemic sclerosis are reviewed and were found to be indistinguishable from the findings in these patients.

Adult↗
PubMed1984

Pulmonary hemorrhage and acute renal failure in a patient with mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is considered to have a benign clinical course. Recently, renal disease has been recognized as a frequent complication (20%). This report describes a patient with MCTD who developed pulmonary hemorrhage and acute renal failure. The renal biopsy, in addition to a long-standing membranous nephropathy, revealed a tubulointerstitial nephritis. Immunofluorescent and electron microscopy showed immune complex deposition along the tubular basement membrane. Life-threatening, immune complex-mediated events can occur in the course of MCTD, manifested as pulmonary hemorrhage and tubulointerstitial nephritis.

Acute Kidney Injury↗
PubMed2005

Treatment of mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is believed to be incurable and seems to have a variable prognosis. Some patients have a mild self-limited disease, whereas others develop major organ involvement that requires aggressive treatment. Because no controlled clinical trials have been performed to guide therapy in MCTD, treatment strategies must rely largely upon the conventional therapies that are used for similar problems in other rheumatic conditions (systemic lupus erythematosus, scleroderma, polymyositis). Given the heterogeneous clinical course of MCTD, therapy should be individualized to address the specific organ involved and the severity of underlying disease activity. Corticosteroids, antimalarials, methotrexate, cytotoxics (most often cyclophosphamide), and vasodilators have been used in the treatment of MCTD with varying degrees of success.

Cardiovascular Diseases↗
PubMed2005

Pulmonary vascular manifestations of mixed connective tissue disease.

Mixed connective tissue disease (MCTD) refers to a disease process with combined clinical features characteristic of systemic lupus erythematous, scleroderma, and polymyositis-dermatomyositis. This article focuses on the pulmonary vasculature manifestations of MCTD. We briefly discuss associations between MCTD and interstitial lung disease, pleural disease, and alveolar hemorrhage.

Humans↗
PubMed1994

[MCTD (mixed connective tissue disease)].

Mixed connective tissue disease (MCTD) was proposed by Sharp and others in 1972. MCTD is a unique disease in which the presence of nuclear RNP antibody is characteristic and the patient shows partial symptoms of SLE, PSS and or PM/DM. Among them, Raynaud's phenomenon and sausage like finger or swollen hand are the most common symptoms. Although patients with MCTD generally respond to small amount of corticosteroid and the prognosis is not so bad, some patients with MCTD especially those with pulmonary hypertension show high mortality.

Humans↗
PubMedDate not supplied

Pulmonary hypertension in a child with mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is characterized by high titers of antibody to ribonucleoprotein (RNP) in patients with features of several rheumatic diseases. We describe a child whose MCTD included arthritis, Raynaud's phenomenon, mucocutaneous ulcerations, sclerodermatous skin changes, restrictive lung disease, reduced carbon monoxide pulmonary diffusing capacity, abnormal esophogeal motility, and severe pulmonary hypertension. High antibody titers to ds-DNA and RNP were present. Clinical improvement followed therapy with prednisone and cyclophosphamide. Improvement in the degree of pulmonary hypertension was documented by repeat cardiac catheterization 8 months after the initiation of combination therapy.

Adolescent↗
PubMedDate not supplied

Successful treatment of acute right cardiac failure due to pulmonary thromboembolism in mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is characterized as a benign rheumatic disease with a favorable response to therapy. When pulmonary hypertension is a complication, however, it is often reported to be fatal. A 32-year-old female patient with MCTD who had developed rapidly progressive pulmonary hypertension and disseminated intravascular coagulopathy was admitted to our hospital and was successfully treated with corticosteroids and anticoagulants. The failure of microcirculation due to coagulopathy is considered to be one of the possible entities of pulmonary hypertension.

