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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↗

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↗

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↗

PubMed1978

The hand in mixed connective tissue disease.

The mixed connective tissue disease syndrome has been described in the medical literature. The clinical and serological characteristics of the syndrome are defined in this paper. The hands of these patients differ from the hands of patients with systemic lupus, rheumatoid arthritis, or systemic sclerosis. In 10 patients there were no erosive changes on radiological examination and all 10 patients had Raynaud's phenomenon. The most striking finding was tightness in the flexors. Mild cases of flexor tightness improved with systemic steroids. One patient with severe flexor tightness required surgical release of adhesions from a chronic inflammatory process of fascia, muscle, and tenosynovium. Biochemical studies showed an abnormal collagen pattern that may be distinct for mixed connective tissue disease.

Adolescent↗

PubMed1992

Lungs in mixed connective tissue disease.

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.

Humans↗

PubMedDate not supplied

Mixed connective tissue disease in siblings.

Mixed connective tissue disease (MCTD) was diagnosed in a brother and sister, and 18 additional family members spanning three generations were studied to detect evidence of autoimmune disease. Symptoms or signs of MCTD without complete expression of the disease were found in 8 relatives of the original cases. Antibodies to ribonucleoprotein and high-titer antinuclear antibodies were found only in the affected siblings. Tests for rheumatoid factor were positive in 9 of 17 relatives of the patients; the titers ranged from 1:160 to 1:2560. The brother and sister with MCTD had an identical HLA genotype--11,12/2,12. The same genotype was inherited by 3 of their siblings, who had impressive rheumatic complaints. This report emphasizes the association between inflammatory connective tissue disease and a specific HLA type within a single kindred.

Adult↗

PubMed1978

The arthritis of mixed connective tissue disease.

Twenty patients with mixed connective tissue disease were followed for 5 years. Arthritis occurred in all 20 patients, being the presenting complaint in 11 patients. The joints most frequently involved were the proximal interphalangeal (PIP), metacarpophalangeal (MCP), wrists, metatarsophalangeal (MTP), and knee; the distribution tended to be symmetrical, mimicking early rheumatoid arthritis. Joint deformities occurred in 6 patients, but apart from 1 patient with arthritis mutilans, significant functional impairment was not encountered. Radiologically small punched out bone erosions, asymmetrically distributed, were the most characteristic finding; other notable changes were aseptic necrosis, tuft erosions, and periarticular calcification. Joint effusions were non-inflammatory, the cellular content was predominantly lymphocytic and the C3 level was normal. Most cases were controlled with non-steroidal anti-inflammatory agents and invariably responded to prednisone less than or equal to 7.5 mg/day.

Adolescent↗

PubMed1976

Mixed connective tissue disease.

Three patients with mixed connective tissue disease (MCTD) had clinical features that included a high incidence of Raynaud phenomenon, arthritis, myositis, and swollen hands. The diagnostic laboratory test result was the presence of high titers of antibody to extractable nuclear antigen. These antibody titers are notably reduced or abolished in patients with MCTD when the tanned red blood cells that are used in the test are pretreated with ribonuclease. Speckled antinuclear antibodies were present in all patients. Patients with MCTD have a low incidence of renal disease, are responsive to treatment with prednisone, and have a good prognosis.

Adolescent↗

PubMed1978

Isolated trigeminal sensory neuropathy: early manifestation of mixed connective tissue disease.

A young woman with mixed connective tissue disease (MCTD) had an isolated trigeminal sensory neuropathy as an early manifestation of the disease. Raynaud phenomenon occurred almost synchronously with the onset of trigeminal neuropathy and was followed by myositis, diffuse hand swelling, synovitis, and increased ribonucleoprotein antibody. Mixed connective tissue disease has overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by high-titer antibody to ribonucleoprotein.

Adult↗

PubMed1992

Orofacial manifestations of mixed connective tissue disease with an uncommon serologic evolution.

Mixed connective tissue disease is a multisystemic disorder with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by a high titer of antibody to ribonucleoprotein. Orofacial manifestations of mixed connective tissue disease include trigeminal neuralgia-like pain, neuropathy, features suggestive of Sjögren's syndrome, and lymphadenopathy. Our recent experience with one patient with trigeminal neuropathy, facial paralysis, Sjögren's syndrome, and aseptic meningitis as early manifestations of the disease, together with an uncommon serologic evolution, is described.

Adult↗

PubMed1977

Immune-complex glomerulonephritis in a patient with mixed connective tissue disease.

Renal involvement and hypocomplementemia in mixed connective tissue disease are reported to be rare. A patient is described here with mixed connective tissue disease and persistently low serum C'3 levels in whom renal insufficiency and nephrotic syndrome developed secondary to immune-complex glomerulonephritis. Light microscopy of the renal biopsy specimen showed predominantly a membranous lesion. Immunofluorescent staining showed granular deposition along the basement membrane of immunoglobulin G, immunoglobulin M, fibrinogen and C3. Electron microscopy showed numerous electron-dense deposits along the glomerular capillary membrane and in the mesangium.