Adult↗
PubMed1990

Orofacial manifestations of mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is a multisystem disorder with overlapping features of systemic lupus erythematosus, scleroderma and polymyositis and is differentiated from them by a high titre of antibody to ribonucleoprotein. Orofacial manifestations of MCTD include trigeminal neuralgia-like pain, neuropathy, features suggestive of Sjögren's syndrome and lymphadenopathy. The recent experience of two patients presenting with trigeminal neuropathy as an early manifestation of this disease is described. One of these patients presented with 'lichenoid' oral mucosal lesions as a further early manifestation of MCTD. This appears to be the first case with this presenting feature to be described in the literature.

Adult↗
PubMed2006

Mixed connective tissue disease.

Mixed connective tissue disease (MCTD) was first described in 1972 as a disease syndrome with overlapping features of systemic sclerosis, systemic lupus erythematosus (SLE) and polymyositis associated with antibodies to RNAse sensitive extractable nuclear antigen. When the antigen was subsequently characterized as polypeptides on the U1 ribonuclear protein component of the splicesosome (U1RNP), MCTD became the first rheumatic disease syndrome to be defined by a serologic test. Clinical features include a high frequency of Raynaud's syndrome, swollen hands, sclerodactyly, arthritis, polymyositis and interstitial lung disease. Over the last 30 years there has been a continuing debate as to whether MCTD constitutes a 'distinct clinical entity'. Here, I will review the pathological, immunogenetic and clinical features of MCTD and conclude that the debate remains unresolved. The early misconception that it has a relatively good prognosis has not stood the test of time with long-term follow-up studies. These have identified a tendency for MCTD to evolve into SLE or systemic sclerosis and highlighted pulmonary hypertension and scleroderma renal crisis as important causes of death. Providing it is realized that our appreciation of the clinical features associated with anti-U1RNP have evolved over time, MCTD remains a useful concept in clinical practice. Whether it can be credited with the term 'disease' awaits the demonstration of common etiopathological events underlying the development of antibodies to U1 RNP and their associated clinical features.

Humans↗
PubMed2005

[MCTD--mixed connective tissue disease].

Mixed connective tissue disease is a disease entity characterized by overlapping symptoms of lupus erythematosus (LE), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA). Diagnostic criteria include high titers of antibodies against U1RNP as well as the presence of at least 3 of 5 of the following clinical features: edema of hands, synovitis, myositis, Raynaud phenomenon and acroscierosis. In terms of the pathogenesis, genetic as well as infectious (viral) factors appear to play a role. The acceptance of MCTD as a distinct disease entity is controversial. Terms such as "undifferentiated connective tissue disease" or "overlapping syndromes" are not helpful. One-quarter of MCTD patients transform into LE, while one-third progress to SSc. Therapeutic recommendations are glucocorticoids in combination with immunosuppressive agents and endothelin receptor antagonists. Double blind studies are not available. The prognosis is relatively good. Causes of death include pulmonary hypertension, infections and both pulmonary and cardiac failure.

Diagnosis, Differential↗
PubMed2006

Rheumatoid factor isotypes in mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is a connective tissue disorder that is often accompanied by various immunological abnormalities. In this study, we analyzed serum levels of rheumatoid factor (RF) isotypes in patients with MCTD and in normal controls to determine if any of these isotypes reflects the severity of the disease. IgM-RF, IgG-RF, and IgA-RF were positive in 48, 38, and 33% of the patients, respectively. The frequency of positive anti-SS-A antibody and decrease in white blood cell counts were significantly greater in patients with elevated IgA-RF levels than that in those with normal levels. These results suggest that the presence of RF isotypes can be regarded as one of the various immunological abnormalities of MCTD.

Adult↗
PubMed1996

Long term follow-up of children with mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is characterized by features of more than one of the rheumatic disorders with antinuclear antibodies in a speckled pattern and with antibodies to nuclear ribonucleoprotein (nRNP). MCTD is uncommon in children and long-term follow-up studies in children are infrequently reported. A retrospective review of clinical experience at five pediatric rheumatology centers provided 11 patients who met the following inclusion criteria: (1) Kasukawa's criteria for MCTD1; (2) presentation younger than 18th birthday; (3) greater than five years of follow-up; (4) completion of data collection form. The widely varying outcomes of these 11 children with MCTD on long-term follow-up may lend doubt that this is a unique and distinctive rheumatologic disorder.

Adolescent↗
PubMed1996

Neutralization of HIV type 1 infectivity by serum antibodies from a subset of autoimmune patients with mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is a rheumatic disorder with clinical similarities to HIV-1 infection, and with characteristic autoimmune anti-RNP antibodies specific for the U1 snRNP splicing complex. Anti-RNP antibodies cross-react with the HIV-1 surface, owing to multiple homologies between the gp120/41 envelope complex and the 70K protein of U1 snRNP. A key epitope of 70K, its RNA-binding site, is homologous to a dominant B and T cell epitope in the third variable loop (V3) of gp120. In this study, we tested the ability of anti-RNP sera to inhibit HIV-1 infectivity in vitro. Of nine sera tested, five were 70-99% effective in neutralizing one or more HIV-1 strains. One serum was > 99% effective in neutralizing HIV-1MN, and 86 and 77% effective against the primary isolates HIV-1(CO) and HIV-1(JR-FL), respectively, an efficacy equal to that of a pool of broadly neutralizing antibodies from HIV-1-infected subjects (HIVIG). The mean neutralizing titer of anti-RNP sera against HIV-1(JR-FL) was 3.9-fold higher than that of HIVIG. Neutralizing potency was associated with high reactivity to gp120 by ELISA, and with the presence of serum rheumatoid factor, known to enhance antibody neutralization of other viruses. The current findings provide further evidence that individuals unexposed to HIV-1 may develop immunologic resistance by alternative mechanisms, possibly including molecular mimicry, or exposure to as yet unidentified retroviruses. Thus MCTD, which involves both B and T cell reactivity to self-epitopes homologous to HIV-1, may elucidate new strategies for generating protective immunity to this virus.

Antibodies, Antinuclear↗
PubMed2005

[Interstitial lung disease in mixed connective tissue disease].

INTRODUCTION: The authors analyzed the incidence of interstitial lung disease in mixed connective tissue disease. They were seeking an answer to the following problems: the nature of the pathological course of mixed connective tissue disease complicated by and the therapy to be used in interstitial lung disease. PATIENTS AND METHODS: 179 patients were followed up during a period of 15.9 +/- 6.1 years. Interstitial lung disease was diagnosed using high resolution computed tomography. The diagnosis of interstitial lung disease was not obvious in 5 patients thus open lung biopsy was performed, which confirmed common interstitial pneumonitis. The patients were followed-up, and the data of computed tomography and respiratory function tests were detected 6 months, and then 4 years after the acute lung disease complicated by mixed connective tissue disease. RESULTS: Out of the 179 mixed connective tissue disease patients 96 (53.6%) had interstitial lung disease. The onset of interstitial lung disease was the most frequent in the 2-4 years of the disease. Four years after the first appearance of interstitial lung disease severe fibrosis was diagnosed in 24 patients (25%). A honey comb formation in the lung developed only in one patient. For the treatment of interstitial lung disease, corticosteroid treatment had to be combined with cyclophosphamide in 51 cases. In 4 patients (24%), pulmonary arterial hypertension evolved 2-4 years following interstitial lung disease. The high pulmonary arterial pressure decreased using pulsed corticosteroid treatment, cyclophosphamide, prostacyclin analogue, anticoagulants therapy and the 4 patients stay alive. The pulmonary arterial hypertension was caused by obliterative vasculopathy. CONCLUSION: Pulmonary involvement is found in more than half of the patients with mixed connective tissue disease. Early diagnosis of interstitial lung disease is possible by computed tomography. Interstitial lung disease can be treated by the combination of corticosteroids and cyclophosphamide. The authors were the first to detect the coexistence of interstitial lung disease and pulmonary arterial hypertension in mixed connective tissue disease. Subsequent respiratory alterations in these patient necessitate regular patient follow up.

Adult↗