Adult↗

PubMed1976

Symptomatic Sjögren's syndrome in mixed connective tissue disease.

Twelve of 25 patients with mixed connective tissue disease complained of xerostomia and/or ocular symptoms of keratoconjunctivitis sicca. In addition to the clinical features of mixed connective tissue disease, all 12 patients had high titers of antibody to the ribonuclease-sensitive component of the extractable nuclear antigen. Eight patients had both clinical xerostomia and keratoconjunctivitis sicca, one had keratoconjunctivitis sicca and salivary gland enlargement, while there had xerostomia but no ocular complaints. Sjörgren's syndrome was confirmed in all 12 patients by means of Schirmer's tests, Rose Bengal staining tests, salivary gland scintiscans, radionuclide excretion studies in saliva, parotid sialographies, and lip biopsies. At least three of these tests were abnormal in all patients.

Collagen Diseases↗

PubMed1978

Mixed connective tissue disease in children.

A diagnosis of Mixed Connective Tissue Disease (MCTD) had been made in five juveniles in the past two years. All have antibodies to the ribonucleoprotein (RNP) component of extractable fluorescence pattern. No child has had life-threatening complications, though three have required steroids to control their symptoms. Antibodies to ENA were looked for in the other sub-groups of Juvenile Chronic Arthritis (JCA); only two of 75 patients with chronic iridocyclitis and antinuclear antibodies had weakly positive tests.

Adolescent↗

PubMed1978

Neuropsychiatric problems in mixed connective tissue disease.

A group of 20 patients with mixed connective tissue disease, followed for up to five years, was found to have a 55 per cent incidence of neuropsychiatric problems. An aseptic meningitis-like syndrome was the most common presentation and was rapidly responsive to corticosteroid therapy. Other findings were psychosis, convulsions, peripheral neuropathy, trigeminal neuropathy and cerebellar ataxia. An abnormal cerebrospinal fluid was found in five patients; mild pleocytosis, an increased protein content and a first phase colloidal gold curve were the main abnormalities. These neuropsychiatric problems have not been a cause of mortality in this group of patients with mixed connective tissue disease.

Adrenal Cortex Hormones↗

PubMed1978

Clinical features of the arthritis of mixed connective tissue disease.

Seventeen of 19 patients with mixed connective tissue disease (MCTD) had arthritis as a significant initial feature of their disease; 8 were given an initial diagnosis of rheumatoid arthritis (RA) and 4 received chrysotherapy. RA-like hand deformities were present in 35% and contractures and/or persistent loss of joint motion in 47%. Joint radiographs showed abnormalities in 41% and included erosions and/or cysts in 30%. The arthritis of MCTD may be both erosive and deforming and this disease should be considered in patients presenting as RA with unusual features.

Adolescent↗

PubMed1977

Mixed connective tissue disease: the spectrum of radiographic manifestations.

Mixed connective tissue disease (MCTD) is a serologically distinct entity defined by a ribonuclease-sensitive extractable nuclear antigen. This unusual overlap syndrome has clinical features of scleroderma, systemic lupus erythematosus, polymyositis, and rheumatoid arthritis. In order to define the radiographic changes in MCTD, radiographs of the hands of 17 patients were studied, utilizing a fine-detail technique. Diffuse and periarticular osteopenia were found in 8 and 10 patients, respectively; soft-tissue swelling in 11; erosive changes in 9; joint-space narrowing in 7; tuft resorption and soft-tissue atrophy in 6; and subluxations in 2. In individual cases radiographs may appear normal or exhibit features of scleroderma, systemic lupus erythematosus or rheumatoid arthritis, thereby mirroring the clinical diversity of this entity.

Adolescent↗

PubMed1992

Pleuritis-pericarditis--an unusual initial manifestation of mixed connective tissue disease.

Cardiac involvement has been reported in mixed connective tissue disease (MCTD). We describe a 16-year-old girl in whom pleuritis and pericarditis occurred as the initial clinical manifestations of MCTD. Although pleuritis and pericarditis form a common clinical entity in MCTD, it is rarely seen as an initial manifestation. If MCTD is suspected, the diagnosis can be made by the clinical findings and the occurrence of a high titre of antibody against ribonuclease-sensitive ribonucleoprotein (RNP). This report emphasizes the importance of screening for connective tissue disease in patients with pericarditis/pleuritis.

Adolescent↗

PubMed1977

[Sharp's syndrome (mixed connective tissue disease). Clinical aspects diagnosis and prognosis].

Sharp syndrome (mixed connective tissue disease) is a distinct rheumatic syndrome with symptoms of various connective tissue diseases (rheumatoid arthritis, systemic lupus erythematodes, progressive systemic sclerosis, polymyositis and others). 15 patients with mixed connective tissue disease are described. The clinical picture and diagnostic criteria are evaluated and the course of the disease, treatment and prognosis are discussed.

Adolescent↗